Other Birth Defects of the Heart

Aortopulmonary window is an abnormal opening between the aorta and the main pulmonary artery that takes blood to the lungs. It is very rare. Aortopulmonary window can occur on its own or occur with other heart defects, such as atrial septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more , coarctation of the aorta Coarctation of the Aorta Coarctation of the aorta is a narrowing of part of the aorta, the main blood vessel bringing red oxygenated blood from the heart to the body. The aorta narrows, causing the heart to pump harder... read more , or tetralogy of Fallot Tetralogy of Fallot In tetralogy of Fallot, four specific heart defects occur together. This condition includes four heart defects that can lead to oxygen-poor blood going directly to the body. Symptoms include... read more .

Symptoms are those of left-to-right shunt Shunting of blood flow . Large left-to-right shunts cause too much blood to flow to the lungs and too much blood to return to the left ventricle, which may lead to signs of heart failure Heart Failure (HF) Heart failure is a disorder in which the heart is unable to keep up with the demands of the body, leading to reduced blood flow, back-up (congestion) of blood in the veins and lungs, and/or... read more (for example, rapid breathing, poor feeding, easy tiring, excessive sweating). During infancy, these symptoms often result in failure to thrive Failure to Thrive in Children Failure to thrive is a delay in weight gain and physical growth that can lead to delays in development and maturation. Medical disorders and a lack of proper nutrition are causes of failure... read more . A large left-to-right shunt also eventually leads to high blood pressure in lung arteries (pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension is a condition in which blood pressure in the arteries of the lungs (the pulmonary arteries) is abnormally high. Many disorders can cause pulmonary hypertension. People... read more ). Most infants with an aortopulmonary window have a heart murmur.

Doctors make the diagnosis based on what they find on examining the infant and results of imaging tests, usually echocardiography.

Surgical repair of aortopulmonary window should be done as soon as possible after diagnosis.

Congenitally corrected transposition of the great arteries is relatively rare. In this anomaly, the normal connections of the aorta and the pulmonary artery are reversed, but the lower heart chambers (the ventricles) are also reversed, thus "correcting" the circulation so that the oxygen-poor venous blood goes into the lungs and oxygenated blood goes out to the body as it normally should. Most affected infants have other heart abnormalities as well, such as ventricular septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more , pulmonic stenosis Pulmonary Valve Stenosis in Children Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs. The heart valve... read more , and Ebstein anomaly or another defect of the tricuspid valve.

These abnormalities result in a wide range of symptoms. An abnormally slow heart rhythm (called heart block Overview of Heart Block Heart block is a delay in the conduction of electrical current as it passes through the conduction system of the heart, including the atrioventricular node, bundle of His, or both bundle branches... read more ) may develop. In heart block, the electrical signals from the upper chambers of the heart do not travel down to the lower chambers. Heart blocks is treated with a pacemaker.

As affected children reach adulthood, a common concern is the development of worsening function of the muscular wall of the right ventricle, which may cause heart failure Heart Failure (HF) Heart failure is a disorder in which the heart is unable to keep up with the demands of the body, leading to reduced blood flow, back-up (congestion) of blood in the veins and lungs, and/or... read more .

If severe dysfunction develops, heart transplantation Heart Transplantation Heart transplantation is the removal of a healthy heart from a recently deceased person and then its transfer into the body of a person who has a severe heart disorder that can no longer be... read more may be needed.

In double outlet right ventricle, both the aorta and the pulmonary artery are connected to the right ventricle and no artery attaches to the left ventricle. Infants with double outlet right ventricle also have a hole between the ventricles (ventricular septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more ). Oxygen-rich blood in the left ventricle flows through the ventricular septal defect, allowing the oxygen-rich blood to mix with the oxygen-poor blood in the right ventricle before getting pumped out of the heart. Therefore, some oxygen-poor blood is circulated to the body. Symptoms vary depending on the associated abnormalities in the heart such as pulmonic valve stenosis Pulmonary Valve Stenosis in Children Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs. The heart valve... read more . Symptoms include rapid breathing, poor feeding, easy tiring, and/or bluish color of the lips, nail beds, and skin (cyanosis). Surgical repair is needed.

Ebstein anomaly is an abnormality of the tricuspid valve that causes the valve to leak blood backward into the atrium. Some studies have shown an increased risk of this abnormality in infants whose mothers used the drug lithium during pregnancy. Atrial septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more , pulmonic valve stenosis Pulmonary Valve Stenosis in Children Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs. The heart valve... read more , and Wolff-Parkinson-White syndrome Wolff-Parkinson-White (WPW) Syndrome Wolff-Parkinson-White syndrome is a disorder in which an extra electrical connection between the atria and the ventricles is present at birth. People may have episodes of a very rapid heartbeat... read more often occur together with this abnormality.

Newborns may be bluish in color (indicating the body is not receiving enough oxygen), or symptoms may not develop until adulthood, when an abnormal heart rhythm Overview of Abnormal Heart Rhythms Abnormal heart rhythms (arrhythmias) are sequences of heartbeats that are irregular, too fast, too slow, or conducted via an abnormal electrical pathway through the heart. Heart disorders are... read more occurs.

If the abnormal tricuspid valve causes severe symptoms, the valve is surgically repaired.

In pulmonary atresia, the pulmonic (pulmonary) valve does not form properly and does not open, so blood does not flow into the lungs and pick up oxygen. Therefore, oxygen-poor blood is circulated to the body. Pulmonary atresia frequently occurs together with other heart disorders and with abnormalities of the coronary arteries. The symptoms and surgical treatment options depend on what type of other abnormalities are also present.

Single ventricle abnormalities include any complex heart defect in which there is only one functional ventricle. Symptoms depend on the exact abnormality and whether pulmonic valve stenosis Pulmonary Valve Stenosis in Children Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs. The heart valve... read more is also present. Most infants have a bluish color of the skin (cyanosis). Infants with severe pulmonic stenosis may be very blue and require a surgical procedure fairly quickly. Infants without pulmonic stenosis have excessive blood flow to their lungs and develop symptoms of heart failure. Surgery is also required.

Tricuspid atresia is absence of the tricuspid valve accompanied by an underdeveloped right ventricle. Other heart defects are present and may include atrial septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more , ventricular septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more , patent ductus arteriosus Patent Ductus Arteriosus In patent ductus arteriosus (PDA), the blood vessel connecting the pulmonary artery and the aorta (ductus arteriosus) fails to close as it usually does shortly after birth. Patent ductus arteriosus... read more , pulmonic valve stenosis Pulmonary Valve Stenosis in Children Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs. The heart valve... read more , and transposition of the great arteries Transposition of the Great Arteries Transposition of the great arteries is a reversal of the normal connections of the aorta and the pulmonary artery with the heart. The aorta and pulmonary artery are reversed, which causes oxygen-poor... read more . Infants have a bluish color of the skin (cyanosis). Other symptoms depend on what other abnormalities are also present. Diagnosis is by echocardiography. Treatment is surgical repair.