Monday, 3 December 2018

PSORIASIS

PSORIASIS
Psoriasis is a skin disorder that causes skin cells to multiply up to 10 times faster than normal. This makes the skin build up into bumpy red patches covered with white scales. They can grow anywhere, but most appear on the scalp, elbows, knees, and lower back. Psoriasis can't be passed from person to person. It does sometimes happen in members of the same family.
PSORIASIS  

PSORIASIS

 Psoriasis usually appears in early adulthood. For most people, it affects just a few areas. In severe cases, psoriasis can cover large parts of the body. The patches can heal and then come back throughout a person's life.get the basics on psoriasis
SYMPTOMS
The symptoms of psoriasis vary depending on the type you have. Some common symptoms for plaque psoriasis -- the most common variety of the condition -- include
Plaques of red skin, often covered with silver-colored scales. These plaques may be itchy and painful, and they sometimes crack and bleed. In severe cases, the plaques will grow and merge, covering large areas.
Disorders of the fingernails and toenails, including discoloration and pitting of the nails. The nails may also crumble or detach from the nail bed.
Plaques of scales or crust on the scalp.
People with psoriasis can also get a type of arthritis called psoriatic arthritis. It causes pain and swelling in the joints. The National Psoriasis Foundation estimates that between 10% to 30% of people with psoriasis also have psoriatic arthritis.
TYPES
Other types of psoriasis include:
PUSTULAR PSORIASIS- which causes red and scaly skin with tiny pustules on the palms of the hands and soles of the feet.
GUTTATE PSORIASIS- which often starts in childhood or young adulthood, causes small, red spots, mainly on the torso and limbs. Triggers may be respiratory infections, strep throat, tonsillitis, stress, injury to the skin, and taking antimalarial and beta-blocker medications.
INVERSE PSORIASIS- which makes bright red, shiny lesions that appear in skin folds, such as the armpits, groin, and under the breasts.
ERYTHRODERMIC PSORIASIS- which causes fiery redness of the skin and shedding of scales in sheets. It's triggered by severe sunburn, infections, certain medications, and stopping some kinds of psoriasis treatment. It needs to be treated immediately because it can lead to severe illness.
CAUSES
No one knows the exact cause of psoriasis, but experts believe that it’s a combination of things. Something wrong with the immune system causes inflammation, triggering new skin cells to form too quickly. Normally, skin cells are replaced every 10 to 30 days. With psoriasis, new cells grow every 3 to 4 days. The buildup of old cells being replaced by new ones creates those silver scales.
Psoriasis tends to run in families, but it may be skip generations. For instance, a grandfather and his grandson may be affected, but not the child's mother.
Things that can trigger an outbreak of psoriasis include:
  • Cuts, scrapes, or surgery
  • Emotional stress
  • Strep infections
  • Medications, including
  • Blood pressure medications (like beta-blockers)
  • Hydroxychloroquine, antimalarial medication
  • Diagnosis
  • Physical exam. It’s usually easy for your doctor to diagnose psoriasis, especially if you have plaques on areas such as your:
  • Scalp
  • Ears
  • Elbows
  • Knees
  • Belly button
  • Nails
Lab tests. The doctor might do a biopsy -- remove a small piece of skin and test it to make sure you don’t have a skin infection. There’s no other test to confirm or rule out psoriasis.
TREATMENT
Luckily, there are many treatments. Some slow the growth of new skin cells, and others relieve itching and dry skin. Your doctor will select a treatment plan that is right for you based on the size of your rash, where it is on your body, your age, your overall health, and other things. Common treatments include:
STEROIDS CREAM
Moisturizers for dry skin
Coal tar (a common treatment for scalp psoriasis available in lotions, creams, foams, shampoos, and bath solutions)
cream or ointment (a strong kind ordered by your doctor. Vitamin D in foods and pills has no effect.)
Retinoid creams
Treatments for moderate to severe psoriasis include:
LIGHT THERAPY- A doctor shines ultraviolet light on your skin to slow the growth of skin cells. PUVA is a treatment that combines a medicine called psoralen with a special form of ultraviolet light.
PSORIASISPSORIASIS LIGHT THERAPY
  PSORIASIS LIGHT THERAPY

 METHOTRAXATE- This drug can cause bone marrow and liver disease as well as lung problems, so it’s only for serious cases. Doctors closely watch patients. You will have to get lab tests, perhaps a chest X-ray, and possibly a liver biopsy.
RETINOIDS These pills, creams, foams, lotions, and gels are a class of drugs related to vitamin A. Retinoids can cause serious side effects, including birth defects, so they’re not recommended for women who are pregnant or planning to have children.
. This drug, made to suppress the immune system, may be taken for serious cases that do not respond to other treatments. It can damage the kidneys and raise blood pressure, so your doctor will closely watch your health while you take it.
BIOLOGIC TREATMENT-These work by blocking the body's immune system (which is overactive in psoriasis) to better control the inflammation from psoriasis. Biologic medications include adalimumab (Humira), brodalumab (Siliq), certolizumab pegol (Cimzia) etanercept (Enbrel), guselkumab (Tremfya), infliximab (Remicade), ixekizumab (Taltz), secukinumab (Cosentyx), tildrakizumab (Ilumya), and ustekinumab (Stelara).
An enzyme inhibitor. The medication apremilast (Otezla) is a new kind of drug for long-term inflammatory diseases like psoriasis and psoriatic arthritis. It's a pill that blocks a specific enzyme, which helps to slow other reactions that lead to inflammation.

PHOTOTHERAPY
Phototherapy or light therapy, involves exposing the skin to ultraviolet light on a regular basis and under medical supervision. Treatments are done in a doctor's office or psoriasis clinic or at home with phototherapy unit. The key to success with light therapy is consistency.
National Psoriasis Foundation does not support the use of indoor tanning beds as a substitute for phototherapy performed with a prescription and under a doctor's supervision. Indoor tanning raises the risk of melanoma by 59 percent, according to the American Academy of Dermatology and the World Health Organization, and does not provide the type of light that most effectively treats psoriasis. Read more on the Psoriasis Foundation position on indoor tanning beds »
Find a provider who offers phototherapy in our Health Care Provider Directory »
Learn more about phototherapy by contacting our Patient Navigation Center »
  • Learn about different types of light therapy.
    Ultraviolet light B (UVB)
  • Sunlight
  • Psoralen + UVA (PUVA)
  • Laser Treatments
  • Tanning beds
ULTRAVIOLET LIGHT B (UVB)
UVB PHOTOTHERAPY
Present in natural sunlight, ultraviolet B (UVB) is an effective treatment for psoriasis. UVB penetrates the skin and slows the growth of affected skin cells. Treatment involves exposing the skin to an artificial UVB light source for a set length of time on a regular schedule. This treatment is administered in a medical setting or at home.
There are two types of UVB treatment, broad band and narrow band. The major difference between them is that narrow band UVB light bulbs release a smaller range of ultraviolet light. Narrow-band UVB is similar to broad-band UVB in many ways. Several studies indicate that narrow-band UVB clears psoriasis faster and produces longer remissions than broad-band UVB. It also may be effective with fewer treatments per week than broad-band UVB.
During UVB treatment, your psoriasis may worsen temporarily before improving. The skin may redden and itch from exposure to the UVB light. To avoid further irritation, the amount of UVB administered may need to be reduced. Occasionally, temporary flares occur with low-level doses of UVB. These reactions tend to resolve with continued treatment.
UVB can be combined with other topical and/or systemic agents to enhance efficacy, but some of these may increase photosensitivity and burning, or shorten remission. Combining UVB with systemic therapies may increase efficacy dramatically and allow for lower doses of the systemic medication to be used.
UVB treatment is offered in different ways. This can include small units for localized areas such as the hands and feet, full body units or handheld units. Some UVB units use traditional UV lamps or bulbs, and others use LED bulbs.
HOME UVB PHOTOTHERAPY
Treating psoriasis with a UVB light unit at home is an economical and convenient choice for many people. Like phototherapy in a clinic, it requires a consistent treatment schedule. Individuals are treated initially at a medical facility and then begin using a light unit at home.
It is critical when doing phototherapy at home to follow a doctor's instructions and continue with regular check-ups. Home phototherapy is a medical treatment that requires monitoring by a health care professional.
All phototherapy treatments, including purchase of equipment for home use, require a prescription. Some insurance companies will cover the cost of home UVB equipment. Vendors of home phototherapy equipment often will assist you in working with your insurance company to purchase a unit.
SUNLIGHT
Although both UVB and ultraviolet light A (UVA) are found in sunlight, UVB works best for psoriasis. UVB from the sun works the same way as UVB in phototherapy treatments.
Short, multiple exposures to sunlight are recommended. Start with five to 10 minutes of noontime sun daily. Gradually increase exposure time by 30 seconds if the skin tolerates it. To get the most from the sun, all affected areas should receive equal and adequate exposure. Remember to wear sunscreen on areas of your skin unaffected by psoriasis.
Avoid overexposure and sunburn. It can take several weeks to see improvement. Have your doctor check you regularly for sun damage.
Some topical medications can increase the risk of sunburn. These include tazarotene, coal tar, Elidel (pimecrolimus) and Protopic (tacrolimus). Individuals using these products should talk with a doctor before going in the sun.
People who are using PUVA or other forms of light therapy should limit or avoid exposure to natural sunlight unless directed by a doctor.

PSORALEN + UVA (PUVA)
Like UVB, ultraviolet light A (UVA) is present in sunlight. Unlike UVB, UVA is relatively ineffective unless used with a light-sensitizing medication psoralen, which is administered topically or orally. This process, called PUVA, slows down excessive skin cell growth and can clear psoriasis symptoms for varying periods of time. Stable plaque psoriasis, guttate psoriasis, and psoriasis of the palms and soles are most responsive to PUVA treatment.
[caption id="attachment_1758" align="aligncenter" width="550"]PUVA PUVA[/caption]The most common short-term side effects of PUVA are nausea, itching and redness of the skin. Drinking milk or ginger ale, taking ginger supplements or eating while taking oral psoralen may prevent nausea. Antihistamines, baths with colloidal oatmeal products or application of topical products with capsaicin may help relieve itching. Swelling of the legs from standing during PUVA treatment may be relieved by wearing support hose.
LASER TRETMENT
EXCIMER LASER
The excimer laser—recently approved by the Food and Drug Administration (FDA) for treating chronic, localized psoriasis plaques—emits a high-intensity beam of ultraviolet light B (UVB).
The excimer laser can target select areas of the skin affected by mild to moderate psoriasis, and research indicates it is a particularly effective treatment for scalp psoriasis. Researchers at the University of Utah, for example, reported in The Journal of Drugs in Dermatology that in a small series of patients, laser treatment, combined with a topical steroid, cleared scalp psoriasis that resisted other treatment.

Individual response to the treatment varies. It can take an average of four to 10 sessions to see results, depending on the particular case of psoriasis. It is recommended that patients receive two treatments per week, with a minimum of 48 hours between treatments.
There is not yet enough long-term data to indicate how long the improvement will last following a course of laser therapy.

TANNING BEDS
Some people visit tanning salons as an alternative to natural sunlight. Tanning beds in commercial salons emit mostly UVA light, not UVB. The beneficial effect for psoriasis is attributed primarily to UVB light. National Psoriasis Foundation does not support the use of indoor tanning beds as a substitute for phototherapy performed with a prescription and under a doctor's supervision. Read more on the Psoriasis Foundation position on indoor tanning bed.

The American Academy of Dermatology, the Food and Drug Administration (FDA) and the Centers for Disease Control and Prevention all discourage the use of tanning beds and sun lamps. Indoor tanning raises the risk of melanoma by 59 percent, according to the American Academy of Dermatology and the World Health Organization. In May 2014, the FDA reclassified sunlamps (which are used in tanning beds and booths) from Class I (low risk) to Class II (moderate risk) products. The FDA can exert more regulatory control over Class II products, according to a press release on the FDA website.
The ultraviolet radiation from these devices can damage the skin, cause premature aging and increase the risk of skin cancer.

Wednesday, 21 November 2018

TATROLOGY OF FALLOT

INTRODUCTION
Tetralogy of Fallot (teh-TRAL-uh-jee of fuh-LOW) is a rare condition caused by a combination of four heart defects that are present at birth (congenital).

These defects, which affect the structure of the heart, cause oxygen-poor blood to flow out of the heart and to the rest of the body. Infants and children with tetralogy of Fallot usually have blue-tinged skin because their blood doesn't carry enough oxygen.

Tetralogy of Fallot is often diagnosed during infancy or soon after. However, tetralogy of Fallot might not be detected until later in life in some adults, depending on the severity of the defects and symptoms.
With early diagnosis followed by appropriate surgical treatment, most children and adults who have tetralogy of Fallot live relatively normal lives, though they'll need regular medical care throughout life and might have restrictions on exercise
.
SYMPTOMS
Tetralogy of Fallot symptoms vary, depending on the extent of obstruction of blood flow out of the right ventricle and into the lungs. Signs and symptoms may include:
A bluish coloration of the skin caused by blood low in oxygen (cyanosis)
Shortness of breath and rapid breathing, especially during feeding or exercise
Loss of consciousness (fainting)
Clubbing of fingers and toes — an abnormal, rounded shape of the nail bed
Poor weight gain
Tiring easily during play or exercise
Irritability
Prolonged crying
A heart murmur
Tet spells
Sometimes, babies who have tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying or feeding, or when agitated.
These episodes are called tet spells and are caused by a rapid drop in the amount of oxygen in the blood. Tet spells are most common in young infants, around 2 to 4 months old. Toddlers or older children might instinctively squat when they're short of breath. Squatting increases blood flow to the lungs.
Seek medical help if you notice that your baby has the following symptoms:
  • Difficulty breathing
  • Bluish discoloration of the skin
  • Passing out or seizures
  • Weakness
  • Unusual irritability
  • If your baby becomes blue (cyanotic), place your baby on his or her side and pull your baby's knees up to his or her chest. This helps increase blood flow to the lungs.
CAUSES
 
                                                       Tatrology of fallot
Tetralogy of Fallot occurs during fetal growth, when the baby's heart is developing. While factors such as poor maternal nutrition, viral illness or genetic disorders might increase the risk of this condition, in most cases the cause of tetralogy of Fallot is unknown.
The four abnormalities that make up the tetralogy of Fallot include:
  • PULMONARY VALVE STENOSIS- Pulmonary valve stenosis is a narrowing of the pulmonary valve — the valve that separates the lower right chamber of the heart (right ventricle) from the main blood vessel leading to the lungs (pulmonary artery).
Narrowing (constriction) of the pulmonary valve reduces blood flow to the lungs. The narrowing might also affect the muscle beneath the pulmonary valve. In some severe cases, the pulmonary valve doesn't form properly (pulmonary atresia) and causes reduced blood flow to the lungs.
  • VENTRICULAR SEPTAL DEFECT- A ventricular septal defect is a hole (defect) in the wall (septum) that separates the two lower chambers of the heart -the left and right ventricles. The hole allows deoxygenated blood in the right ventricle — blood that has circulated through the body and is returning to the lungs to replenish its oxygen supply — to flow into the left ventricle and mix with oxygenated blood fresh from the lungs.
Blood from the left ventricle also flows back to the right ventricle in an inefficient manner. This ability for blood to flow through the ventricular septal defect reduces the supply of oxygenated blood to the body and eventually can weaken the heart.
  • OVERRIDING AORTA- Normally the aorta — the main artery leading out to the body — branches off the left ventricle. In tetralogy of Fallot, the aorta is shifted slightly to the right and lies directly above the ventricular septal defect.
In this position the aorta receives blood from both the right and left ventricles, mixing the oxygen-poor blood from the right ventricle with the oxygen-rich blood from the left ventricle.
  • RIGHT VENTRICULAR HYPERTROPHY- When the heart's pumping action is overworked, it causes the muscular wall of the right ventricle to thicken. Over time this might cause the heart to stiffen, become weak and eventually fail.
    Some children or adults who have tetralogy of Fallot may have other heart defects, such as a hole between the heart's upper chambers (atrial septal defect), a right aortic arch or abnormalities of the coronary arteries.
RISK FACTOR
While the exact cause of tetralogy of Fallot is unknown, various factors might increase the risk of a baby being born with this condition. These risk factors include:
  • A viral illness during pregnancy, such as rubella (German measles)
  • Alcoholism during pregnancy
  • Poor nutrition during pregnancy
  • A mother older than age 40
  • A parent who has tetralogy of Fallot
  • The presence of Down syndrome or DiGeorge syndrome
COMPLICATIONS
All babies who have tetralogy of Fallot need corrective surgery. Without treatment, your baby might not grow and develop properly.
Your baby may also be at an increased risk of serious complications, such as infective endocarditis — an infection of the inner lining of the heart or heart valve caused by a bacterial infection.
Untreated cases of tetralogy of Fallot usually develop severe complications over time, which might result in death or disability by early adulthood.

PHYSICAL THERAPY
 
PRE-OPERATIVE – Most infants with TOF will receive thoracic surgery to correct the associated abnormalities. The most important physical therapy intervention pre-operative for the patient and family is education. Many institutes are using a method of education that describes a pre-operative program that helps to decrease post-operative complications. Many institutions use a doll to explain to children about the tubes and how to care for them.
POST-OPERATIVE– Pulmonary function is a main area of concentration after thoracic surgery. The interventions vary with age but the general goal is the same, to mobilize secretions, increase aeration and increase general mobility. Atelectasis is a concern after thoracic surgery and is caused by the slowed mucous transport, altered breathing patterns, prolonged positioning in supine and diaphragmatic dysfunction in early post-operative period. The yawn maneuver and prolonged inspiration to increase inflation are ways to prevent atelectasis. The incentive spirometry is an effective tool for preventing atelectasis in the pediatric population. Other ways that can be more fun for children are activities such as blowing bubbles or blowing on a windmill. There are expiratory maneuvers but the children typically take a large breath before blowing, thus they become inspiratory maneuvers also.

Segmental expansion techniques are effective to increase segmental aeration. These techniques are performed by placing your hand over whichever segment of the lung is not effectively inflating and allowing your hand to move with the respiratory cycle. Gentle pressure may be applied to the chest at the end of the expiratory phase, just before the inhalation phase. This elicits a stretch reflex that facilitates air flow to that particular segment.

Percussion and vibration may be performed in conjunction with segmental expansion. Percussion is the rhythmic clapping with cupped hands over the involved lung segment performed throughout the respiratory cycle, with the goal of mechanically dislodging pulmonary secretions. Vibration is performed by creating a fine oscillating movement of the hands on the chest wall just before expiration begins and throughout the expiration phase. If the child does not tolerate percussion and vibration, placing the patient in side lying and gently rocking back and forth may stimulate expansion, secretion motion and relaxation. This technique is affective if the child is upset and may decrease respiratory rate.

Positioning may be useful for patients, but certain positions may be contraindicated post thoracic surgery. Check with the MD or nurse before using positioning to make sure that it is safe for the child.

POSTURAL DRAINAGE
If the child is intubated suctioning may be used to remove secretions from the airways, however children with cyanotic defects (such as TOF) tend to desaturate during suctioning and it is very important to hyperventilate these patients before and after suctioning and to monitor oxygen levels.
Coughing is an effective way to mobilize secretions if the patient is able and willing to cough. A teddy bear or pillow can be used by the child to self-splint the incision. Have the child squeeze the stuffed animal against their chest to decrease pain.

Range of motion (ROM) exercises should be initiated as soon as possible after surgery. ROM exercises are extremely important with thoracic surgery because this type of incision tends to produce more guarding. Child position should be changed regularly to avoid a pooling of secretions in the dependent part of the lungs and regular position changes reduce the risk of fevers after surgery. Ambulation should be implemented as soon as possible to decrease both pulmonary and circulatory complications. As soon as atrial lines and groin lines are removed, patients should begin to ambulate, even if only 5-10 ft.

DIFFERENTIAL DIAGNOSIS
Any other cyanotic heart defects, such as, pulmonary stenosis, transposed arterial trunks, common arterial trunk and tricuspid atresia. Patients with heart murmurs will also be tests for persistent pulmonary hypertension.

Thursday, 15 November 2018

COARCTACTION OF AORTA

Coarctation (ko-ahrk-TAY-shun) of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrowed part of your aorta.















Coarctation of the aorta is generally present at birth (congenital). The condition can range from mild to severe, and might not be detected until adulthood, depending on how much the aorta is narrowed.

Coarctation of the aorta often occurs along with other heart defects. While treatment is usually successful, the condition requires careful lifelong follow-up.

SYMPTOMS
Coarctation of the aorta symptoms depend on the severity of the condition. Most people don't have symptoms. Children with serious aortic narrowing may show signs and symptoms earlier in life, but mild cases with no symptoms might not be diagnosed until adulthood. People may also have signs or symptoms of other heart defects that they have along with coarctation of the aorta.

Babies with severe coarctation of the aorta may begin having signs and symptoms shortly after birth. These include:

Pale skin
Irritability
Heavy sweating
Difficulty breathing
Difficulty feeding
Left untreated, aortic coarctation in babies might lead to heart failure or death.

Older children and adults with coarctation of the aorta often don't have symptoms because their narrowing may be less severe. If you have signs or symptoms that appear after infancy, you most commonly will have high blood pressure (hypertension) measured in your arms. However, your blood pressure is likely to be lower in your legs. Signs and symptoms might include:

High blood pressure
Headache
Muscle weakness
Leg cramps or cold feet
Nosebleeds
Chest pain
When to see a doctor
Seek medical help if you or your child has the following signs or symptoms:

Severe chest pain
Fainting
Sudden shortness of breath
Unexplained high blood pressure
While experiencing these signs or symptoms doesn't necessarily mean that you have a serious problem, it's best to get checked out quickly. Early detection and treatment might help save your life.

CAUSES
Doctors aren't certain what causes coarctation of the aorta (aortic coarctation). For unknown reasons, mild to severe narrowing develops in part of the aorta. Although aortic coarctation can occur anywhere along the aorta, the coarctation is most often located near a blood vessel called the ductus arteriosus. The condition generally begins before birth (congenital). Congenital heart defects are the most common of all birth defects.

Rarely, coarctation of the aorta develops later in life. Traumatic injury might lead to coarctation of the aorta. Rarely, severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu's arteritis) can narrow the aorta, leading to aortic coarctation.

Coarctation of the aorta usually occurs beyond the blood vessels that branch off to your upper body and before the blood vessels that lead to your lower body. This can often lead to high blood pressure in your arms but low blood pressure in your legs and ankles.

With coarctation of the aorta, the lower left heart chamber (left ventricle) of your heart works harder to pump blood through the narrowed aorta, and blood pressure increases in the left ventricle. This may cause the wall of the left ventricle to thicken (hypertrophy).

RISK FACTORS
Coarctation of the aorta often occurs along with other congenital heart defects, although doctors don't know what causes multiple heart defects to form together. The condition is more common in males than in females. You or your child may be more likely to have aortic coarctation if certain heart conditions exist, including:

1.Bicuspid aortic valve. The aortic valve separates the lower left chamber (left ventricle) of the heart from the aorta. A bicuspid aortic valve has two flaps (cusps) instead of the usual three. Many people with coarctation of the aorta have a bicuspid aortic valve.
Patent ductus arteriosus. Before birth, the ductus arteriosus is a blood vessel connecting the left pulmonary artery to the aorta — allowing blood to bypass the lungs.

Shortly after birth, the ductus arteriosus usually closes. If it remains open, it's called a patent ductus arteriosus.

Holes in the wall between the left and right sides of the heart. You may have a hole in the wall (septum) between the upper chambers of the heart (atrial septal defect) or the lower chambers of the heart (ventricular septal defect) when you're born.

This causes oxygen-rich blood from the left side of the heart to mix with oxygen-poor blood in the right side of the heart.

2.Aortic valve stenosis. This is a narrowing of the valve that separates the left ventricle of the heart from the aorta (aortic valve). This means your heart has to pump harder to get adequate blood flow to your body.

Over time, this can cause your heart muscle to thicken and lead to symptoms such as chest pain, fainting spells and breathlessness, or heart failure.

3.Aortic valve regurgitation. This occurs when the aortic valve doesn't close tightly, causing blood to leak backward into the left ventricle.
Mitral valve stenosis. This is a narrowing of the valve (mitral valve) between the upper left heart chamber (left atrium) and the left ventricle that lets blood flow through the left side of your heart.

In this condition, blood may back up into your lungs, causing shortness of breath or lung congestion. Like aortic valve stenosis, this condition can also lead to heart failure.

4.Mitral valve regurgitation. This occurs when the mitral valve doesn't close tightly, causing blood to leak backward into the left atrium.
Coarctation of the aorta is also more common in people who have certain genetic conditions, such as Turner syndrome. Women and girls with Turner syndrome have 45 chromosomes, with one missing or incomplete X chromosome, instead of 46. About 10 percent of women and girls with Turner syndrome have aortic coarctation.

COMPLICATIONS
Untreated coarctation of the aorta frequently leads to complications. Some complications may be a result of long-standing high blood pressure caused by the aortic coarctation. Complications are also possible after treatment for coarctation of the aorta.

Complications of coarctation of the aorta may include:

Narrowing of the aortic valve (aortic stenosis)
High blood pressure
Stroke
Enlargement in a section of the wall of the aorta (aneurysm)
Aortic rupture or tear (dissection)
Premature coronary artery disease — narrowing of the blood vessels that supply the heart
Heart failure
A weakened or bulging artery in the brain (brain aneurysm) or bleeding in the brain (hemorrhage)
In addition, if the coarctation of the aorta is severe, your heart might not be able to pump enough blood to your other organs. This can cause damage to your heart and also can result in kidney failure or other organ failure.

If your coarctation of the aorta was treated when you were young, you have a risk of the aorta re-narrowing (re-coarctation) over time. You also have a higher risk of developing high blood pressure. You will need lifelong follow-up for coarctation of the aorta, and you may require additional treatments.

PREVENTION
Coarctation of the aorta can't be prevented, because it's usually present at birth (congenital). However, if you or your child has a condition that increases the risk of aortic coarctation, such as Turner syndrome, bicuspid aortic valve or another heart defect, or a family history of congenital heart disease, early detection can help.

PHYSIOTHERAPY IN PRE- AND POSTOPERATIVE PERIOD

Physiotherapy in the pre- and postoperative period is indicated in pediatric cardiac surgery in order to reduce the risk of pulmonary complications (retention of secretions, atelectasis and pneumonia)  as well as to treat such complications as it contributes to the appropriate ventilation and successful extubation .

In the preoperative, physiotherapy uses techniques of clearance, reexpansion, abdominal support and guidance on the importance and objectives of physiotherapy intervention for parents or escorts, or patients able to understand such guidance . The techniques used by postoperative physiotherapy include vibration in the chest wall, percussion , compression , manual hyperinflation , reexpansion maneuver , positioning  postural drainage , cough stimulation, aspiration , breathing exercises , mobilization  and AEF (acceleration of expiratory flow)  .

There are few current studies on the role of physiotherapy in the postoperative of pediatric cardiac surgery , especially those that approach the effectiveness of physiotherapy in the preoperative to prevent pulmonary complications after heart surgery.

Felcar et al. performed a study with 141 children with congenital heart disease, aged varying between one day old to six years, randomly divided into two groups, whereas one of them received physiotherapy in the pre- and postoperative and the other only postoperatively. The study obtained statistically significant difference regarding the presence of pulmonary complications (pneumonia and atelectasis), being more frequent in the group undergoing physiotherapy only postoperatively. Moreover, when the presence of pulmonary complications was associated with other complications regarding the time of hospital stay, such as sepsis, pneumothorax, pleural effusion and others, the group that received physiotherapy before and after surgery showed a lower risk of developing such complications. These findings demonstrate the importance of preventive action of physiotherapy preoperatively.

The study by Main et al.  compared the effectiveness of aspiration with the techniques of respiratory physiotherapy (manual vibration, percussion, compression, manual hyperinflation, positioning and postural drainage) in 83 participants with a mean age of 9 months. The respiratory parameters (expiratory tidal volume, resistance and lung compliance) were measured 15 minutes before treatment and after 30 minutes, and lasting for 60 minutes after the intervention in case there was no need for clinical intervention. The duration of physiotherapy was higher than the aspiration . It was observed that physiotherapy tends to produce improvement in expiratory tidal volume, pulmonary compliance and pulmonary resistance.

According to Kavanagh , the treatment for atelectasis consists of physiotherapy, deep breathing, incentive spirometry. However, sometimes, atelectasis is difficult to reverse and it is necessary association with another method, as in the case report from Silva et al. , in which a child with congenital heart disease underwent heart surgery and developed this pulmonary complication after extubation in the postoperative period and the reversal of this presentation was achieved after the association of respiratory physiotherapy with inhalation of hypertonic saline solution with NaCl at 6%.

Chest radiographs and four physiotherapy sessions lasting 20 minutes were performed daily in this study, using maneuvers of pulmonary reexpansion and bronchial hygiene, bronchial postural drainage and tracheal aspiration. Immediately before and after physiotherapy inhalation of hypertonic saline solution with NaCl at 6% was associated. The authors found that this association was shown to be effective in this case .

Breathing exercises are indicated in cases of atelectasis due to thoracic or upper abdominal surgery, because they improve the respiratory efficiency, increase the diameter of the airways, which helps to dislodge secretions, preventing alveolar collapse, and facilitating the expansion of the lung and peripheral airways clerance .

Campos et al.  analyzed the effect of increased expiratory flow (IEF) in heart rate, respiratory rate and oxygen saturation in 48 children diagnosed with pneumonia. The variables were assessed before physiotherapy, in the first and fifth minutes after physiotherapy. The authors found a statistically significant increase in oxygen saturation and statistically significant reduction in cardiac and respiratory rate after intervention with IEF and concluded that this physiotherapeutic technique for bronchial hygiene is effective in improving lung function.

Wednesday, 24 October 2018

PATENT DUCTUS ARTERIOSUS

PATENT DUCTUS ARTERIOSUS


PDA is a heart problem that is frequently noted in the first few weeks or months after birth. It is characterized by the persistence of a normal fetal connection between the aorta and the pulmonary artery which allows oxygen-rich (red) blood that should go to the body to recirculate through the lungs.
All babies are born with this connection between the aorta and the pulmonary artery. While your baby was developing in the uterus, it was not necessary for blood to circulate through the lungs because oxygen was provided through the placenta. During pregnancy, a connection was necessary to allow oxygen-rich (red) blood to bypass your baby's lungs and proceed into the body. This normal connection that all babies have is called a ductus arteriosus.
At birth, the placenta is removed when the umbilical cord is cut. Your baby's lungs must now provide oxygen to his or her body. As your baby takes the first breath, the blood vessels in the lungs open up, and blood begins to flow through them to pick up oxygen. At this point, the ductus arteriosus is not needed to bypass the lungs. Under normal circumstances, within the first few days after birth, the ductus arteriosus closes and blood no longer passes through it.
Illustration of the anatomy of a heart with a patent ductus arteriosus
Click Image to Enlarge
In some babies, however, the ductus arteriosus remains open (patent) and the condition now becomes known as patent ductus arteriosus (PDA). The opening between the aorta and the pulmonary artery allows oxygen-rich (red) blood to recirculate into the lungs.
Patent ductus arteriosus occurs twice as often in girls as in boys.
CAUSES OF PDA
A PDA is almost always present at birth. In some children, the PDA does not close. Although exact reasons why this happens in some patients and not in others are not known, the most common association for a PDA is prematurity.
PDA can also occur in combination with other heart defects.
WHY IS PDA A CONCERN
When the ductus arteriosus stays open, oxygen-rich (red) blood passes from the aorta to the pulmonary artery, mixing with the oxygen-poor (blue) blood already flowing to the lungs. The blood vessels in the lungs have to handle a larger amount of blood than normal. How well the lung vessels are able to adapt to the extra blood flow depends on how big the PDA is and how much blood is able to pass through it from the aorta.
Extra blood causes higher pressure in the blood vessels in the lungs. The larger the volume of blood that goes to the lungs at high pressure, the more the lungs have to cope with this extra blood at high pressure.
Children may have difficulty breathing because of this extra blood flow to the lungs at high pressure. They may remain on the ventilator for a longer period of time if they are premature. The support from the ventilator also may be high, due to this extra blood flow to the lungs.
Rarely, untreated PDA may lead to long-term lung damage. This is uncommon, however, since most children will have been treated for their PDA before the lungs get damaged.
Often, the PDA may be "silent," that is, causing no symptoms. This is especially true in older patients (beyond the first few months of life) with small PDAs.
SYMPTOMS
The size of the connection between the aorta and the pulmonary artery will affect the type of symptoms noted, the severity of symptoms, and the age at which they first occur. The larger the opening, the greater the amount of blood that passes through that overloads the lungs.
A child with a small patent ductus arteriosus might not have any symptoms, and your child's doctor may have only noted the defect by hearing a heart murmur. Other infants with a larger PDA may exhibit different symptoms. The following are the most common symptoms of PDA. However, each child may experience symptoms differently. Symptoms may include
Fatigue
Sweating
Rapid breathing
Heavy breathing
Congested breathing
Disinterest in feeding, or tiring while feeding
Poor weight gain
The symptoms of a PDA may resemble other medical conditions or heart problems. Always consult your child's doctor for a diagnosis.
DIAGNOSIS
Your child's doctor may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. In this case, a heart murmur is a noise caused by the turbulence of blood flowing through the PDA.
A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest where the murmur is heard best, as well as the loudness and quality of the murmur (such as, harsh or blowing) will give the cardiologist an initial idea of which heart problem your child may have. Diagnostic testing for congenital heart disease varies by the child's age, clinical condition, and institutional preferences. Some tests that may be recommended include the following
:Chest X-ray.
A diagnostic test that uses invisible X-ray beams to produce images of internal tissues, bones, and organs onto film. With a PDA, the heart may be enlarged due to larger amounts of blood flow recirculating through the lungs back to the heart. Also, there may be changes that take place in the lungs due to extra blood flow that can be seen on an X-ray.
Electrocardiogram (ECG or EKG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress.
Echocardiogram (echo). A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. An echo can show the pattern of blood flow through the PDA, and determine how large the opening is, as well as how much blood is passing through it. An echo is the most common way that a PDA is diagnosed.

Cardiac catheterization. A cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart. The cardiac catheterization procedure may also be an option for treatment. During the procedure, the child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Once the catheter is in the heart, the cardiologist will pass a special device, either a coil or a PDA occluder (depending on the size of the PDA). This device will close the PDA and therefore stop the blood flow through the PDA.

TREATMENT OF PDA
A small patent ductus arteriosus may close spontaneously as your child grows. A PDA that causes symptoms will require medical management, and possibly even surgical repair. Your child's cardiologist will check periodically to see whether the PDA is closing on its own. If a PDA does not close on its own, it will be repaired to prevent lung problems that will develop from long-time exposure to extra blood flow. Treatment may include:
Medical management. In premature infants, an intravenous (IV) medication called indomethacin may help close a patent ductus arteriosus. Indomethacin is related to aspirin and ibuprofen and works by stimulating the muscles inside the PDA to constrict, thereby closing the connection. Your child's doctor can answer any further questions you may have about this treatment. As previously mentioned, some children will have no symptoms, and require no medications. However, others may need to take medications to help the heart and lungs work better. Medications may be prescribed, such as diuretics. Diuretics help the kidneys remove excess fluid from the body. This may be necessary because the body's water balance can be affected when the heart is not working as efficiently as it could. Your doctor may also ask you to restrict the amount of fluid your child takes in.
Adequate nutrition. Most infants with PDA eat and grow normally, but premature infants or those infants with a large PDA may become tired when feeding, and are not able to eat enough to gain weight. Options that can be used to ensure your baby will have adequate nutrition include the following:

High-calorie formula or breast milk. Special nutritional supplements may be added to formula or pumped breast milk that increase the number of calories in each ounce, thereby allowing your baby to drink less and still consume enough calories to grow properly.

Supplemental tube feedings. Feedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, can either supplement or take the place of bottle-feedings. Infants who can drink part of their bottle, but not all, may be fed the remainder through the feeding tube. Infants who are too tired to bottle-feed may receive their formula or breast milk through the feeding tube alone.

PDA repair or closure. The majority of children and some infants with PDA are candidates for repair in the cardiac cath lab. The goal is to repair the PDA before the lungs become diseased from too much blood flow and pressure and to restore an efficient pattern of blood flow. Surgical repair is also indicated if one of the previously mentioned conservative treatments have not been successful. Repair is usually indicated in infants younger than 6 months of age who have large defects that are causing symptoms, such as poor weight gain and rapid breathing. For infants who do not exhibit symptoms, the repair may often be delayed until after 6 to 12 months of age. Your child's cardiologist will recommend when the repair should be performed. Transcatheter coil closure of the PDA is frequently performed first if possible because it is minimally invasive. Children need to be at least 5 kg to be considered for transcatheter closure. Thus, premature infants, because of their small size, are not candidates for this procedure, and require surgical closure of the PDA. Your child's PDA may be repaired surgically in the operating room. The surgical repair, also called PDA ligation, is performed under general anesthesia. The procedure involves closing the open PDA with stitches or clips in order to prevent the surplus blood from entering your child's lungs.
POSTPROCEDURE  CARE  FOR  YOUR  CHILD :
Cath lab repair or closure procedure. When the procedure is complete, the catheter(s) will be withdrawn. Several gauze pads and a large piece of medical tape will be placed on the site where the catheter was inserted to prevent bleeding. In some cases, a small, flat weight or sandbag may be used to help keep pressure on the catheterization site and decrease the chance of bleeding. If blood vessels in the leg were used, your child will be told to keep the leg straight for a few hours after the procedure to minimize the chance of bleeding at the catheterization site. Your child will be taken to a unit in the hospital where he or she will be monitored by nursing staff for several hours after the test. The length of time it takes for your child to wake up after the procedure will depend on the type of medicine given to your child for relaxation prior to the test, and also on your child's reaction to the medication. After the procedure, your child's nurse will monitor the pulses and skin temperature in the leg or arm that was used for the procedure. Your child may be able to go home after a specified period of time, providing he or she does not need further treatment or monitoring. You will receive written instructions regarding care of the catheterization site, bathing, activity restrictions, and any new medications your child may need to take at home.

Surgical repair. Some children who undergo PDA ligation may need to spend some time in the intensive care unit after surgery. Others may return to a regular hospital room. Your child will be kept as comfortable as possible with medications which relieve pain or anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child. You will also learn how to care for your child at home before your child is discharged. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged. Most children will only need to stay in the hospital for a few days after the operation.
HOME CARE
Most infants and older children feel comfortable when they go home. Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child's doctor will discuss pain control before your child is discharged from the hospital.
Often, infants who fed poorly prior to surgery have more energy after the recuperation period, and begin to eat better and gain weight faster.
After surgery, older children usually have a fair tolerance for activity. Within a few weeks, your child should be fully recovered and able to participate in normal activity.
You will receive additional instructions from your child's doctors and the hospital staff.

PATENT DUCTUS ARTERIOSUS
PDA is a heart problem that is frequently noted in the first few weeks or months after birth. It is characterized by the persistence of a normal fetal connection between the aorta and the pulmonary artery which allows oxygen-rich (red) blood that should go to the body to recirculate through the lungs.
All babies are born with this connection between the aorta and the pulmonary artery. While your baby was developing in the uterus, it was not necessary for blood to circulate through the lungs because oxygen was provided through the placenta. During pregnancy, a connection was necessary to allow oxygen-rich (red) blood to bypass your baby's lungs and proceed into the body. This normal connection that all babies have is called a ductus arteriosus.
At birth, the placenta is removed when the umbilical cord is cut. Your baby's lungs must now provide oxygen to his or her body. As your baby takes the first breath, the blood vessels in the lungs open up, and blood begins to flow through them to pick up oxygen. At this point, the ductus arteriosus is not needed to bypass the lungs. Under normal circumstances, within the first few days after birth, the ductus arteriosus closes and blood no longer passes through it.
Illustration of the anatomy of a heart with a patent ductus arteriosus
Click Image to Enlarge
In some babies, however, the ductus arteriosus remains open (patent) and the condition now becomes known as patent ductus arteriosus (PDA). The opening between the aorta and the pulmonary artery allows oxygen-rich (red) blood to recirculate into the lungs.
Patent ductus arteriosus occurs twice as often in girls as in boys.
CAUSES OF PDA
A PDA is almost always present at birth. In some children, the PDA does not close. Although exact reasons why this happens in some patients and not in others are not known, the most common association for a PDA is prematurity.
PDA can also occur in combination with other heart defects.
WHY IS PDA A CONCERN
When the ductus arteriosus stays open, oxygen-rich (red) blood passes from the aorta to the pulmonary artery, mixing with the oxygen-poor (blue) blood already flowing to the lungs. The blood vessels in the lungs have to handle a larger amount of blood than normal. How well the lung vessels are able to adapt to the extra blood flow depends on how big the PDA is and how much blood is able to pass through it from the aorta.
Extra blood causes higher pressure in the blood vessels in the lungs. The larger the volume of blood that goes to the lungs at high pressure, the more the lungs have to cope with this extra blood at high pressure.
Children may have difficulty breathing because of this extra blood flow to the lungs at high pressure. They may remain on the ventilator for a longer period of time if they are premature. The support from the ventilator also may be high, due to this extra blood flow to the lungs.
Rarely, untreated PDA may lead to long-term lung damage. This is uncommon, however, since most children will have been treated for their PDA before the lungs get damaged.
Often, the PDA may be "silent," that is, causing no symptoms. This is especially true in older patients (beyond the first few months of life) with small PDAs.
SYMPTOMS
The size of the connection between the aorta and the pulmonary artery will affect the type of symptoms noted, the severity of symptoms, and the age at which they first occur. The larger the opening, the greater the amount of blood that passes through that overloads the lungs.
A child with a small patent ductus arteriosus might not have any symptoms, and your child's doctor may have only noted the defect by hearing a heart murmur. Other infants with a larger PDA may exhibit different symptoms. The following are the most common symptoms of PDA. However, each child may experience symptoms differently. Symptoms may include
Fatigue
Sweating
Rapid breathing
Heavy breathing
Congested breathing
Disinterest in feeding, or tiring while feeding
Poor weight gain
The symptoms of a PDA may resemble other medical conditions or heart problems. Always consult your child's doctor for a diagnosis.
DIAGNOSIS
Your child's doctor may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. In this case, a heart murmur is a noise caused by the turbulence of blood flowing through the PDA.
A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest where the murmur is heard best, as well as the loudness and quality of the murmur (such as, harsh or blowing) will give the cardiologist an initial idea of which heart problem your child may have. Diagnostic testing for congenital heart disease varies by the child's age, clinical condition, and institutional preferences. Some tests that may be recommended include the following
:Chest X-ray.
A diagnostic test that uses invisible X-ray beams to produce images of internal tissues, bones, and organs onto film. With a PDA, the heart may be enlarged due to larger amounts of blood flow recirculating through the lungs back to the heart. Also, there may be changes that take place in the lungs due to extra blood flow that can be seen on an X-ray.
Electrocardiogram (ECG or EKG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress.
Echocardiogram (echo). A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. An echo can show the pattern of blood flow through the PDA, and determine how large the opening is, as well as how much blood is passing through it. An echo is the most common way that a PDA is diagnosed.
Cardiac catheterization. A cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart. The cardiac catheterization procedure may also be an option for treatment. During the procedure, the child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Once the catheter is in the heart, the cardiologist will pass a special device, either a coil or a PDA occluder (depending on the size of the PDA). This device will close the PDA and therefore stop the blood flow through the PDA.
TREATMENT OF PDA
A small patent ductus arteriosus may close spontaneously as your child grows. A PDA that causes symptoms will require medical management, and possibly even surgical repair. Your child's cardiologist will check periodically to see whether the PDA is closing on its own. If a PDA does not close on its own, it will be repaired to prevent lung problems that will develop from long-time exposure to extra blood flow. Treatment may include:
Medical management. In premature infants, an intravenous (IV) medication called indomethacin may help close a patent ductus arteriosus. Indomethacin is related to aspirin and ibuprofen and works by stimulating the muscles inside the PDA to constrict, thereby closing the connection. Your child's doctor can answer any further questions you may have about this treatment. As previously mentioned, some children will have no symptoms, and require no medications. However, others may need to take medications to help the heart and lungs work better. Medications may be prescribed, such as diuretics. Diuretics help the kidneys remove excess fluid from the body. This may be necessary because the body's water balance can be affected when the heart is not working as efficiently as it could. Your doctor may also ask you to restrict the amount of fluid your child takes in.
Adequate nutrition. Most infants with PDA eat and grow normally, but premature infants or those infants with a large PDA may become tired when feeding, and are not able to eat enough to gain weight. Options that can be used to ensure your baby will have adequate nutrition include the following:
High-calorie formula or breast milk. Special nutritional supplements may be added to formula or pumped breast milk that increase the number of calories in each ounce, thereby allowing your baby to drink less and still consume enough calories to grow properly.
Supplemental tube feedings. Feedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, can either supplement or take the place of bottle-feedings. Infants who can drink part of their bottle, but not all, may be fed the remainder through the feeding tube. Infants who are too tired to bottle-feed may receive their formula or breast milk through the feeding tube alone.
PDA repair or closure. The majority of children and some infants with PDA are candidates for repair in the cardiac cath lab. The goal is to repair the PDA before the lungs become diseased from too much blood flow and pressure and to restore an efficient pattern of blood flow. Surgical repair is also indicated if one of the previously mentioned conservative treatments have not been successful. Repair is usually indicated in infants younger than 6 months of age who have large defects that are causing symptoms, such as poor weight gain and rapid breathing. For infants who do not exhibit symptoms, the repair may often be delayed until after 6 to 12 months of age. Your child's cardiologist will recommend when the repair should be performed. Transcatheter coil closure of the PDA is frequently performed first if possible because it is minimally invasive. Children need to be at least 5 kg to be considered for transcatheter closure. Thus, premature infants, because of their small size, are not candidates for this procedure, and require surgical closure of the PDA. Your child's PDA may be repaired surgically in the operating room. The surgical repair, also called PDA ligation, is performed under general anesthesia. The procedure involves closing the open PDA with stitches or clips in order to prevent the surplus blood from entering your child's lungs.
POSTPROCEDURE  CARE  FOR  YOUR  CHILD :
Cath lab repair or closure procedure. When the procedure is complete, the catheter(s) will be withdrawn. Several gauze pads and a large piece of medical tape will be placed on the site where the catheter was inserted to prevent bleeding. In some cases, a small, flat weight or sandbag may be used to help keep pressure on the catheterization site and decrease the chance of bleeding. If blood vessels in the leg were used, your child will be told to keep the leg straight for a few hours after the procedure to minimize the chance of bleeding at the catheterization site. Your child will be taken to a unit in the hospital where he or she will be monitored by nursing staff for several hours after the test. The length of time it takes for your child to wake up after the procedure will depend on the type of medicine given to your child for relaxation prior to the test, and also on your child's reaction to the medication. After the procedure, your child's nurse will monitor the pulses and skin temperature in the leg or arm that was used for the procedure. Your child may be able to go home after a specified period of time, providing he or she does not need further treatment or monitoring. You will receive written instructions regarding care of the catheterization site, bathing, activity restrictions, and any new medications your child may need to take at home.
Surgical repair. Some children who undergo PDA ligation may need to spend some time in the intensive care unit after surgery. Others may return to a regular hospital room. Your child will be kept as comfortable as possible with medications which relieve pain or anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child. You will also learn how to care for your child at home before your child is discharged. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged. Most children will only need to stay in the hospital for a few days after the operation.

HOME CARE
Most infants and older children feel comfortable when they go home. Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child's doctor will discuss pain control before your child is discharged from the hospital.
Often, infants who fed poorly prior to surgery have more energy after the recuperation period, and begin to eat better and gain weight faster.

After surgery, older children usually have a fair tolerance for activity. Within a few weeks, your child should be fully recovered and able to participate in normal activity.
You will receive additional instructions from your child's doctors and the hospital staff.

PHYSIOTHERAPY INPHYSIOTHERAPY IN PRE- AND POSTOPERATIVE PERIOD
Physiotherapy in the pre- and postoperative period is indicated in pediatric cardiac surgery in order to reduce the risk of pulmonary complications (retention of secretions, atelectasis and pneumonia)  as well as to treat such complications as it contributes to the appropriate ventilation and successful extubation .
In the preoperative, physiotherapy uses techniques of clearance, reexpansion, abdominal support and guidance on the importance and objectives of physiotherapy intervention for parents or escorts, or patients able to understand such guidance . The techniques used by postoperative physiotherapy include vibration in the chest wall, percussion , compression , manual hyperinflation , reexpansion maneuver , positioning  postural drainage , cough stimulation , aspiration, breathing exercises , mobilization  and AEF (acceleration of expiratory flow)  .
There are few current studies on the role of physiotherapy in the postoperative of pediatric cardiac surgery , especially those that approach the effectiveness of physiotherapy in the preoperative to prevent pulmonary complications after heart surgery. 

The study obtained statistically significant difference regarding the presence of pulmonary complications (pneumonia and atelectasis), being more frequent in the group undergoing physiotherapy only postoperatively. Moreover, when the presence of pulmonary complications was associated with other complications regarding the time of hospital stay, such as sepsis, pneumothorax, pleural effusion and others, the group that received physiotherapy before and after surgery showed a lower risk of developing such complications. These findings demonstrate 
 the importance of preventive action of physiotherapy preoperatively. 

 The respiratory parameters (expiratory tidal volume, resistance and lung compliance) were measured 15 minutes before treatment and after 30 minutes, and lasting for 60 minutes after the intervention in case there was no need for clinical intervention.

According to Kavanagh , the treatment for atelectasis consists of physiotherapy, deep breathing, incentive spirometry. However, sometimes, atelectasis is difficult to reverse and it is necessary association with another method, as in the case report from Silva et al. , in which a child with congenital heart disease underwent heart surgery and developed this pulmonary complication after extubation in the postoperative period and the reversal of this presentation was achieved after the association of respiratory physiotherapy with inhalation of hypertonic saline solution with NaCl at 6%. 

Chest radiographs and four physiotherapy sessions lasting 20 minutes were performed daily in this study, using maneuvers of pulmonary reexpansion and bronchial hygiene, bronchial postural drainage and tracheal aspiration. Immediately before and after physiotherapy inhalation of hypertonic saline solution with NaCl at 6% was associated. The authors found that this association was shown to be effective in this case . 

Breathing exercises are indicated in cases of atelectasis due to thoracic or upper abdominal surgery, because they improve the respiratory efficiency, increase the diameter of the airways, which helps to dislodge secretions, preventing alveolar collapse, and facilitating the expansion of the lung and peripheral airways clerance .

Thursday, 18 October 2018

ATRIOVENTRICULAR SEPTAL DEFECT

INTRODUCTION                                                
Congenital heart diseases affect about 8 to 10 children per 1000 live births and it is estimated the occurrence of 28,846 new cases per year in Brazil, where, on average, 23,077 surgical procedures are needed per year .
The most common congenital heart diseases in the study of Miyague et al. were acyanotic anomalies such as ventricular septal defect (30.5%), atrial septal defect (19.1%), patent ductus arteriosus (17% ), pulmonary valve stenosis (11.3%) and aortic coarctation (6.3%), while the most common cyanotic anomalies were tetralogy of Fallot (6.9%), transposition of great vessels (4.1%), tricuspid atresia (2.3%) and total anomalous pulmonary veins drainage (2%).
Children with congenital heart disease often develop changes in respiratory mechanics . In addition, heart surgery associated with cardiopulmonary bypass (CPB) also leads to a number of respiratory complications . Thus, physiotherapy in pre- and postoperative period have as main objectives the pulmonary reexpansion, airway clearance and guidance for those responsible concerning prevention of these complications .
This review aimed to update knowledge regarding the role of physiotherapy in the preoperative and postoperative pediatric cardiac surgery in the prevention of pulmonary complications.
An atrioventricular septal defect (pronounced EY-tree-oh-ven-TRIC-u-lar SEP-tal DEE-fekt) or AVSD is a heart defect affecting the valves between the heart’s upper and lower chambers and the walls between the chambers.
DEFINATION
An atrioventricular septal defect (AVSD) is a heart defect in which there are holes between the chambers of the right and left sides of the heart, and the valves that control the flow of blood between these chambers may not be formed correctly. This condition is also called atrioventricular canal (AV canal) defect or endocardial cushion defect. In AVSD, blood flows where it normally should not go. The blood may also have a lower than normal amount of oxygen, and extra blood can flow to the lungs. This extra blood being pumped into the lungs forces the heart and lungs to work hard and may lead to congestive heart failure.
There are two general types of AVSD that can occur, depending on which structures are not formed correctly:
COMPLETE AVSD

                                                        Complete-AV-Canal-Defect

A complete AVSD occurs when there is a large hole in the center of the heart which allows blood to flow between all four chambers of the heart. This hole occurs where the septa (walls) separating the two top chambers (atria) and two bottom chambers (ventricles) normally meet. There is also one common atrioventricular valve in the center of the heart instead of two separate valves – the tricuspid valve on the right side of the heart and the mitral valve on the left side of the heart. This common valve often has leaflets (flaps) that may not be formed correctly or do not close tightly. A complete AVSD arises during pregnancy when the common valve fails to separate into the two distinct valves (tricuspid and mitral valves) and when the septa (walls) that split the upper and lower chambers of the heart do not grow all the way to meet in the center of the heart.
PATRIAL OR INCOMPLETE AVSD
A partial or incomplete AVSD occurs when the heart has some, but not all of the defects of a complete AVSD. There is usually a hole in the atrial wall or in the ventricular wall near the center of the heart. A partial AVSD usually has both mitral and tricuspid valves, but one of the valves (usually mitral) may not close completely, allowing blood to leak backward from the left ventricle into the left atrium.
OCCURENCE
The Centers for Disease Control and Prevention (CDC) estimates that about 2,000 babies (1 in 2,120 babies) are born with AVSD every year in the United States.
CAUSES AND RISK FACTORS
The causes of congenital heart defects, such as AVSD, among most babies are unknown. Some babies have heart defects because of changes in their genes or chromosomes. In particular, AVSD is common in babies with Down syndrome, a genetic condition that involves an extra chromosome 21 (also called trisomy 21). Congenital heart defects are also thought to be caused by the combination of genes and other risk factors, such as things the mother comes in contact with in her environment, what she eats or drinks, or certain medications she uses during pregnancy.
DIAGNOSIS
AVSD may be diagnosed during pregnancy or soon after the baby is born.
DURING PREGNANCY
During pregnancy, there are screening tests (also called prenatal tests) to check for birth defects and other conditions. AVSD may be diagnosed during pregnancy with an ultrasound test (which creates pictures of the baby using sound waves), but whether or not the defect can be seen with the ultrasound test depends on the size or type (partial or complete) of the AVSD. The healthcare provider can request a fetal echocardiogram to confirm the diagnosis if AVSD is suspected. A fetal echocardiogram is an ultrasound of the baby’s heart which shows more detail than the routine prenatal ultrasound test. The fetal echocardiogram can show problems with the structure of the heart and how well the heart is working.
AFTER BABY IS BORN
During a physical exam of an infant, a complete AVSD may be suspected. Using a stethoscope, a doctor will often hear a heart murmur (an abnormal “whooshing” sound caused by blood flowing through the abnormal hole). However, not all heart murmurs are present at birth. Babies with a complete AVSD usually do show signs of problems within the first few weeks after birth. When symptoms do occur, they may include
  • Breathing problems
  • Pounding heart
  • Weak pulse
  • Ashen or bluish skin color
  • Poor feeding, slow weight gain
  • Tiring easily
  • Swelling of the legs or belly
  • For partial AVSDs, if the holes between the chambers of the heart are not large, the signs and
  • symptoms may not occur in the newborn or infancy periods. In these cases, people with a partial
  • AVSD might not be diagnosed for years.
Symptoms which might indicate that a child’s complete AVSD or partial AVSD is getting worse include
Arrhythmia, an abnormal heart rhythm. An arrhythmia can cause the heart to beat too fast, too slow, or erratically. When the heart does not beat properly, it can’t pump blood effectively.
Congestive heart failure, when the heart cannot pump enough blood and oxygen to meet the needs of the body.
Pulmonary hypertension, a type of high blood pressure that affects the arteries in the lungs and the right side of the heart.
The healthcare provider can request one or more tests to confirm the diagnosis of AVSD. The most common test is an echocardiogram. This is an ultrasound of the heart that can show problems with the structure of the heart, like holes between the chambers of the right and left side of the heart, and any irregular blood flow. An electrocardiogram (EKG), which measures the electrical activity of the heart, chest x-rays, and other medical tests may also be used to make the diagnosis. Because many babies with Down syndrome have an AVSD, all infants with Down syndrome should have an echocardiogram to look for an AVSD or other heart defects.
TREATMENT
All AVSDs, both partial and complete types, usually require surgery. During surgery, any holes in the chambers are closed using patches. If the mitral valve does not close completely, it is repaired or replaced. For a complete AVSD, the common valve is separated into two distinct valves – one on the right side and one on the left side.
The age at which surgery is done depends on the child’s health and the specific structure of the AVSD. If possible, surgery should be done before there is permanent damage to the lungs from too much blood being pumped to the lungs. Medication may be used to treat congestive heart failure, but it is only a short term measure until the infant is strong enough for surgery.
Infants who have surgical repairs for AVSD are not cured; they might have lifelong complications. The most common of these complications is a leaky mitral valve. This is when the mitral valve does not close all the way so that it allows blood to flow backwards through the valve. A leaky mitral valve can cause the heart to work harder to get enough blood to the rest of the body; a leaky mitral valve might have to be surgically repaired. A child or adult with an AVSD will need regular follow-up visits with a cardiologist (a heart doctor) to monitor his or her progress, avoid complications, and check for other health conditions that might develop as the child gets older. With proper treatment, most babies with AVSD grow up to lead healthy, productive lives.
PULMONARY COMPLICATIONS IN PEDIATRIC CARDIAC SURGERY
Pulmonary complications of postoperative pediatric cardiac surgery observed : atelectasis, pneumonia, pleural effusion, pneumothorax, chylothorax, pulmonary hypertension, pulmonary hemorrhage and diaphragmatic paralysis, whereas the first two aforementioned complications are the more common ones.

Atelectasis, defined as collapse of a certain region of the lung parenchyma . is the most common complication in the postoperative period of cardiac surgery , by worsening oxygenation, decreasing pulmonary compliance, leading to inhibition of cough and pulmonary clearance and may lead to respiratory failure and increase pulmonary vascular resistance.

Heart surgeries associated with CPB have as adverse effect the increased capillary permeability that causes edema, which results in decreased lung compliance and gas exchange , in addition to lead to airway obstruction, atelectasis, decreased functional residual capacity and, therefore, hypoxemia .
Stayer et al. assessed the changes in resistance and dynamic pulmonary compliance in 106 children aged less than one year, with congenital heart disease who underwent cardiac surgery with CPB. These variables were measured on two occasions: before the surgical incision with ten minutes of mechanical ventilation and after disconnection of CPB and sternal closure. The authors found that newborns and patients with increased pulmonary blood flow presented preoperatively decreased lung compliance and increased respiratory resistance, whereas after surgery the latter parameter has improved. On the other hand, the infants with normal pulmonary blood flow in the preoperative had decreased lung compliance and developed in the postoperative deterioration of dynamic compliance, however, the pulmonary resistance was not affected. This study showed that heart surgery can alter the respiratory mechanics in newborns and infants.

Among the most common causes of death it can be highlighted the low cardiac output syndrome (48%), followed by lung infections (11%).

Pneumonia is one of the frequent causes of nosocomial infection in the postoperative period of heart surgery and is considered a major cause of morbidity and mortality in this populatio
 . .
PHYSIOTHERAPY IN PRE- AND POSTOPERATIVE PERIOD
Physiotherapy in the pre- and postoperative period is indicated in pediatric cardiac surgery in order to reduce the risk of pulmonary complications (retention of secretions, atelectasis and pneumonia)  as well as to treat such complications as it contributes to the appropriate ventilation and successful extubation .
In the preoperative, physiotherapy uses techniques of clearance, reexpansion, abdominal support and guidance on the importance and objectives of physiotherapy intervention for parents or escorts, or patients able to understand such guidance . The techniques used by postoperative physiotherapy include vibration in the chest wall, percussion , compression , manual hyperinflation , reexpansion maneuver , positioning. postural drainage , cough stimulation , aspiration , breathing exercises , mobilization and AEF (acceleration of expiratory flow) .

There are few current studies on the role of physiotherapy in the postoperative of pediatric cardiac surgery , especially those that approach the effectiveness of physiotherapy in the preoperative to prevent pulmonary complications after heart surgery.

Felcar et al. performed a study with 141 children with congenital heart disease, aged varying between one day old to six years, randomly divided into two groups, whereas one of them received physiotherapy in the pre- and postoperative and the other only postoperatively. The study obtained statistically significant difference regarding the presence of pulmonary complications (pneumonia and atelectasis), being more frequent in the group undergoing physiotherapy only postoperatively. Moreover, when the presence of pulmonary complications was associated with other complications regarding the time of hospital stay, such as sepsis, pneumothorax, pleural effusion and others, the group that received physiotherapy before and after surgery showed a lower risk of developing such complications. These findings demonstrate the importance of preventive action of physiotherapy preoperatively.
According to Kavanagh , the treatment for atelectasis consists of physiotherapy, deep breathing, incentive spirometry. However, sometimes, atelectasis is difficult to reverse and it is necessary association with another method, as in the case report from Silva et al. [16], in which a child with congenital heart disease underwent heart surgery and developed this pulmonary complication after extubation in the postoperative period and the reversal of this presentation was achieved after the association of respiratory physiotherapy with inhalation of hypertonic saline solution with NaCl at 6%.
Chest radiographs and four physiotherapy sessions lasting 20 minutes were performed daily in this study, using maneuvers of pulmonary reexpansion and bronchial hygiene, bronchial postural drainage and tracheal aspiration. Immediately before and after physiotherapy inhalation of hypertonic saline solution with NaCl at 6% was associated. The authors found that this association was shown to be effective in this case .
Breathing exercises are indicated in cases of atelectasis due to thoracic or upper abdominal surgery, because they improve the respiratory efficiency, increase the diameter of the airways, which helps to dislodge secretions, preventing alveolar collapse, and facilitating the expansion of the lung and peripheral airways clerance .
Campos et al. analyzed the effect of increased expiratory flow (IEF) in heart rate, respiratory rate and oxygen saturation in 48 children diagnosed with pneumonia. The variables were assessed before physiotherapy, in the first and fifth minutes after physiotherapy. The authors found a statistically significant increase in oxygen saturation and statistically significant reduction in cardiac and respiratory rate after intervention with IEF and concluded that this physiotherapeutic technique for bronchial hygiene is effective in improving lung function.
FINAL CONSIDERATIONS
The occurrence of pulmonary complications in the postoperative of heart surgery is quite common, and the atelectasis and pneumonia are highlighted among them. Since the frequency of heart surgery in children with congenital heart disease is high, it is important to make use of effective means to prevent, reduce or treat such complications.
Physiotherapy included in the multidisciplinary team contributes significantly to the better prognosis of pediatric patients undergoing heart surgery, as it prevents and treats pulmonary complications by means of specific techniques such as vibration, percussion, compression, manual hyperinflation, reexpansion maneuver, positioning, postural drainage, cough stimulation, aspiration, breathing exercises, IEF and mobilization.
It was observed the effectiveness of physiotherapy in reducing the risk and/or treating pulmonary complications caused by surgical procedure in children with congenital heart disease. Thus, more research is needed to assess the physiotherapy in the pre- and postoperative of pediatric cardiac surgery, by comparing the different techniques used by the physiotherapist in order to minimize the frequent postoperative pulmonary complications.

Monday, 15 October 2018

VENTRICULAR SEPTAL DEFECT

ventricular septal defect (VSD), a hole in the heart, is a common heart defect that's present at birth (congenital). The hole (defect) occurs in the wall (septum) that separates the heart's lower chambers (ventricles) and allows blood to pass from the left to the right side of the heart. The oxygen-rich blood then gets pumped back to the lungs instead of out to the body, causing the heart to work harder.
A small ventricular septal defect may cause no problems, and many small VSDs close on their own. Medium or larger VSDs may need surgical repair early in life to prevent complication.




VENTRICULAR SEPTAL DEFECT
SYMPTOMS
Signs and symptoms of serious heart defects often appear during the first few days, weeks or months of a child's life.
Ventricular septal defect (VSD) symptoms in a baby may include:
  • Poor eating, failure to thrive
  • Fast breathing or breathlessness
  • Easy tiring
Doctor may not notice signs of a ventricular septal defect at birth. If the defect is small, symptoms may not appear until later in childhood — if at all. Signs and symptoms vary depending on the size of the hole and other associated heart defects.
Doctor may first suspect a heart defect during a regular checkup if he or she hears a murmur while listening to your baby's heart with a stethoscope. Sometimes VSDs can be detected by ultrasound before the baby is born.
Sometimes a VSD isn't detected until a person reaches adulthood. Symptoms and signs can include shortness of breath or a heart murmur doctor hears when listening to your heart with a stethoscope.
Call doctor if your baby or child:
Tires easily when eating or playing
Is not gaining weight
Becomes breathless when eating or crying
Breathes rapidly or is short of breath
Call doctor if you develop:
Shortness of breath when you exert yourself or when you lie down
Rapid or irregular heartbeat
Fatigue or weakness
CAUSES
Congenital heart defects arise from problems early in the heart's development, but there's often no clear cause. Genetics and environmental factors may play a role. VSDs can occur alone or with other congenital heart defects.
During fetal development, a ventricular septal defect occurs when the muscular wall separating the heart into left and right sides (septum) fails to form fully between the lower chambers of the heart (ventricles).
Normally, the right side of the heart pumps blood to the lungs to get oxygen; the left side pumps the oxygen-rich blood to the rest of the body. A VSD allows oxygenated blood to mix with deoxygenated blood, causing the heart to work harder to provide enough oxygen to the body's tissues.
VSDs may be various sizes, and they can be present in several locations in the wall between the ventricles. There may be one or more VSD.
It's also possible to acquire a VSD later in life, usually after a heart attack or as a complication following certain heart procedures.
RISK FACTORS
Ventricular septal defects may run in families and sometimes may occur with other genetic problems, such as Down syndrome. If you already have a child with a heart defect, a genetic counselor can discuss the risk of your next child having one.
COMPLICATION
A small ventricular septal defect may never cause any problems. Medium or large defects can cause a range of disabilities — from mild to life-threatening. Treatment can prevent many complications.
Heart failure. In a heart with a medium or large VSD, the heart needs to work harder to pump enough blood to the body. Because of this, heart failure can develop if medium to large VSDs aren't treated.
Pulmonary hypertension. Increased blood flow to the lungs due to the VSD causes high blood pressure in the lung arteries (pulmonary hypertension), which can permanently damage them. This complication can cause reversal of blood flow through the hole (Eisenmenger syndrome).
Endocarditis. This heart infection is an uncommon complication.
Other heart problems. These include abnormal heart rhythms and valve problems.

PREVENTION
In most cases, you can't do anything to prevent having a baby with a ventricular septal defect. However, it's important to do everything possible to have a healthy pregnancy. Here are the basics:
Get early prenatal care, even before you're pregnant. Talk to your doctor before you get pregnant about your health and discuss any lifestyle changes that your doctor may recommend for a healthy pregnancy. Also, be sure you talk to your doctor about any medications you're taking.
Eat a balanced diet. Include a vitamin supplement that contains folic acid. Also, limit caffeine.
Exercise regularly. Work with your doctor to develop an exercise plan that's right for you.
Avoid risks. These include harmful substances such as alcohol, tobacco and illegal drugs.
Avoid infections. Be sure you're up to date on all of your vaccinations before becoming pregnant. Certain types of infections can be harmful to a developing fetus.
Keep diabetes under control. If you have diabetes, work with your doctor to be sure it's well-controlled before getting pregnant.
If you have a family history of heart defects or other genetic disorders, consider talking with a genetic counselor before getting pregnant.

Thursday, 11 October 2018

ATRIAL SEPTAL DEFECT

OVERVIEW
An atrial septal defect (ASD) is a hole in the wall between the two upper chambers of your heart (atria). The condition is present at birth (congenital).
Small defects may never cause a problem and may be found incidentally. It's also possible that small atrial septal defects may close on their own during infancy or early childhood.
Large and long-standing atrial septal defects can damage your heart and lungs. An adult who has had an undetected atrial septal defect for decades may have a shortened life span from heart failure or high blood pressure that affects the arteries in the lungs (pulmonary hypertension). Surgery may be necessary to repair atrial septal defects to prevent complications.


SYMPTOMS
Many babies born with atrial septal defects don't have associated signs or symptoms. In adults, signs or symptoms may begin around age 30, but in some cases signs and symptoms may not occur until decades later.
Atrial septal defect signs and symptoms may include:
Shortness of breath, especially when exercising
Fatigue
Swelling of legs, feet or abdomen
Heart palpitations or skipped beats
Stroke
Heart murmur, a whooshing sound that can be heard through a stethoscope
Shortness of breath
Tiring easily, especially after activity
Swelling of legs, feet or abdomen
Heart palpitations or skipped beats
These could be signs or symptoms of heart failure or another complication of congenital heart disease.

CAUSES
How the heart normally works
The heart is divided into four hollow chambers, two on the right and two on the left. To pump blood throughout the body, the heart uses its left and right sides for different tasks.
The right side of the heart moves blood to the lungs through vessels called pulmonary arteries. In the lungs, blood picks up oxygen then returns to the heart's left side through the pulmonary veins. The left side of the heart then pumps the blood through the aorta and out to the rest of the body.

DEVELOPMENT OF HEART DEFECT
Doctors know that heart defects present at birth (congenital) arise from errors early in the heart's development, but there's often no clear cause. Genetics and environmental factors may play a role.
ATRIAL SEPTUM DEFECT
The heart is divided into four hollow chambers, two on the right and two on the left. To pump blood throughout the body, the heart uses its left and right sides for different tasks.
The right side of the heart moves blood to the lungs through vessels called pulmonary arteries. In the lungs, blood picks up oxygen then returns to the heart's left side through the pulmonary veins. The left side of the heart then pumps the blood through the aorta and out to the rest of the body.

DEELOPMENT OF ASD
Doctors know that heart defects present at birth (congenital) arise from errors early in the heart's development, but there's often no clear cause. Genetics and environmental factors may play a role.

HEART WORKS WITH ASD
An atrial septal defect (ASD) allows freshly oxygenated blood to flow from the left upper chamber of the heart (left atrium) into the right upper chamber of the heart (right atrium). There, it mixes with deoxygenated blood and is pumped to the lungs, even though it's already refreshed with oxygen.
If the atrial septal defect is large, this extra blood volume can overfill the lungs and overwork the right side of the heart. If not treated, the right side of the heart eventually enlarges and weakens. If this process continues, the blood pressure in your lungs may increase as well, leading to pulmonary hypertension.

TYPES OF ASD
 
SECUNDUM- This is the most common type of ASD, and occurs in the middle of the wall between the atria (atrial septum).
PRIMUM- This defect occurs in the lower part of the atrial septum, and may occur with other congenital heart problems.
SINUS VENOSUS- This rare defect usually occurs in the upper part of the atrial septum.
CORONARY SINUS- In this rare defect, part of the wall between the coronary sinus — which is part of the vein system of the heart — and the left atrium is missing.
RISK FACTORS
It's not known why atrial septal defects occur, but congenital heart defects appear to run in families and sometimes occur with other genetic problems, such as Down syndrome. If you have a heart defect, or you have a child with a heart defect, a genetic counselor can estimate the odds that any future children will have one.
Some conditions that you have or that occur during pregnancy may increase your risk of having a baby with a heart defect, including:
  • RUBELLA INFECTION - bcoming infected with rubella (German measles) during the first few months of your pregnancy can increase the risk of fetal heart defects.
  • Drug, tobacco or alcohol use, or exposure to certain substances. Use of certain medications, tobacco, alcohol or drugs, such as cocaine, during pregnancy can harm the developing fetus.
  • Diabetes or lupus. If you have diabetes or lupus, you may be more likely to have a baby with a heart defect.
  • Obesity. Being extremely overweight (obese) may play a role in increasing the risk of having a baby with a birth defect.
  • Phenylketonuria (PKU). If you have PKU and aren't following your PKU meal plan, you may be more likely to have a baby with a heart defect.
  • COMPLICATIONS
    A small atrial septal defect may never cause any problems. Small atrial septal defects often close during infancy.
Larger defects can cause serious problems, including:
  • Right-sided heart failure
  • Heart rhythm abnormalities (arrhythmias)
  • Increased risk of a stroke
  • Shortened life span
  • Less common serious complications may include:
  • Pulmonary hypertension. If a large atrial septal defect goes untreated, increased blood flow to your lungs increases the blood pressure in the lung arteries (pulmonary hypertension).
Eisenmenger syndrome. Pulmonary hypertension can cause permanent lung damage. This complication, called Eisenmenger syndrome, usually develops over many years and occurs uncommonly in people with large atrial septal defects.
Treatment can prevent or help manage many of these complications.

ATRIAL SEPTAL DEFECT AND PREGNANCY
Most women with an atrial septal defect can tolerate pregnancy without any problems. However, having a larger defect or having complications such as heart failure, arrhythmias or pulmonary hypertension can increase your risk of complications during pregnancy. Doctors strongly advise women with Eisenmenger syndrome not to become pregnant because it can endanger their lives.
The risk of congenital heart disease is higher for children of parents with congenital heart disease, whether in the father or the mother. Anyone with a congenital heart defect, repaired or not, who is considering starting a family should carefully discuss it beforehand with a doctor. Some medications may need to be stopped or adjusted before you become pregnant because they can cause serious problems for a developing fetus.

PREVENTION
In most cases, atrial septal defects can't be prevented. If you're planning to become pregnant, schedule a preconception visit with your health care provider. This visit should include:
Getting tested for immunity to rubella. If you're not immune, ask your doctor about getting vaccinated.
Going over your current health conditions and medications. You'll need to carefully monitor certain health problems during pregnancy. Doctor may also recommend adjusting or stopping certain medications before you become pregnant.
Reviewing your family medical history. If you have a family history of heart defects or other genetic disorders, consider talking with a genetic counselor to determine what the risk might be before getting pregnant.