Truncus Arteriosus

What It Is
Truncus arteriosus is characterized by a large ventricular septal defect over which a large, single great vessel (truncus) arises. This single great vessel carries blood both to the body and to the lungs.

Surgical Treatment
Surgery is required to close the ventricular septal defect and separate blood flow to the body from blood flow to the lungs. This is generally done early in infancy to prevent high blood pressure from damaging the lungs' arteries. A patch is used to close the ventricular defect. The pulmonary arteries are then disconnected from the single great vessel and a tube (a conduit or tunnel) is placed from the right ventricle to these pulmonary arteries. This is sometimes called a Rastelli repair.

Medical
Patients with truncus arteriosus need regular follow-up with a pediatric or adult cardiologist with special training in congenital heart disease. You may need to take medicine after your operation to help your heart pump better. Your cardiologist will track you with a variety of tests. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms to determine when another procedure such as cardiac catheterization may be needed.

Activity Restrictions
If you have decreased heart function or rhythm disturbances, you may need to limit your activity. Your cardiologist will help determine what is necessary.

Problems You May Have

Heart Function
In the long-term period after the operation, the heart muscle's ability to contract may decrease. You may need medication including diuretics, agents to help your heart pump better and drugs to control your blood pressure.

Heart Rhythm Disturbances
Patients with repaired truncus arteriosus have a higher risk for heart rhythm disturbances, called arrhythmias. These arrhythmias can originate from the atria (the heart's two upper chambers) or the ventricles (the two lower chambers). Sometimes they may cause dizziness or fainting. Medication may be required to control them. In rare cases, a procedure in the cardiac catheterization laboratory or the operating room may be required to eliminate these arrhythmias and control symptoms.

High Blood Pressure in the Lungs (Pulmonary Hypertension)
Sometimes, even when the defect is repaired early, the pulmonary hypertension becomes progressively worse. You may experience shortness of breath, decreased exercise endurance and sometimes headaches and dizziness.

Pregnancy
Women with repaired truncus arteriosus may handle pregnancy well. However, some may not.

Tags: congenital heart defects, Truncus Arteriosus, heart disease, congenital heart disease

Single-Ventricle Defects

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.

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.

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.

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.

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.

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:

1. Rhythm problems, generally fast heart rate (tachycardia, supraventricular tachycardia, atrial flutter) or slow heart rate.
2. Fluid retention, particularly in the abdomen and lower extremities. Some adults may develop varicose veins after the operation.
3. More risk of a weakening and failing heart muscle when there's only one ventricle.
4. 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