Hypoplastic right heart syndrome is a congenital heart defect where the right ventricle fails to develop properly, causing immediate heart failure after birth. Diagnosis is made through echocardiograms, and treatment involves the Fontan procedure to bypass the right ventricle. Survival rates have improved, but heart function may deteriorate, leading to the need for a transplant.
Hypoplastic right heart syndrome is a congenital heart defect in which the right ventricle of the heart fails to grow and develop properly. The underdeveloped ventricle cannot hold the proper amount of blood being pumped from the right atrium, and because the muscle structure is poor, further problems occur as the heart attempts to pump blood to the pulmonary valve for transfer to the lungs. This condition requires immediate and emergency treatment, as the heart begins to fail almost immediately after birth. An inadequate blood supply to the lungs means poor return of oxygenated blood to the body, and the muscle in the ventricle will be easily exhausted.
Babies born with hypoplastic right heart syndrome tend to be cyanotic, turning blue shortly after birth. If the condition hasn’t been diagnosed previously, your obstetrician or pediatrician will hear a recognizable murmur with a stethoscope. An echocardiogram can then confirm the diagnosis. Some radiologists notice a significant difference in the size of the ventricles during routine prenatal ultrasounds. Unfortunately, not everyone does, and fetal echocardiograms are not routine, although they are significantly less invasive than other routine procedures, such as amniocentesis.
If this heart defect is diagnosed in a fetus, the parents are referred to a hospital with a pediatric cardiology and pediatric cardiothoracic surgery team. It is best if the baby is born in this tier 3 hospital, so cardiologists are on hand to evaluate and treat the newborn right away. If the mother does not live near a level 3 hospital, an induced birth or caesarean section can be arranged in advance if the mother cannot have a vaginal birth.
As with most heart problems, a hypoplastic ventricle poses no problems for the fetus, but after birth, when the baby’s body needs to supply its own oxygen, problems begin almost immediately. If the baby is born in a hospital that cannot support immediate care, the baby is immediately transferred to a tier 3 hospital. Pregnant women who do not live close to a tier 3 hospital, especially those planning a home birth should request a fetal echocardiogram, particularly if there is a family history of heart defects.
There is no way to repair a small right ventricle, although its effects can be addressed. Surgeons rely on the Fontan procedure to bypass the right ventricle. Fontan is divided into two procedures, Glenn shunt and Fontan completion; early Fontans were done in one step, but the subdivision of the procedure is more successful. In the first few days of life, your baby will also be given a temporary shunt, called a Blalock-Taussig shunt, which provides a temporary way for blood to get to the lungs.
In about three months, the baby undergoes Glenn’s shunt, in which the superior vena cava is attached to the pulmonary arteries. Sometime in the next three to five years, surgeons will complete the Fontan, which connects the inferior vena cava to the superior vena cava through an intracardiac diaphragm or tunnel, or through an extracardiac shunt. The result is a complete bypass of the right ventricle, so blood flows back from the body directly into the lungs.
Many surgeons now prefer extra-cardiac shunting because Fontan supplementation can then be performed on a warm, beating heart. It does not require heart-lung bypass and has a lower risk of inducing arrhythmias. Some surgeons still prefer the older intracardiac baffle, however, and parents should ask what type of Fontan surgeons tend to employ.
Most surgeons now predict a 15 to 30 year survival rate after Fontan. That doesn’t mean the baby will die at this time, however, and as heart function begins to deteriorate, the individual will be listed for transplant. Some parents opt for a transplant immediately after birth instead of the Fontan. Other parents feel that transplant technology still needs work and prefer to buy time to improve this technology by going the Fontan way.
The outlook for children with hypoplastic right heart syndrome is better now than it has ever been, and improvements in care and surgery have made a huge change. This birth defect used to be considered almost certainly fatal, but now many babies survive the initial surgeries and are able to pursue active, purposeful lives.
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