What are Single Ventricle Defects?

Single ventricle defects are heart problems that a person was born with (congenital heart defects). The heart is made up of four chambers. The upper chambers are called the right atrium and left atrium and the lower chambers are called the right ventricle and left ventricle. The right ventricle pumps blood to the lungs in order to get oxygen. After blood returns from the lungs, the left ventricle pumps it out to the rest of the body.

There are many different forms of single ventricle defects. They can involve one or more valves in your heart and a ventricle that does not pump effectively. In almost all cases, a single ventricle defect means that blood flow to your body or to your lungs is less than it should be. Some common types of single ventricle defects include:

• Hypoplastic left heart syndrome. This means that the left side of the heart has not developed enough.
• Pulmonary atresia. This is when there is no pulmonary valve. This valve separates the right ventricle from a pathway that leads to the lungs.
• Tricuspid atresia. This is when there is no tricuspid valve. This valve separates the right atrium from the right ventricle.

What are the symptoms of single ventricle defects?

You may continue to have some symptoms of your heart defect as an adult even if you had surgery to correct your heart defect as an infant or child. Your symptoms depend on the type of defect you were born with. If you have had surgery, the success of your surgery also makes a difference. Your symptoms may include:

• Bluish skin and lips (cyanosis), especially after exercise.
• Low energy.
• Frequent infections.
• A heart rate that is too fast (tachycardia) or too slow (bradycardia).
• Fluid buildup in your abdomen or legs (edema).
• Shortness of breath, especially when exercising.

What complications am I at risk for if I was born with a single ventricle defect?

You may be at risk for some problems that often affect adults who were born with a congenital heart defect. These may include:

• Heart failure.
• Heart rhythm problems (arrhythmias).
• Heart infections (endocarditis).
• Liver problems.
• Leaky heart valves.

How do I know if I am developing complications from my single ventricle defect?

It is important to have regular checkups with your heart specialist (cardiologist) to make sure you are not developing any complications. Your cardiologist will ask if you have any new symptoms and will do a physical exam. You may also have other tests, such as:

• Electrocardiogram (EKG) to check for arrhythmias.
• EKG during exercise (stress test).
• Echocardiogram to visualize your heart and check for size, shape, and functional changes.
• Blood tests to check your liver function.
• Using a portable type of EKG that you wear over a period of time (Holter or event monitoring).
• Checking the blood flow through your heart and coronary arteries. This is done by:
  • Inserting a long thin tube (catheter) through a vein in your arm or groin.
  • Injecting dye into the blood that is entering your heart. This dye passes through your heart and coronary arteries (cardiac catheterization).
  • The dye is then observed by your cardiologist on an X-ray machine.

What treatments can I expect?

Treatment may include:

• Medicines:
  • To control your heart rate.
  • To control your heart’s ability to pump blood throughout your body.
  • To control your blood pressure.
  • To prevent blood clots from forming in your heart.
• A pacemaker to regulate your heart rate.
• Surgery to repair or replace a leaky heart valve.
• A heart transplant if heart failure develops. This is rare.

What instructions should I follow?

Lifestyle

• Exercise as told by your health care provider. You may need to limit strenuous exercise, such as weight lifting or competitive sports.
• Eat a heart-healthy diet and maintain a healthy weight. Work with a diet specialist (dietitian) if necessary.
• Do not use any products that contain nicotine or tobacco, such as cigarettes and e-cigarettes. If you need help quitting, ask your health care provider.
• Do not abuse drugs or alcohol.
• If you are a woman and you become pregnant or want to become pregnant, you will need care from a cardiologist and a pregnancy specialist (obstetrician), both of whom should be familiar with managing heart defects during pregnancy.

General instructions

• Keep a record of the medical information to share with your health care providers. These may include:
  • What type of single ventricle defect you were born with.
  • What surgeries you have had.
  • What medicines you are taking.
• Ask your health care provider if you need to take antibiotic medicines before certain procedures.
• Take over-the-counter and prescription medicines only as told by your health care provider.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

• You have weakness or fatigue.
• You have swelling in your abdomen, legs, or ankles.
• You gain weight unexpectedly.
• You have shortness of breath during exercise or activity.
• You are a woman and you become pregnant or want to become pregnant.

Get help right away if:

• You have chest pain or trouble breathing.

Summary

• Single ventricle defects are heart problems that a person was born with (congenital heart defects).
• You may continue to have some symptoms of your heart defect even if you had surgery to correct the defect.
• Treatment may include medicines, pacemaker, or surgery.