Treatment for Your Child’s Hypoplastic Ventricle: Stage II

In hypoplastic left heart syndrome (HLHS), blood returning from the lungs must flow through an opening in the wall between the atria. The right ventricle pumps the blood into the pulmonary artery and blood reaches the aorta through a patent ductus arteriosus.

Your child has a heart problem that includes a hypoplastic ventricle. This means that one of the ventricles is either too small or is absent. The most common treatment for this problem is heart surgery. This is often done in 3 stages. This sheet explains what is done during the second stage (stage II). The surgery cannot repair your child’s heart problem. But it can help relieve your child’s symptoms. It may also increase your child’s chances of living a more normal life. The doctor can answer your questions and tell you more as needed.

Front view cross section of heart showing atria on top and ventricles on bottom showing aorta, pulmonary artery, mitral valve, aortic valve, left atrium, left ventricle, right atrium, right ventricle, tricuspid valve, pulmonary valve, superior vena cava, and inferior vena cava. Arrows on right side of heart show oxygen-poor blood pumping to lungs. Arrows on left side of heart show oxygen-rich blood pumped to body.

Front view cross section of heart showing bidirectional Glenn procedure for hypoplastic left ventricle. Superior vena cava is connected to pulmonary artery. Arrows show blood flowing from left atrium to right ventricle and mixing with blood from inferior vena cava, then being pumped out aorta.

In a normal heart, oxygen-poor blood is pumped to the lungs from the right ventricle. Oxygen-rich blood is pumped to the body from the left ventricle.

The BDG procedure sends oxygen-poor blood returning from the upper body directly to the lungs. This helps decrease the workload of the single ventricle.

The goals of heart surgery for a hypoplastic ventricle

  • Stage I. Make the single working ventricle the main pumping chamber of the heart, providing blood to both the lungs and the body. 

  • Stage II. Decrease the workload of the single ventricle, and reduce the mixing of oxygen-poor and oxygen-rich blood. 

  • Stage III. Separate the circulation of blood in the heart so that there is very little mixing of oxygen-rich and oxygen-poor blood.

What are the risks and complications?

  • Abnormal heart rhythm

  • Problems in the lungs

  • Infection

  • Bleeding

  • Problems with the nervous system, such as seizure of stroke

  • Abnormal buildup of fluid around the heart or lungs

Stage II: The bidirectional Glenn (BDG) procedure

The second stage of the surgery is called the BDG procedure. It is most often done when a child is 3 to 6 months old. A hospital stay of 5 to 7 days may be needed. The procedure provides low-pressure blood flow to the lungs. And it helps reduce the workload of the single ventricle. During the surgery, the surgeon:

  • Separates the superior vena cava (SVC) from the heart. The SVC is the large blood vessel that brings oxygen-poor blood from the upper body to the right atrium. This blood vessel is cut from the right atrium. The end closest to the right atrium is closed off. The other end is sewn to the right pulmonary artery. This allows oxygen-poor blood from the upper body to flow directly to the lungs, without passing through the heart. There, it receives oxygen.

  • Removes any additional source of blood flow to the pulmonary artery. If a shunt, band, or other device was placed during a previous procedure to direct blood to the lungs, it is removed at this time.

  • Fixes any other abnormal structures.

When to call your child's healthcare provider

Call your child's healthcare provider right away if any of these occur:

  • Increased redness, draining, swelling, or bleeding at the incision site

  • Increased swelling of the head and neck

  • Cyanosis

  • Fever 100.4°F (38°C) or higher

  • Trouble feeding, poor appetite, or poor weight gain

  • Irritability

  • Belly pain

  • Tiredness

  • Shortness of breath or trouble breathing

  • Cough that won’t go away

  • Nausea or vomiting that continues

  • Irregular heartbeat

  • No noticeable improvement