The heart has four chambers, a left atrium and right atrium and a left ventricle and right ventricle. Having a single ventricle defect means that only one ventricle works well enough to pump blood.
What Are the Signs & Symptoms of a Single Ventricle Defect?
A newborn with a single ventricle defect can have:
blue or grayish color of the skin and nails
lethargy (very little activity)
weak pulses in the arms and legs
few wet diapers
These signs can start a few hours to a day or so after the birth. Without treatment, the baby's blood pressure will fall too low to meet the body's needs. Usually, though, doctors find the problem before the baby's birth.
Most cases of single ventricle defects happen in the developing heart during the first 8 weeks of pregnancy. Some might be due to a combination of genes and things in the baby's and mother's environment during this early stage.
How Is a Single Ventricle Defect Diagnosed?
Doctors find most single ventricle defects during pregnancy by doing a fetal echocardiogram ("echo"). If not, they diagnose them shortly after a baby's birth with an echocardiogram. An echo is a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart.
Babies born with a single ventricle defect that wasn't found before birth may become very ill. The exact symptoms will depend on the defect causing the single ventricle.
How Is a Single Ventricle Defect Treated?
Single ventricle defects are treated by two or three surgeries. The first surgery varies, depending on the diagnosis. But the second and third surgeries are usually the same no matter what kind of heart defect a child has.
The goal of the surgeries is to rebuild the heart and make sure that it:
pumps blood out to the body
returns the blood to the lungs (without being pumped by the heart as it would be in a child with two normal ventricles)
All babies with a single ventricle will need care and surgery before they go home from the hospital after their birth. Then, they'll be enrolled in a single ventricle program, and an expert care team will monitor them very closely.