Atrial septal defect is a hole between the left and right atrium. If not corrected, it leads to arrhythmia, paradoxal systemic embolization and eventually pulmonary hypertension. Although patients may reach an adult age, they will become symptomatic with a shorter life expectancy. 

Treatment: Patients may be treated with a cosmetic right mini axillary thoracotomy, which is done through a small incision under the right arm.

Atrioventricular septal defect (also called atrioventricular canal defect) is a hole between the two ventricles (VSD) and the two atria (ASD); the valves that control the blood flow between these chambers may not be formed correctly and can leak. Without surgery, pulmonary hypertension quickly develops, and heart failure worsens. The best time to operate is 2-6 months of age, as delaying surgery may only increase the risks.

Treatment: Medication can improve heart failure symptoms, but treatment is surgical. Surgery consists of closure of the VSD with a patch, closure of the ASD (patch or direct), repair of the mitral valve (closure of cleft) and eventual repair of the tricuspid valve.

Coarctation of the aorta occurs when a part of the aorta is narrower than usual. If the narrowing is severe enough, the baby may have serious problems and need surgery after birth. Without surgery, left ventricular hypertrophy and myocardial infarction with heart failure may develop along with other health issues that reduce life expectancy.

Treatment: The narrow aorta needs to be widened, ideally before symptoms are present. This can be done with surgery or a balloon angioplasty, a procedure that uses a thin, flexible tube called a catheter that is inserted into a blood vessel and directed to the aorta. When the catheter reaches the narrow area of the aorta, a balloon at the tip is inflated to expand the blood vessel. Sometimes a mesh-covered tube (stent) is inserted to keep the vessel open. The stent is used more often to initially widen the aorta or re-widen it if the aorta narrows again after surgery. During surgery to correct a coarctation, the narrow portion is removed and the aorta is reconstructed or patched to allow blood to flow normally. Even after successful surgery, children with a coarctation of the aorta may have high blood pressure that is treated with medicine.

Cor triatriatum is a congenital heart defect where the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is subdivided by a thin membrane, resulting in three atrial chambers.

Treatment: Surgery may be performed through a cosmetic right mini axillary thoracotomy, and consists of removing the obstructing membrane.

Double chambered right ventricle is a congenital defect in which anomalous muscle bands divide the right ventricle into two chambers—a proximal high-pressure and distal low-pressure chamber. It may be associated with mid right ventricular obstruction, and is commonly associated with a ventricular septal defect (VSD).

Treatment: This defect should be treated surgically, because the obstruction is progressive and ends in heart failure. Surgery involves excising the obstructive muscle bundles in the right ventricle, and closing the VSD.

HLHS is a rare, complex, serious form of congenital heart disease in which the left ventricle (or chamber) of the heart is underdeveloped. As a result, HLHS is called a “single-ventricle heart defect.” Without treatment, the right chamber becomes overworked, which eventually leads to heart failure and the outcome is fatal in a matter of days to weeks.

Treatment: Multiple operations, known as staged palliation, are necessary to redirect blood flow through the heart. Often, cardiac catheterization is performed prior to each operation to assess the child's circulation. The three stages of staged palliation are:

• Stage 1: Norwood procedure. Performed in the first week of life, this procedure reconfigures the great vessels so that the right chamber can act as the main “pump” of blood to both the body and the lungs.
• Stage 2: Bidirectional cavo-pulmonary anastomosis. Sometimes known as the bidirectional Glenn procedure or the hemi-Fontan procedure, this is performed at around 3 to 6 months of age. It alters the patient’s circulation so that venous blood from the upper body supplies blood to the lungs. 
• Stage 3: Fontan procedure. Usually performed between the ages of 2 and 3 years, the Fontan procedure reroutes blood flow from the lower body to the lungs by creating a channel outside of the heart, completing the separation of the systemic and pulmonary blood supplies. This helps bring the child’s oxygen level to a more normal range

Interrupted aortic arch is a rare condition in which the aorta is incomplete or interrupted, preventing oxygenated blood from reaching the rest of the body. A newborn can survive with this disconnection as long as a blood vessel called the ductus arteriosus—an alternate route for oxygenated blood to reach the lower body—remains open. If the ductus arteriosus does not close after the baby is born, it is called a patent ductus arteriosus (PDA). After it closes (within hours or days of birth), an infant will quickly become very sick without medical intervention.

Treatment: Surgery involves connecting the two ends of the aorta, often with concomitant enlargement so that they form a complete and adequately sized arch.

Left outflow tract stenosis is narrowing that can be at, above or below the aortic valve, respectively ascending aorta and arch, or below the aortic valve in the left ventricle, with possible associated coronary anomalies. Rarely, multiple levels of narrowing are present in combination.

Treatment: Medication may help to reduce hypertension or control left ventricular hypertrophy, but treatment is surgical, and timing is lesion-related. Surgery consists of relief of narrowing and/or patch augmentation of the outflow to open up free flow of blood to the body.

PAPVR is a congenital heart defect in which some of the pulmonary veins carrying blood from the lungs to the heart flow into other blood vessels or into the heart's upper right chamber (atrium) instead of the upper left chamber. This causes oxygen-rich blood from the lungs to mix with oxygen-poor blood before entering the right atrium.

Treatment: Surgery is always needed and can often be done through a mini cosmetic right axillary thoracotomy incision. The surgeon will reconnect the pulmonary veins to the left atrium and close the hole between the atria, if there is one.

There's a hole in the septum (wall) that separates the upper chambers of the heart. The valve between the upper and lower left chambers (mitral valve) often also has a defect that causes it to leak (mitral valve regurgitation).

Treatment: Surgery involves closing the hole in the septum between the heart chambers with a patch and repairing the mitral valve. Often, this defect may be repaired through a mini cosmetic right axillary thoracotomy incision.

The ductus arteriosus is a connecting blood vessel between the two major arteries that usually closes shortly after birth. If it remains open, it's called a patent ductus arteriosus.

Treatment: Medication may be attempted in premature babies to close the ductus; if unsuccessful it can be closed with a device in the catheter laboratory called a Piccolo™. In full-term babies and children, treatment is with transcatheter device closure, or more rarely, surgical. Surgery consists of ligation or clip closure of the ductus arteriosus.

Right outflow tract stenosis is narrowing at or above the pulmonary valve (pulmonary artery 
stenosis), narrowing at the pulmonary valve, or narrowing below the pulmonary valve in the right ventricle. Specific diagnoses include pulmonary atresia with intact ventricular septum (PA-IVS), pulmonary atresia with ventricular septal defect and major aorto-pulmonary artery collaterals (PA-VSD-MAPCAs), and tetralogy of Fallot.

Treatment: This is surgical or catheter-based, and timing is lesion-related. Surgery consists of relief of narrowing and/or patch augmentation of the outflow.

A baby with a single ventricle anomaly is born with a heart that has only one ventricle that is large enough or strong enough to pump effectively.

Treatment: Two to three operations are necessary to separate the body (left) and lung (right) circulations, leaving the one good ventricle to support circulation, and the body veins to be rerouted directly toward the lungs. The planned operations are ideally completed by 2-4 years of age.

• Operation 1: Systemic-to-pulmonary artery shunt takes place between the first days and weeks of life. It provides the only source of blood to the lungs using a small Gore-Tex tube. Blood-thinning medication (aspirin) is given until the following operation. This may be part of the Norwood stage I operation for HLHS.
• Operation 2: Cavo-pulmonary anastomosis (Glenn operation) takes place between 3 and 8 months of age. The Gore-Tex tube is removed and a connection is made between the upper body vein (superior vena cava) and the pulmonary arteries. Occasionally, the defect between the two atria needs to be enlarged (septectomy, ASD enlargement) to allow for better mixing of saturated and desaturated blood. Aspirin is also required after this operation until the last operation.
• Operation 3: Total cavo-pulmonary connection (Fontan operation) takes place between 2 and 4 years of age. A connection is made between the lower body vein (inferior vena cava) and the pulmonary arteries, with the interposition of a large Gore-Tex tube. Increased blood pressure in the upper and lower body veins results in slight swelling of the face and the arms and can lead to prolonged drainage of fluid from the chest tubes after the operation. After this third operation, lifelong medication is required to keep the blood thin with aspirin.

Tetralogy of Fallot is a complex form of congenital heart disease that changes the normal flow of blood through the heart. It is the most common cyanotic condition in childhood. Because not enough blood is pumped to the lungs to be oxygenated, oxygen-poor blood flows to the body. Four main heart defects are associated with this disease:

• Ventricular septal defect (VSD), a hole in the septum between the right and left ventricles (lower chambers) of the heart. 
• Override of the aorta over the VSD, a defect in which the aorta is positioned over the VSD, rather than over the left ventricle.
• Right ventricular outflow tract obstruction (pulmonary stenosis), a defect in which the right ventricular outflow tract is narrowed at valvar, subvalvar and/or supravalvar levels.
• Right ventricular hypertrophy, a condition in which the heart muscle is thickened due to obstruction of blood flow to the lung.

Treatment: This usually requires at least one open-heart surgery before the first year of life. Lifelong surveillance is required due to the increased incidence of arrhythmia, exercise intolerance and reduced right ventricular function.

Periodic assessment is necessary to assess the health of the right ventricle. Later in life, some patients may require a pulmonary valve replacement. While usually performed surgically, pulmonary valve placement is becoming available in the cardiac catheterization laboratory by deployment of the Melody™ or Sapien valves.

Total anomalous pulmonary veins is a rare congenital malformation in which all four pulmonary veins do not connect normally to the left atrium. Without surgery, pulmonary hypertension develops within weeks to months, and heart failure worsens. It may be a surgical emergency within the first hours to days of life if the communicating vein closes. Beyond a few weeks, pulmonary hypertension may become irreversible, and surgery is no longer possible or only so with increased risk.

Treatment: This requires open heart surgery and is advisable to operate upon diagnosis. Surgery entails opening the confluence so that the veins can drain into the left atrium. An atrial septal defect and patent ductus arteriosus, which are commonly present, are also closed.

TGA, also known as transposition of the great vessels, is one of the most common cyanotic congenital cardiac conditions in infancy. It occurs when the aorta and pulmonary arteries are in the wrong position, which causes oxygen-rich blood to go back to the lungs and oxygen-deprived blood to go to the body. Since this disorder deprives the organs of much needed oxygen, it must be treated immediately with medication and/or intervention for the infant to survive. Effects of untreated TGA can include:

• Hypoxia, or insufficient oxygen flow to tissues. This typically leads to death unless there is some presence of oxygenated blood within the infant’s body.
• Heart failure, resulting from the right ventricle working in overdrive to deliver enough oxygen to the rest of the body.

Treatment: TGA requires surgery, usually within the first week of the baby’s life. Prior to surgery, it is often necessary to perform a transcatheter balloon atrial septostomy to allow oxygen-rich and oxygen-poor blood to mix better.

After the baby is stabilized, the surgical team completes the arterial switch operation where the two great arteries are switched to their proper positions coming from the heart, and the coronary arteries transferred to their correct position out of the left ventricle.
 

Truncus arteriosus is a rare type of congenital heart disease in which the truncus arteriosus, a single blood vessel, comes out of the right and left ventricles instead of the pulmonary artery and aorta. Without surgery, pulmonary hypertension rapidly develops (within weeks), and heart failure worsens. Beyond a few months, this may become irreversible and surgery is no longer possible or only so with increased risk.

Treatment: Medication can improve heart failure symptoms, but treatment is surgical and is advisable in the first days to weeks of life upon diagnosis. Surgery consists of disconnecting the pulmonary arteries from the aorta, creating a communication between the right ventricle and the pulmonary arteries (commonly with a biological valved conduit) and closing the VSD (patch).

The congenital problem vascular ring is an abnormal formation of the aorta, the large artery that carries blood from the heart to the rest of the body. The abnormal arrangement of blood vessels creates a ring around the esophagus and the trachea (windpipe). The most common symptoms are stridor (noisy breathing), wheezing, chronic cough, difficulties swallowing or repeat respiratory infections.

These symptoms are often encountered early in life. However, in some cases, the symptoms may not be realized or diagnosed until adolescence or adulthood. Varying degrees of symptoms are reported, depending on the type of vascular ring encountered.

Treatment: Surgery is required, and timing is dependent on the severity and type of lesion. It is generally recommended as of infancy before older childhood, even in asymptomatic patients, to avoid the development of persistent airway deformity from long-standing vascular compression. Surgery consists of dividing the vascular ring and releasing the compression on the esophagus and the trachea to relieve the symptoms.

Ventricular septal defect is a common structural heart disease that can be present at birth: a hole or defect in the septum (wall) that divides the two lower chambers of the heart (ventricles). In a fetus, the right and left ventricles of the heart are not separate.

Treatment: Untreated, large ventricular septal defects can cause heart failure and/or pulmonary hypertension, and therefore require surgical treatment. Closing the VSD is done to prevent serious problems later, and open-heart surgery may be required in infancy or childhood, even in patients with few symptoms, to prevent complications. Most defects may be repaired through a mini cosmetic right axillary thoracotomy incision.