Journal article

Heterotaxy Adverse Long-Term Outcomes After Fontan Completion

Supreet P Marathe, Diana Zannino, Jacob Y Cao, Karin du Plessis, Shilpa S Marathe, Julian Ayer, David S Celermajer, Thomas L Gentles, Gary F Sholler, Robert N Justo, Nelson Alphonso, Yves d'Udekem, David S Winlaw

The Annals of Thoracic Surgery | ELSEVIER SCIENCE INC | Published : 2020


BACKGROUND: Heterotaxy is considered a risk factor for poor outcomes after the Fontan operation. However, long-term data to support this notion are lacking. The aims of this study were to ascertain the long-term outcomes of patients with heterotaxy after hospital discharge after Fontan completion and to compare these outcomes with those of a contemporary nonheterotaxy cohort. METHODS: A binational Fontan registry (n = 1540) was analyzed to identify patients with heterotaxy and compare them with patients without heterotaxy. The primary composite end point was Fontan failure, encompassing death, heart transplantation, Fontan takedown or conversion, protein-losing enteropathy, plastic bronchiti..

View full abstract


Awarded by National Health and Medical Research Council (NHMRC) partnership grant

Funding Acknowledgements

This work was supported in part by (1) the Wishaw Trust, Heart Centre for Children, The Children's Hospital at Westmead; and (2) National Health and Medical Research Council (NHMRC) partnership grant (1076849). The authors thank their research assistants for their support in maintaining the Australian and New Zealand Fontan Registry and the support provided to the Murdoch Children's Research Institute by the Victorian Government's Operational Infrastructure Support Program. Prof d'Udekem is a Clinician Practitioner Fellow of the NHMRC (1082186).