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Single Ventricle Defects

Single ventricle defects are disorders affecting one lower chamber of the heart. The chamber may be smaller than usual, underdeveloped, or missing a valve.

The main three single ventricle defects are:

  • Hypoplastic left heart syndrome (HLHS): An underdeveloped left side of the heart. The aorta and left ventricle are too small, and the holes in the artery and septum did not properly mature and close.

  • Pulmonary atresia/Intact ventricular septum: The pulmonary valve does not exist. The only blood receiving oxygen is the blood diverted to the lungs through openings that normally close during development.

  • Tricuspid atresia: The tricuspid valve between two of the heart's chambers isn't formed. Instead, there's solid tissue between the chambers, which restricts blood flow and causes the right lower heart chamber (ventricle) to be underdeveloped.

What happens once you have single ventricle defects?

Hypoplastic left heart syndrome (HLHS): With hypoplastic left heart syndrome, the baby may appear ashen, have rapid and difficult breathing and have difficulty feeding. This heart defect is usually fatal within the first days or month of life unless it's treated.

Pulmonary atresia: In pulmonary atresia, the infant appears blue (cyanotic) because there's less oxygen in the blood. The only source of lung blood flow is the patent ductus arteriosus (PDA), an open passageway between the pulmonary artery and the aorta. If the PDA narrows or closes, the lung blood flow is reduced to critically low levels.

Tricuspid atresia: In this condition, a baby, child or adult with tricuspid atresia can't get enough oxygen through its body. Because of this abnormal circulation, the patient looks blue (cyanotic).

What causes single ventricle defects?

In most cases, the cause of single ventricle defects is not known. Some children can have other heart defects along with these.

What types of tests are used to diagnose single ventricle defects?

Single ventricle defects are diagnosed during pregnancy by a fetal echocardiogram or shortly after birth with a regular echocardiogram. An echocardiogram uses sound waves to build a series of pictures of the heart.

What types of treatments and procedures are used to treat single ventricle defects?

HLHS
HLHS is treated through several surgical procedures or a heart transplant. Until an operation is performed, the ductus is kept open by intravenous medication. These operations are done in several stages and need to be adapted to each unique patient. The goal is to allow the right ventricle to pump only oxygenated blood to the body so the body has a normal blood oxygen level.

Heart transplantation can provide the infant with a healthy heart. However, the infant will require life-long medications to prevent the body from rejecting the heart.

Pulmonary atresia
A temporary treatment for pulmonary atresia includes a drug to keep the PDA from closing. A surgeon can also create a shunt between the aorta and the pulmonary artery that may help the blood flow more easily to the lungs.

If the right ventricle stays too small to ever be a good pumping chamber, the surgeon can connect the body veins directly to the pulmonary arteries. The atrial defect also can be closed to relieve cyanosis. These surgeries are called the Glenn and Fontan procedures.

Tricuspid atresia
It may be necessary to create a shunt, to increase blood flow to the lungs. If there is too much blood flowing to the lungs, the child may need a different type of surgery, called pulmonary artery banding, to decrease blood flow.

Most children with tricuspid atresia can have surgery to allow their hearts to work more like normal. Connections are created between the body veins and the lung (pulmonary) arteries. These surgeries are called the Glenn and Fontan procedures.

What can I do to support me or my child's health with single ventricle defects?

Most single ventricle surgeries are performed in the first two to four years of life and don't require more surgery. Many people with single-ventricle defects require daily or multiple medications.

Children with single ventricle defects will require lifelong follow-up by a cardiologist for repeated checks of how their heart is working. They may be advised to limit their physical activities to their own endurance. They are also at increased risk of developing endocarditis. Ask your pediatric cardiologist about your child's need to take antibiotics before certain dental procedures to help prevent endocarditis.

Almost all adults with single ventricles have had at least one heart operation in childhood. Many have survived three to five decades after surgery and are doing well with good activity levels. You may need to take antibiotics before certain dental procedures to prevent endocarditis. You'll need regular checkups by cardiologists all your life.

There are some common problems for people with single ventricle defects:

  1. Fast heart rate (tachycardia, supraventricular tachycardia, atrial flutter) or slow heart rate (bradycardia).

  2. Fluid retention, particularly in the abdomen and lower extremities. Some adults may develop varicose veins after the operation.

  3. Failing heart muscle when there's only one ventricle.

  4. Blood clots inside the heart that may require anticoagulation therapy.

You may need to limit your activity, particularly competitive sports. Your cardiologist will help you determine if you must limit your activities.

Some women who have had a Fontan-type operation can conceive and safely carry a pregnancy. Talk to your cardiologist to find out the risks.

Why choose Presbyterian for single ventricle defects treatment?

Presbyterian Heart and Vascular Care has the only pediatric and congenital cardiothoracic surgery team in New Mexico. Their team uses some of the most advanced techniques to perform this procedure. These techniques can improve recovery time, reduce complications and provide a quicker return to daily activities.