hypoplastic left heart syndrome
A severe congenital heart defect characterized by underdevelopment of the left-sided cardiac structures.
Full Definition
Hypoplastic left heart syndrome (HLHS) is a complex congenital heart disease where the left ventricle, mitral valve, aortic valve, and ascending aorta are severely underdeveloped or absent. This condition is incompatible with life without immediate intervention, as systemic circulation depends entirely on the right ventricle and ductal flow. Treatment options include staged surgical palliation (Norwood procedure series) or heart transplantation. The condition requires immediate prostaglandin infusion to maintain ductal patency and careful balance of pulmonary and systemic circulations.
Usage
Usage note: HLHS is acceptable after first use. Critical to distinguish from other forms of left heart obstruction.
In Context
- "The newborn with hypoplastic left heart syndrome was started on prostaglandin infusion and prepared for stage I palliation." — Cardiac ICU admission note
- "HLHS patients require careful monitoring of systemic oxygen saturations and avoiding excessive pulmonary blood flow." — Cardiothoracic surgery consultation