Pulmonary Arteriovenous Malformations in Heterotaxy Syndrome: The Case for Early, Direct Hepatic Vein-to-Azygos Vein Connection
- Resource Type
- Authors
- Marshall L. Jacobs; Constantine Mavroudis; Michael D. Puchalski; Danielle S. Burstein; Robert D. Stewart; Carlos J. Blanco
- Source
- World journal for pediatriccongenital heart surgery. 2(1)
- Subject
- medicine.medical_specialty
Heart disease
business.industry
Context (language use)
General Medicine
medicine.disease
law.invention
Surgery
medicine.anatomical_structure
Heterotaxy Syndrome
law
Internal medicine
Pediatrics, Perinatology and Child Health
cardiovascular system
Cardiopulmonary bypass
Deep hypothermic circulatory arrest
medicine
Cardiology
cardiovascular diseases
Azygos vein
Cardiology and Cardiovascular Medicine
Vein
business
Heterotaxy
- Language
- ISSN
- 2150-1351
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.