HYPOPLASTIC LEFT HEART SYNDROME (HLHS)
Hypoplastic left heart syndrome (HLHS) is the most complex and highest-risk congenital heart defect, but is a rare condition, and even the largest children’s hospitals have only a small number of HLHS patients. This heart defect is fatal without early intervention.
A small percentage of children with HLHS receive heart transplants, while the large majority receive a series of three cardiac surgeries to survive:
Stage I (Norwood) procedure occurs within a few days of birth
Stage II (Glenn) typically occurs within 4-6 months of birth
Stage III (Fontan) procedure occurs between 2-4 years of age
Some children do not survive to undergo any surgical procedure. HLHS patients that do survive commonly need home tube feedings, and have frequent outpatient office visits and unplanned hospital readmissions.
NPC-QIC enrolls over 95% of patients with HLHS cared for during the interstage period across participating care centers.