Long-term results of biventricular repair after initial Giessen hybrid approach for hypoplastic left heart variants

J Thorac Cardiovasc Surg. 2015 Apr;149(4):1112-20; discussion 1120-2.e2. doi: 10.1016/j.jtcvs.2014.09.028. Epub 2014 Sep 18.

Abstract

Objective: This article presents the long-term results of our patients with a diagnosis of hypoplastic left heart syndrome (HLHS), hypoplastic left heart complex (HLHC), and variants who received a biventricular repair following hybrid stage I with ductal stenting and bilateral pulmonary artery banding.

Methods: Between June 1998 and June 2013, a total of 154 patients with hypoplastic left heart structures underwent a hybrid stage I procedure. Forty patients were definitely treated by creating a biventricular circulation. Median age and body weight of patients before hybrid stage I were 8.5 days (2-40) and 3.0 kg (1.6-3.8), respectively. The diagnoses were HLHS with mitral and aortic stenosis (n = 7), HLHC (n = 15), HLHC with interrupted aortic arch (n = 9), critical aortic stenosis with hypoplastic aortic arch (n = 4), imbalanced atrioventricular septal defect with hypoplastic aortic arch (n = 2), double-outlet right ventricle with hypoplastic aortic arch (n = 2), and d-transposition of the great arteries with interrupted aortic arch (n = 1). Median age at the time of biventricular correction was 6.7 months (1.6-13.8). The patients were treated with direct biventricular correction, including repair of intracardiac defects (n = 32), Norwood/Rastelli or Yasui (n = 4), arterial switch (n = 2), Rastelli (n = 1), and Ross-Konno (n = 1) operations with ascending aortic/aortic arch reconstruction.

Results: All patients survived hybrid stage I. Median survival after biventricular correction is 7.9 years (0.9-14.9). Overall mortality was 10% (4 patients) at 4 weeks, 5 weeks, 6 weeks, and 4 months after biventricular correction, respectively. One patient had to be switched to univentricular circulation and another patient underwent orthotopic heart transplantation 3 and 4 months after biventricular correction, respectively.

Conclusions: The Giessen hybrid approach is an alternative to the conventional strategy to treat neonates with HLHS, HLHC, and variants. Biventricular repair after hybrid stage I is feasible and can be performed with satisfactory long-term survival.

MeSH terms

  • Cardiac Catheterization* / adverse effects
  • Cardiac Catheterization* / instrumentation
  • Cardiac Catheterization* / mortality
  • Feasibility Studies
  • Heart Ventricles / abnormalities
  • Heart Ventricles / growth & development
  • Heart Ventricles / surgery*
  • Humans
  • Hypoplastic Left Heart Syndrome / diagnosis
  • Hypoplastic Left Heart Syndrome / mortality
  • Hypoplastic Left Heart Syndrome / physiopathology
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant
  • Infant, Newborn
  • Kaplan-Meier Estimate
  • Norwood Procedures* / adverse effects
  • Norwood Procedures* / mortality
  • Palliative Care
  • Pulmonary Artery / physiopathology
  • Pulmonary Artery / surgery*
  • Retrospective Studies
  • Risk Factors
  • Stents
  • Time Factors
  • Treatment Outcome
  • Ventricular Function, Left