What It Is
Complex heart defects that result in one of the heart's pumping chambers (ventricles) being underdeveloped are called single-ventricle defects. Each of these defects is relatively rare. They include such problems as tricuspid valve atresia, hypoplastic left-heart syndrome, hypoplastic right-heart syndrome (pulmonary atresia with intact ventricular septum), mitral valve atresia, and double-inlet ventricle. Other types of heart defects, such as atrioventricular canal defects or double outlet right ventricle, may be complicated by an underdeveloped ventricle.
Complex heart defects that result in one of the heart's pumping chambers (ventricles) being underdeveloped are called single-ventricle defects. Each of these defects is relatively rare. They include such problems as tricuspid valve atresia, hypoplastic left-heart syndrome, hypoplastic right-heart syndrome (pulmonary atresia with intact ventricular septum), mitral valve atresia, and double-inlet ventricle. Other types of heart defects, such as atrioventricular canal defects or double outlet right ventricle, may be complicated by an underdeveloped ventricle.
Surgical Treatment
Patients with single-ventricle defects often need multiple operations. These include shunts such as Blalock-Taussig (B-T) or Glenn, placing a band on the pulmonary artery, or the Fontan operation.
Patients with single-ventricle defects often need multiple operations. These include shunts such as Blalock-Taussig (B-T) or Glenn, placing a band on the pulmonary artery, or the Fontan operation.
The Fontan operation largely separates the heart into two circulations. This lets oxygen-poor blood go to the lungs and oxygen-rich blood go to the body. The Fontan operation substantially reduces the mixing of blue and red blood and produces a normal or near-normal oxygen supply to the body. It also reduces the risk of a stroke or other complications, and decreases the workload on the single ventricle. A Fontan operation can't be done if you have pulmonary hypertension (high blood pressure in the lungs)
Medical
Single-ventricle defects are among the most complex congenital heart problems known. If you have this defect, you'll need regular checkups and ongoing care all your life. Many people with single-ventricle defects require daily or multiple medications. This care is best given by a cardiologist who's very familiar with the anatomical complexities and complications that these patients have. This requires the expertise of a pediatric or an adult cardiologist specifically trained in congenital heart disease.
Single-ventricle defects are among the most complex congenital heart problems known. If you have this defect, you'll need regular checkups and ongoing care all your life. Many people with single-ventricle defects require daily or multiple medications. This care is best given by a cardiologist who's very familiar with the anatomical complexities and complications that these patients have. This requires the expertise of a pediatric or an adult cardiologist specifically trained in congenital heart disease.
You may need yearly checkups to monitor your health. This may mean that you require such tests as electrocardiogram (ECG), echocardiogram (ultrasound of the heart, including transesophageal echocardiograms), cardiac catheterization, Holter and arrhythmia event monitoring, and exercise stress-testing.
Activity Restrictions
You may need to limit your activity, particularly competitive sports. If you have decreased heart function or rhythm disturbances, you may need to limit your activity more. Your cardiologist will help you determine if you must limit activities.
You may need to limit your activity, particularly competitive sports. If you have decreased heart function or rhythm disturbances, you may need to limit your activity more. Your cardiologist will help you determine if you must limit activities.
Problems You May Have
Most patients with single-ventricle defects may have health problems. These include cyanosis (lower oxygen levels, causing blueness), lower energy and a higher risk of infections such as brain abscess or endocarditis (infection of the heart). These problems shorten the lives of some people.
Most patients with single-ventricle defects may have health problems. These include cyanosis (lower oxygen levels, causing blueness), lower energy and a higher risk of infections such as brain abscess or endocarditis (infection of the heart). These problems shorten the lives of some people.
If you've had surgery for a single-ventricle defect, you can live a relatively normal life. However, your ability to exercise vigorously will probably be reduced.
Several basic types of problems are most common in this group of people. These problems may relate to the person's age at the time of the operation and the type of surgery done. Potential problems include:
- Rhythm problems, generally fast heart rate (tachycardia, supraventricular tachycardia, atrial flutter) or slow heart rate.
- Fluid retention, particularly in the abdomen and lower extremities. Some adults may develop varicose veins after the operation.
- More risk of a weakening and failing heart muscle when there's only one ventricle.
- Blood clots inside the heart that may require anticoagulation therapy.
F ew reports exist, but some women have been able to conceive and carry a pregnancy to term after surgery. Be sure to consult your cardiologist and obstetrician before considering pregnancy.
congenital heart defects
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