Page 22 - Journal of Structural Heart Disease Volume 5, Issue 1

Case Report
Journal of Structural Heart Disease, February 2019, Volume 5, Issue 1:11-15
DOI: https://doi.org/10.12945/j.jshd.2019.017.18
Received: May 31, 2018 Accepted: July 03, 2018 Published online: February 2019
Transcatheter Revision of Fontan Circulation
by Connecting the Classical Glenn Circulation with the Inferior Vena Cava and Pulmonary
Artery Circulation
Vishal R. Kaley, MBBS, MD1, E. Oliver Aregullin, MD, FAAP1,2, Stephen C. Cook, MD, FACC1,2, Samuel J. Lacina, MD1,2, Bennett P. Samuel, MHA, BSN, RN1,
Joseph Vettukattil, MBBS, MD, DNB, CCST, FRCPCH, FRSM, FRCP1,2*
1 Congenital Heart Center, Spectrum Health Helen DeVos Children's Hospital, Grand Rapids, Michigan, USA
2 Pediatrics and Human Development, College of Human Medicine, Michigan State University, Grand Rapids, Michigan, USA
Abstract
Patients with single-ventricle physiology require staged palliation. Additional interventions may be re- quired due to Fontan failure, formation of collaterals or pulmonary arteriovenous malformations (PAVMs). Transcatheter interventions are preferable in this set- ting to avoid the risks of redo-sternotomy, and cardio- pulmonary bypass. We present our experience with transcatheter revision of Fontan circulation in a cyanot- ic adult congenital heart patient with hypoplastic left heart syndrome (HLHS). Transcatheter Fontan comple- tion may be a feasible option in patients with favorable anatomy and hemodynamics with optimal outcomes. Copyright © 2019 Science International Corp.
Key Words
Adult congenital heart disease • Hypoplastic left heart syndrome • Pulmonary arteriovenous malformations • Transcatheter intervention
Introduction
Surgical interventions in patients with single-ven- tricle physiology are challenging due to the need for staged palliation involving Norwood procedure fol- lowed by Glenn and Fontan surgery [1, 2]. Despite ad-
vancements in surgical technique, these patients are known to develop complications such as formation of collaterals or pulmonary arteriovenous malforma- tions (PAVMs), Fontan failure, and Fontan associated liver disease (FALD) [3-5]. Multiple transcatheter and surgical interventions may be required for treatment of these complications. Transcatheter interventions are preferable in Fontan patients to decrease the need for re-do sternotomy and cardiopulmonary by- pass (CPB). We present our experience with transcath- eter Fontan completion in a cyanotic adult congenital heart patient with hypoplastic left heart syndrome (HLHS).
Case Presentation
A 31-year-old woman with HLHS presented with cyanosis and decreasing exercise capacity to an adult congenital heart clinic at a tertiary medical center in the Midwest. She was referred to the congenital heart interventional team to evaluate underlying anatomy, hemodynamics, and source of cyanosis.
After HLHS was diagnosed in the early neona- tal period, she underwent stage-1 palliation. At 6 months of age, she was found to have a widely patent
* Corresponding Author:
Joseph Vettukattil, MBBS, MD, DNB, CCST, FRCPCH, FRSM, FRCP Congenital Cardiology
Spectrum Health Helen DeVos Children’s Hospital
100 Michigan NE (MC48), Grand Rapids, Michigan 49503, USA Tel. +1 616 267 0988; Fax: +1 616 267 1408;
E-Mail: joseph.vettukattil@helendevoschildrens.org
Fax +1 203 785 3346
E-Mail: jshd@scienceinternational.org http://structuralheartdisease.org/
© 2019 Journal of Structural Heart Disease Published by Science International Corp. ISSN 2326-4004
Accessible online at:
http://structuralheartdisease.org/