Hypoplastic left heart

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Heart, front view

Heart, section through the middle

Hypoplastic left heart syndrome; HLHS

Initially, a newborn with hypoplastic left heart may appear normal. Symptoms usually occur in the first few hours of life, although it may take up to a few days to develop symptoms. These symptoms may include:

• Lethargy
• Poor suckling and feeding
• Shortness of breath
• Rapid breathing
• Cold extremities
• Enlarged liver
• Poor pulse
• Pounding heart
• Bluish or poor skin color
• Sudden death

In healthy newborns, bluish color occurs in hands and feet as a response to cold (this reaction is called peripheral cyanosis).

However, a bluish color observed in the chest or abdomen, lips, and tongue is abnormal (called central cyanosis) because it reflects lack of adequate levels of oxygen in the blood. This is secondary to the heart malformation and circulatory malfunction. Central cyanosis often increases with crying.

A physical exam may show signs of congestive heart failure, liver enlargement, rapid breathing, and lethargy. Additionally, the pulse at various locations (wrist, groin, and others) is usually very weak.

Tests may include:

• ECG shows enlargement of the right ventricle of the heart
• X-ray of the chest may show signs of other birth defects
• Echocardiogram
• Cardiac catheterization

Review Date: 05/30/2006
Reviewed By: Anne J. L. Chun, M.D., Assistant Professor of Pediatrics, Division of Pediatric Cardiology, New York University School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.