HLHS
Pronunciation: H-L-H-S
Also written as: HLHS — Hypoplastic Left Heart Syndrome
Hypoplastic left heart syndrome - severe congenital heart defect with underdeveloped left-sided cardiac structures.
Full Definition
Hypoplastic left heart syndrome (HLHS) is a complex and severe congenital heart defect characterized by underdevelopment of the left-sided cardiac structures, including the left ventricle, mitral valve, aortic valve, and ascending aorta. This condition accounts for 2-3% of all congenital heart disease but represents one of the most challenging lesions requiring immediate intervention in the neonatal period. The right ventricle must support both systemic and pulmonary circulation through the patent ductus arteriosus. Without surgical intervention, HLHS is universally fatal within the first few weeks of life. Treatment involves a series of staged surgical procedures (Norwood, Glenn, and Fontan operations) to establish single-ventricle physiology with the right ventricle supporting systemic circulation.
Usage
Usage note: Specify anatomical variants when known (mitral/aortic atresia vs stenosis). Requires immediate neonatal cardiac intervention.
In Context
- "Echocardiography confirmed HLHS with mitral atresia and severe hypoplasia of the left ventricle." — Cardiology consultation
- "The autopsy findings were consistent with HLHS showing a diminutive left ventricle and aortic arch hypoplasia." — Cardiac pathology report