Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart...

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect requiring staged surgical palliation.
• Persistent ventricular dysfunction after stage I palliation is a significant concern for HLHS patients.
• Identifying risk factors for adverse outcomes is critical for optimizing management.

Purpose of the Study:

• To describe the clinical course of HLHS patients with persistent ventricular dysfunction post-stage I palliation.
• To identify risk factors associated with death or need for transplantation before stage II palliation.
• To compare the management and outcomes of patients with and without ventricular dysfunction.

Main Methods:

• Retrospective review of 138 children undergoing stage I palliation between 2004 and 2011.
• Inclusion of 22 patients with persistent moderate ventricular dysfunction on serial echocardiograms.
• Case-matched comparison with controls without significant ventricular dysfunction.

Main Results:

• Patients with ventricular dysfunction had significantly higher rates of heart failure (HF) readmission (82% vs 10%) and required more intensive medical management (inotropes, ventilation, ECMO).
• Heart transplant-free survival at 7 months was lower in the dysfunction group (50.6% vs 90.9%).
• Extracorporeal membrane oxygenation (ECMO) support and prolonged inotropic therapy were significant predictors of death or transplantation before stage II.

Conclusions:

• Persistent ventricular dysfunction in HLHS patients is associated with increased morbidity, higher resource utilization, and poorer transplant-free survival.
• ECMO support and duration of inotropic therapy are critical risk factors for early mortality or transplantation.
• Alternative treatment strategies should be considered for patients requiring prolonged inotropic support.