Heart Disease - Treating Congenital Heart Defects

Treatment of congenital heart defects/valve disorders depends on the defect and its severity. Treatment typically consists of drug therapy and/or surgery.

Drug therapy

The following drugs may be prescribed to help treat congenital heart defects. Brand names of drugs are shown in parentheses.

Other drugs used to treat heart defects include:

• Digoxin: improves myocardium (heart muscle) function
• Prostaglandin: keeps the ductus arteriosus (channel between aorta and pulmonary artery) open.
• Indomethacin: treats inflammation; may be given to premature babies to close the ductus arteriosus.

Medical Procedures

Depending on the type of congenital heart defect, any of the following procedures may be performed:

• Coronary angioplasty: Performed in a cardiac catheterization laboratory, this procedure involves placing a catheter with a small balloon on its tip into the patient’s narrowed artery under angiographic guidance. When properly positioned, the balloon is inflated and deflated, moving the plaque build-up further against the artery wall and thereby improving the flow of blood. This procedure may also be called percutaneous transluminal coronary angioplasty (PCTA), coronary artery balloon dilation or balloon angioplasty. Coronary angioplasty may be followed by stenting, a procedure in which a stent (expandable wire mesh tube) is permanently inserted into the artery to keep it open and restore normal blood flow.
• Arterial switch: Surgery to correct the transposition of the great arteries, the arota and pulmonary artery. The procedure involves cutting the end of the pulmonary artery from the left ventricle and sewing it to the right ventricle, and then cutting the end of the aorta from the right ventricle and sewing it to the left ventricle. Arterial switch is typically done within the first weeks after birth. Additional surgeries to correct a large ventricular septal defect or other defects may be necessary. Post-surgical patients should be monitored for life for potential complications.
• Balloon atrial septostomy: A procedure used during heart catheterization to improve oxygen supply in patients with transposition of the great arteries. Involves enlarging the atrial opening and reducing cyanosis.
• Balloon valvuloplasty: Insertion of a catheter into a narrowed valve. After the catheter is inserted, the balloon on its tip is inflated to open the valve. Balloon valvuloplasty improves blood flow in patient’s with pulmonary stenosis and has recently been used in cases of aortic stenosis.
• Damus-Kaye-Stansel procedure: Surgery to correct the transposition of the great arteries by dividing the pulmonary artery in two, and attaching the proximal section to the ascending aorta and connecting the distal section to the right ventricle.
• Fontan procedure: Surgery to bypass the underdeveloped right ventricle, to correct tricuspid atresia or pulmonary atresia. The procedure involves connecting the right atrium directly to the pulmonary artery, or using a conduit to make the connection. An atrial defect should also be closed to reduce cyanosis.
• Pulmonary artery banding: Surgery to treat a ventricular septal defect, atrioventricular canal defect, or tricuspid atresia. The procedure involves placing a band around the pulmonary artery to narrow it and reduce the blood flow and high pressure in the lungs. The band should be removed at a later date and the defect(s) should be treated with additional surgery.
• Ross procedure: Surgery to replace a defective aortic valve with the patient’s pulmonic valve and then replace the removed pulmonic valve with a donor’s valve. In some instances, the aortic valve will be replaced with a donor’s valve and the patient’s pulmonic valve is left alone. The procedure has a high success rate, though patients must be monitored for life and may need antibiotics to prevent endocarditis (inflammation of the endocardium).
• Shunting: Surgery to develop a passage between blood vessels to divert blood from one part of the body to another. The procedure may be used to reduce cyanosis in patients with tricuspid atresia, pulmonary atresia or severe cases of tetraology of fallot. One type of shunting procedure is the Blalock-Taussig, which involves creating a shunt from the aorta to the pulmonary artery.
• Venous switch or intra-atrial baffle: Surgery to create a tunnel inside the atria in patients with transposition of the great arteries. The procedure involves redirecting oxygen-rich blood to the right ventricle and aorta and redirecting venous blood to the left ventricle and pulmonary artery. Examples of this type of surgery include the Mustard procedure (the intra-atrial baffle is made of tissue from the pericardium) and the Senning procedure (the intra-atrial baffle is made of flaps from the atrial wall).

Updated: August 2006