Pediatric and Congenital Heart Disease Session 2: Frontiers in Single Ventricle Heart Disease (2020)-My Worst Fontan Nightmares

Video Transcription

Video Summary

A 17-year-old girl with heterotaxy and complex Fontan anatomy had worsening cyanosis and exercise intolerance. Imaging and cath showed severe right-sided pulmonary AVMs caused by poor hepatic flow distribution. Using multimodality imaging and a biomedical engineering flow model, clinicians redesigned her Fontan so hepatic blood would reach both lungs more evenly. She improved markedly after surgery, but later developed conduit thrombosis and occlusion, requiring revision. After reoperation, her Fontan became patent again and saturations rose to 98%. The case highlights both the promise and pitfalls of innovative, patient-specific Fontan planning, plus the importance of teamwork and long-term follow-up.

Keywords

heterotaxy

Fontan anatomy

pulmonary arteriovenous malformations

hepatic flow distribution

conduit thrombosis