Journal article

Long-term Outcomes of the Fontan Operation in Patients With Total Anomalous Pulmonary Venous Drainage

Matthew S Yong, Michael ZL Zhu, Karin du Plessis, Robert G Weintraub, Tim Hornung, David Winlaw, Nelson Alphonso, Yves d'Udekem, Igor E Konstantinov

The Annals of Thoracic Surgery | ELSEVIER SCIENCE INC | Published : 2019


BACKGROUND: Few patients with total anomalous pulmonary venous drainage (TAPVD) and a univentricular circulation survive to Fontan completion. Hence, we sought to determine the long-term outcomes of the Fontan operation in patients with TAPVD. METHODS: Patients with TAPVD who underwent the Fontan operation and survived to hospital discharge in Australia and New Zealand between 1985 to 2017 were identified (n=54) from a bi-national Fontan registry. RESULTS: Thirty-two (60%) patients underwent repair of TAPVD at a median age of 0.8 years (interquartile range: 0.3, 1.6 years). Thirty-seven (69%) patients had heterotaxy. The median age at time of Fontan operation was 5.7 years. There were 4 late..

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Awarded by National Health and Medical Research Council (NHMRC)

Funding Acknowledgements

The authors wish to thank their research assistants for their support in maintaining the Australian and New Zealand Fontan Registry, and also acknowledge the support provided to the Murdoch Children's Research Institute by the Victorian Government's Operational Infrastructure Support Program. Matthew Yong is supported by the David B. Rosenthal Scholarship, Australian Government Research Training Program Scholarship and National Health and Medical Research Council (NHMRC) Postgraduate Scholarship (APP1133977). Yves d'Udekem is a Career Development Fellow of the National Heart Foundation of Australia (CR 10M 5339), and Clinician Practitioner Fellow of the NHMRC (1082186).