Hypoplastic Left Heart Syndrome, or HLHS is a rare and complex congenital heart defect where the left side of a baby's heart is underdeveloped, affecting blood flow and making it difficult for the heart to pump oxygenated blood to the body. If not treated, HLHS is life-threatening. At CHRISTUS Health, our team of pediatric cardiologists, surgeons, and specialists are here to support you and your child every step of the way. Through a series of surgeries and ongoing care, we are committed to helping your child live a full, healthy life.
The most common signs and symptoms of hypoplastic left syndrome include:
It is important to note that some newborns may not display any signs or symptoms of hypoplastic left heart syndrome at birth, but may appear later on.
Risk factors for hypoplastic left heart syndrome include:
DiagnosisHypoplastic Left Heart Syndrome is usually diagnosed by ultrasound during pregnancy or shortly after birth. Imaging studies such as echocardiography, MRI, CT scans, and X-rays may be used to confirm the diagnosis and assess the severity of the condition.
Laboratory tests may detect any associated genetic abnormalities or chromosomal anomalies.
A physician will conduct a thorough physical examination, including a review of the baby’s respiratory and cardiac function, to determine the presence of other underlying problems.
Genetic testing may be performed to confirm the diagnosis and additional tests may be necessary to assess the severity of the condition and determine a treatment plan.
A pediatric cardiothoracic surgeon can perform the Norwood procedure within days or weeks after a baby is born.
This surgery allows the right ventricle to do the work of the left ventricle and pump blood into the body instead of toward the lungs.
A new aorta is constructed from part of the pulmonary artery and directs both oxygenated and deoxygenated blood into the body.
Additionally, a specialized pathway called a shunt is created to send blood directly to the lungs.
This procedure acts as a temporary solution while babies gain strength and prepare for another surgery, typically performed at around six months of age.
A second surgery, performed at around six months of age, removes the shunt and creates a direct pathway from the upper body to the lungs.
The surgeon disconnects the superior vena cava, a large vein that carries blood from the head and arms to the heart, from the heart and attaching it directly to the pulmonary artery.
The rerouting allows oxygenated blood to go straight into the lungs, giving the heart a rest by bypassing its delivery of blood to both body and lungs.
Between 18 and 36 months of age, the Fontan procedure is performed in which the cardiothoracic surgeon disconnects the inferior vena cava, a large vein that carries deoxygenated blood from the legs and abdomen to the heart, and connects it to the pulmonary artery.
Following the procedure, oxygen-depleted blood from the upper and lower body will now flow directly into the lungs. To help regulate overall blood flow, surgeons also create a small hole to allow for some of the blood to return to the heart.
Oxygenated blood is then pumped to the rest of the body through a single actively functioning ventricle in the heart.
PreventionHypoplastic Left Heart Syndrome cannot be prevented. However, women who are pregnant should attend regular checkups with their doctor and maintain a healthy lifestyle.
A detailed family history from both the mother and father can help a physician determine the risk of a cardiovascular defect.
Finally, some doctors may suggest prenatal testing such as ultrasound or echocardiography to identify potential abnormalities in fetal development.
CHRISTUS Children's specializes in pediatric care for infants, children and adolescents as well as maternal and fetal care for women with high-risk and routine pregnancies. From birth to age 18, CHRISTUS Children’s provides comprehensive, coordinated care from a team of pediatric and maternal experts.
