Pulmonary Arteriovenous Malformations in Heterotaxy Syndrome: The Case for Early, Direct Hepatic Vein–to–Azygos Vein Connection

Danielle S. Burstein, Constantine Mavroudis, Michael D. Puchalski, Robert D. Stewart, Carlos J. Blanco, Marshall L. Jacobs

Research output: Contribution to journalArticle

Abstract

We report a surgical approach using hepatic vein–to–azygos vein connection without cardiopulmonary bypass or deep hypothermic circulatory arrest in a patient with heterotaxy syndrome and interrupted inferior vena cava with persistence of pulmonary arteriovenous malformations (PAVMs) after previous Fontan completion. We advocate early performance of hepatic vein–to–azygos vein connection following the Kawashima operation for heterotaxy with functionally univentricular heart and interrupted inferior vena cava. We review the physiology of heterotaxy syndrome with congenital heart disease and justify our approach in the context of a review of previous surgical strategies used in this patient population.

Original languageEnglish (US)
Pages (from-to)119-128
Number of pages10
JournalWorld Journal for Pediatric and Congenital Heart Surgery
Volume2
Issue number1
DOIs
StatePublished - Jan 2011
Externally publishedYes

Keywords

  • CHD univentricular heart
  • Fontan
  • heterotaxy
  • pulmonary arteriovenous malformations

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Fingerprint Dive into the research topics of 'Pulmonary Arteriovenous Malformations in Heterotaxy Syndrome: The Case for Early, Direct Hepatic Vein–to–Azygos Vein Connection'. Together they form a unique fingerprint.

  • Cite this