Heart Health

Home |Health Centers |Heart Health |Pediatric Heart

Congenital Heart Disease

Also called: Congenital Cardiovascular Disease, Heart Birth Defect, Defects, Heart Defect, Congenital Heart Defect, Congenital Septal Heart Defect

- Summary
- About congenital heart disease
- Potential causes
- Types and differences
- Signs and symptoms
- About fetal heart development
- Diagnosis methods
- Treatment options
- Prospects for recovery
- Pediatric heart transplants
- Questions for your doctor

Reviewed By:
Fred Weiss, M.D., FAAP, FACC
Robert I. Hamby, M.D., FACC, FACP

Test Your Knowledge
-	Cardiac Anatomy Quiz
-	Children's Heart Health Quiz
Quizzes A-Z

Treatment options for congenital heart disease

Treatment for a congenital heart defect depends on a number of factors, including the type and severity of the defect, age of the patient and other related elements. Prescription medications may be the first line of treatment and can include:

Digoxin. Improves the function of the heart muscle.
Diuretics. Allow the body to remove excess fluid that builds up in heart failure.
Prostaglandin E1. Allows the ductus arteriosus to remain open. Injected intravenously, prostaglandin is used in babies who need more blood flow to the lungs, or in babies who have difficulty getting blood to their lower body. This is usually used to temporarily increase blood flow in the heart until an underlying congenital defect can be fixed surgically.
ACE inhibitors. Decrease the constriction of the arteries and allows the blood to flow more easily to the body.
Beta blockers. Slow the heart rate and lower blood pressure, which reduces the workload on the heart.
Inotropes. Often given through an intravenous (I.V.) line, these medications strengthen the heart’s contractions so that it can circulate more blood with each beat.
Indomethicin. Given to neonates who have a large PDA that is overworking the lungs and resulting in respiratory distress, especially in premature infants.

If medications are not effective, then a more invasive treatment may be necessary. Catheter-based procedures may be used in the following situations:

Aortic stenosis or pulmonary stenosis may be treated by inflating a balloon–tipped catheter inside the narrowed valve to create a larger opening.
Patent ductus arteriosus can be closed by placing a stainless steel coil or other device inside the vessel and allowing it to close.
Certain types of atrial septal defects can be closed by inserting a device or patch through a catheter and using it to cover the hole.
A balloon–tipped catheter that expands, opening the narrowed pulmonary artery, can treat narrowing stenosis of the pulmonary artery. If balloon dilation is not effective, then a wire mesh metal tube stent can be placed inside the narrow part and expanded to hold the vessel open.
Coarctation of the aorta can be treated through either balloon dilation or stenting.
Arterial-venous fistulas are abnormal blood vessels that can be closed with stainless steel coils.

If none of the catheter–based procedures were, or would be, effective, then surgery may be necessary. Surgery may either be palliative, which relieves symptoms but does not cause blood to flow normally, or reparative, which fixes the underlying problem. Examples of reparative surgery include:

Closing ventricular septal defects (VSDs) and atrial septal defects (ASDs) with sutures or a patch made from either artificial material or the patient’s own tissue.
Repairing the atrioventricular valves and closing the VSDs and/or ASDs associated with AV septal defect.
Widening the pulmonic valve and closing the VSD associated with Tetralogy of Fallot.
Switching the great arteries back to their normal position and repositioning the coronary arteries in children who have transposition of the great arteries.
Widening the narrowed aorta in children with coarctation of the aorta.

Examples of palliative surgery that is used to improve the patient’s condition, but does not completely repair the heart include:

Modified Blalock-Taussig procedure. A tube is inserted to connect the aorta to the pulmonary artery to increase blood flow to the lungs.

Pulmonary artery band. A band is placed around the pulmonary artery to decrease blood flow to the lungs.
Fontan procedure. A surgery that is performed when only one ventricle exists (single ventricle). The Fontan procedure allows deoxygenated blood to drain into the lungs by bypassing the heart. The single ventricle then pumps oxygenated blood to the body.