Advanced Pathophysiology for the Advanced Practice Nurse
Dr. Chovan and Dr. Butz
HLHS, Hypoplastic Left Heart Syndrome, Congenital Heart Disease, Congential Heart Defect
Medicine and Health Sciences | Nursing
Hypoplastic left heart syndrome (HLHS) is a severe and rare form of congenital heart disease with no known cause that decreases life expectancy and occurs during fetal cardiac development. Without treatment HLHS is fatal. HLHS consists of the underdevelopment of the left-sided heart structures leading to the failure of the left ventricle to support the systemic circulation resulting in single-ventricle physiology (Mussa & Barron, 2016). Prior to the 1980s HLHS was a universally lethal condition until 1982, when surgical management became available (Mussa & Barron, 2016). However, surgical management is not curative because the heart can never be “normal” (Mussa & Barron, 2016). HLHS can be diagnosed prenatally and postnatally. A prenatal diagnosis by ultrasound allows for parent preparedness, prognosis, possible outcomes, and planning of postnatal management (Mussa & Barron, 2016). Signs and symptoms of HLHS are present at birth or shortly after. Nurses are critical for providing care, assessing family needs, providing education and support. Lifelong routine follow-up visits with a cardiologist is recommended and ongoing genetic research continues to understand the cause of HLHS.
Carey, Stacey, "Hypoplastic Left Heart Syndrome" (2020). Nursing Student Class Projects (Formerly MSN). 431.