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Medical Edge Newspaper Column from Mayo Clinic

REPAIRING A NEWBORN’S DEFECTIVE HEART

DEAR MAYO CLINIC: I have a baby who was diagnosed with several congenital heart defects: tetralogy of Fallot, an RPA (right pulmonary artery) discontinuity and severe infundibular stenosis. What kind of treatment do you offer for this kind of condition? What are the outcomes? — United Arab Emirates
ANSWER:
It’s very stressful when your newborn is diagnosed with serious heart problems. But the good news is that an experienced pediatric cardiovascular surgeon can repair these defects — and your baby should only need one operation to fix all of them. Surgery is usually performed during the first year of life.

First, some background. Heart defects that are congenital (present at birth) occur in approximately one in 120 infants, affecting some 35,000 newborns and their families each year in the United States alone. Though the causes remain largely unknown, the risk of a baby’s having a congenital heart defect is enhanced under certain circumstances, such as when — during the first two months of pregnancy — the mother has rubella (German measles) or uncontrolled diabetes, or uses alcohol, cocaine or certain medications. Genetics may also play a role.

Tetralogy of Fallot is a set of four defects: 1) a hole in the wall separating the heart’s right and left ventricle; 2) an obstruction in the valve that ordinarily passes blood from the right ventricle into the lungs in order to oxygenate it; 3) a misplaced aorta; and 4) a thickening of the right ventricular wall.

Tetralogy of Fallot must be treated through surgery, though only the first two defects need be repaired. The most prominent symptom of tetralogy of Fallot is cyanosis (blue baby), caused by low levels of oxygen in the blood.

The surgical correction has two main purposes: to cover the hole and to eliminate the obstruction so that blood may flow more normally through the pulmonary artery into the lungs. Each of these objectives is accomplished most commonly through placement of a patch — in the first instance, with a cloth material that closes the hole between the ventricles; and in the second, with another patch so that the opening of the valve is enlarged. Efforts should be made to save the pulmonary valve, if possible.

Infundibular stenosis refers to a blockage of the right ventricle’s infundibulum, a doughnut-shaped, sphincter-like muscle that is abnormally thickened. It tends to tighten, thereby limiting blood flow into the lungs. This defect may be repaired in a variety of ways by enlarging the “doughnut’s” hole.

In RPA discontinuity repair, the surgeon reattaches the disconnected right pulmonary artery to the main pulmonary artery. Later intervention may be required to widen a narrowing at the reattachment site.

Generally speaking, once your baby undergoes the necessary surgical repairs, there is an excellent chance that his or her quality of life — and life span — will be close to normal. An additional operation — especially pulmonary-valve replacement — may be needed later in life in some 15 to 20 percent of patients. Consultation with a pediatric cardiologist will give you more information about your baby’s medical needs.

— Joseph A. Dearani, M.D., Cardiovascular Surgery, Mayo Clinic, Rochester, Minn.

Additional Resources:
Congenital Heart Defects
Appointment Information
Treatment of Congenital Heart Defects
Understanding Congenital Heart Defects

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Medical Edge from Mayo Clinic is an educational resource and doesn’t replace regular medical care. To e-mail a question, go to www.mayoclinic.org, or write: Medical Edge from Mayo Clinic, c/o TMS, 2225 Kenmore Ave., Suite 114, Buffalo, N.Y., 14207. For health information, visit www.mayoclinic.com.