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[Hypoplastic left heart syndrome: 10 year experience with staged surgical management]

Gonzalo Urcelay1, Francisca Arancibia1, Javiera Retamal1

  • 1Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

Revista Chilena De Pediatria
|October 13, 2015
PubMed

Insights

Hypoplastic left heart syndrome (HLHS) treatment involves staged surgery, with outcomes similar to major centers. Restrictive atrial septal defects and diminutive aortas are key mortality risk factors.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Surgery
  • Neonatal Cardiac Surgery

Background:

  • Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect with high mortality without treatment.
  • Staged surgical palliation, starting with the Norwood operation, is the primary treatment for HLHS.
  • Long-term survival data for treated HLHS patients are crucial for understanding treatment efficacy.

Purpose of the Study:

  • To evaluate the institutional experience in managing HLHS patients from 2000 to 2012.
  • To identify significant risk factors associated with mortality in HLHS patients.
  • To assess the long-term survival rates following staged surgical management.

Main Methods:

  • Retrospective analysis of 76 HLHS patients treated at a single institution.
  • Review of clinical, surgical, and follow-up records.
  • Multivariate analysis to identify mortality risk factors.

Main Results:

  • Surgical mortality for the first stage of treatment was 23%, with a 21.3% mortality for the Norwood operation.
  • Actuarial survival rates were 64% at one year and 57% at five years.
  • Restrictive atrial septal defect and diminutive aorta were identified as significant risk factors for mortality.

Conclusions:

  • Staged surgical management of HLHS yields outcomes comparable to large centers.
  • A significant decrease in surgical mortality was observed in the latter half of the study period.
  • Identification of mortality risk factors aids in refining treatment strategies for HLHS.
Abstract

Keywords:
Cardiopatías congénitasCongenital heart diseasesHypoplastic left heart syndromeMortalidad quirúrgicaNorwood procedureOperación de NorwoodSurgical mortalitySíndrome de hipoplasia de corazón izquierdo

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