Hypoplastic Right Heart Syndrome (HRHS)
Hypoplastic right heart syndrome (HRHS) is a severe congenital heart defect in which the right side of the heart is underdeveloped or too small.
This means the right ventricle, the pulmonary artery and the tricuspid and pulmonary valves may be too small, and because of that, the right side of the heart cannot pump oxygen-rich blood to the body properly.
HRHS can be more serious for some people than others. Severe cases of HRHS can cause low oxygen levels, necessitating urgent treatment. Other people with HRHS will have milder issues, and may not be diagnosed until later in life.
What are the symptoms of HRHS?
What treatment options are there?
Treatment for HRHS may include medication, minimally invasive procedures or surgery. The goal of these treatments is to help blood get to your baby’s lungs so they can get more oxygen into it.
Medication
Prostaglandin can help keep a baby’s ductus arteriosus open. This is a blood vessel the fetus uses to get oxygen-rich blood from its birth mother. Usually, a ductus arteriosus closes a few days after birth, at which point most babies can get oxygen using their lungs. Keeping the ductus arteriosus open after birth can help a baby with HRHS get oxygen-rich blood to its lungs.
Minimally invasive procedures
Stent: This procedure uses a catheter to place a stent (small metal tube) inside a baby’s ductus arteriosus to keep it open.
Pulmonary valve perforation: In a pulmonary valve perforation, a catheter wire is threaded through a blood vessel to get to the pulmonary valve that doesn’t open as it should. Then they make a hole in the pulmonary valve to let blood flow through to the pulmonary artery. Some providers use ablation for this procedure.
Surgery
Surgery may be needed to treat severe cases of hypoplastic right heart syndrome.
Procedures to address HRHS include:
Blalock-Taussig-Thomas (BTT) shunt can establish blood flow to the lungs in a baby with an HRHS heart. It uses synthetic fabric to link their subclavian artery to their pulmonary artery.
Months or years later, a surgeon may perform a Glenn procedure and/or Fontan procedure. Each of these operations creates a new route for blood to flow around the right ventricle (lower heart chamber) that isn’t working well. A fully developed right ventricle can pump blood to your pulmonary artery.
The Glenn and Fontan procedures allow blood to flow from a major vein to your pulmonary artery. From there, blood flows to your lungs, where it can get oxygen.
