Hypoplastic Left Heart Syndrome
Book AppointmentHypoplastic left heart syndrome (HLHS) is a rare and complex congenital (present at birth) heart defect. In this disorder the left side of the heart is dangerously underdeveloped. The heart contains four chambers; two upper chambers called atria and two lower chambers called ventricles. The oxygen-poor blood enters the right side of the heart from the rest of the body, which is then pumped to the lungs to be oxygenated. The oxygen-rich blood returns to the left side of the heart and from there, pumped to the rest of the body. In a baby born with HLHS, the lower left chamber (left ventricle) is unable to pump the blood to the body as it is too small. Other parts of the heart that lay on the left side can also be underdeveloped. These include:
Mitral valve which controls the blood flow between the left atrium and left ventricle
Aortic valve which transport blood from the left ventricle into the aorta
Aorta, the largest artery of the blood, which supplies blood from the heart to the rest of the body
Therefore, during the first few days of life, the right side pumps the blood to the rest of the body and to the lungs. This is done through:
Ductus arteriosus which is a blood vessel that connects the aorta and the pulmonary artery (which carries blood from the heart to the lungs for oxygenation)
Foramen ovale which is a small opening between the right and left atria
Both of these connections normally close after birth. It is important to keep these connections open until the first surgical procedure. HLHS occurs during foetal development due to unknown reasons. It is more common in children who already have siblings with this disorder.
When to seek expert advise
HLHS is mostly diagnosed in babies during prenatal ultrasound. Nonetheless, you should seek immediate medical help from our doctors at the Children's Hospital Service Line at The Aga Khan University Hospital, the only internationally accredited hospital in Pakistan, if you notice the above mentioned symptoms in your child.
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Disclaimer
The information provided on our website is for educational purposes and not intended to be a substitute for medical advice, diagnosis or treatment. You should always seek the advice of your doctor or other healthcare professional provider.
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