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Congenital Heart Disease (Pediatric)

What is congenital heart disease (CHD)?

CHD refers to a variety of heart conditions that are present at birth. They arise because the heart or the major blood vessels have not formed properly.

A normal heart has four chambers: two collect blood from the lungs and the rest of the body, and two pump the blood back into the body (once it has been oxygenated) and the lungs, so it can be oxygenated once more. When the heart is working correctly, blood flows into the right atrium (a collecting chamber) from the body. The right ventricle then pumps it out through the pulmonary artery and into the lungs to pick up oxygen. Next, it returns to the left atrium (another collecting chamber). Finally, it moves to the left ventricle which pumps it out through the aorta, delivering the oxygen to the organs.

Valves between the collecting chambers and the pumping chambers keep the blood moving in the right direction.

Problems with any of these structures, or with the vessels that return blood to the heart, can result in CHD. Immediate treatment is required when there isn't enough blood going to the lungs or being circulated to the body.

Our physicians and surgeons are known for achieving the best possible outcomes for these congenital heart conditions, as follows:

Coarctation of the Aorta

This is a narrowing or constriction of the aorta that blocks the blood flow to the lower part of the body. It also increases blood pressure above the constriction. The first signs are usually high blood pressure (often higher in the arms than legs.) 

Symptoms may appear at birth, but usually don’t manifest for days or weeks. In some cases, this condition is not apparent until late childhood.
Surgery may be needed if the child develops high blood pressure and congestive heart failure. 

Hypoplastic Left Heart Syndrome (HLHS)

This condition involves several abnormalities of the heart and the major blood vessels. In HLHS, most of the structures on the left side of the heart (left ventricle, mitral valve, aorta, and aortic valve) are small and underdeveloped.

The left ventricle pumps oxygenated blood to the rest of the body, but this important function is impaired in babies with HLHS. This congenital defect is typically fatal without treatment. Yet infants can be given a medication to keeps the fetal blood vessels open, Later on, they often need surgery to correct this structural problem.

Patent Ductus Arteriosus (PDA)

Before birth, all babies have a small passageway called the Ductus Arteriosus that connects the pulmonary artery and the aorta. At this point, the baby gets oxygen directly from the placenta, and the ductus arteriosus allows the blood to bypass the baby’s lungs. Usually the ductus arteriosus closes within a few hours or days after birth.

With premature babies, the ductus arteriosus may stay open much longer, leading to PDA. This condition interferes with a baby’s breathing, kidney and/or intestinal function.
A PDA can usually be treated with medication, yet some cases require surgery to close the passageway.

Tetralogy of Fallot 

This congenital heart defect consists of four different abnormalities:

  • Ventricular septal defect, an abnormal opening between the two ventricles of the heart.
  • Pulmonary stenosis, a narrowing at or under the pulmonary valve that partially blocks the flow of blood from the heart to the lungs.
  • Right ventricular hypertrophy, a condition where the right ventricle is overworked and the muscle becomes overdeveloped.
  • Overriding aorta, when the aorta lies directly over a ventricular septal defect. As a result, some unoxygenated blood goes to the body instead of going to the lungs. This may cause cyanosis (a blue tinge to the skin) and rapid breathing.

Reconstructive surgery for Tetralogy of Fallot may be done in the NICU, or later, in stages, once the child is bigger and stronger.