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[Fontan procedure in patients with hypoplastic left heart syndrome]

Hajime Ichikawa1

  • 1Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Japan.

Kyobu Geka. the Japanese Journal of Thoracic Surgery
|August 7, 2013
PubMed

Insights

Improving Fontan completion rates for hypoplastic left heart syndrome (HLHS) requires addressing key issues like ventricular function and pulmonary resistance. Modern strategies focus on comprehensive palliation and surgical techniques for better long-term outcomes.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease Surgery
  • Cardiovascular Physiology

Context:

  • Hypoplastic left heart syndrome (HLHS) presents complex challenges in single-ventricle palliation.
  • While Fontan completion rates have improved, achieving optimal hemodynamics remains a significant hurdle.
  • Key goals for successful Fontan circulation include maintaining good ventricular function and low pulmonary vascular resistance.

Purpose:

  • To outline current strategies for optimizing Fontan circulation in HLHS patients.
  • To identify critical factors and potential complications impacting long-term Fontan outcomes.
  • To emphasize the importance of addressing specific anatomical and physiological issues pre-Fontan and post-operatively.

Summary:

  • Modern HLHS treatment involves staged palliation, including the Norwood procedure with RV-PA conduit.
  • Addressing residual arch obstruction, impaired right ventricular (RV) function, tricuspid regurgitation, and aorto-pulmonary collaterals is crucial.
  • Extracardiac total cavopulmonary connection (TCPC) is the preferred Fontan type, with fenestration considered if necessary.
  • Pre-Fontan coil embolization of aorto-pulmonary collateral arteries and meticulous post-operative follow-up are essential for managing complications like arrhythmia and protein-losing enteropathy (PLE).

Impact:

  • Improved surgical techniques and comprehensive management strategies enhance Fontan circulation outcomes in HLHS.
  • Early identification and intervention for potential complications can mitigate long-term risks.
  • This approach aims to improve the quality of life and long-term survival for patients with HLHS.

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