Medicine (RMH) - Research Publications

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Author: D'Udekem D'Acoz, Yves × Author: Grigg, Leeanne × Author: Iyengar, Ajay × End date: 2022 ×

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    Pre- and Post-operative determinants of transplantation-free survival after Fontan. The Australia and New Zealand experience
    Poh, CL ; Cordina, RL ; Iyengar, AJ ; Zannino, D ; Grigg, LE ; Wheaton, GR ; Bullock, A ; Ayer, J ; Alphonso, N ; Gentles, TL ; Celermajer, DS ; D'Udekem, Y (ELSEVIER IRELAND LTD, 2021-08)
    BACKGROUND: This review identifies the predictors of late mortality and heart transplantation that remain relevant in the contemporary population of patients with a Fontan circulation, focusing on the potential impact of post-Fontan morbidities on the late outlook of these patients. METHODS AND RESULTS: A total of 1561 patients who had survived the Fontan operation in Australia or New Zealand from 1975 to 2018 were included in this review. Over a median duration of 11.4 years, there was a total of 117 deaths (7%) and 32 heart transplantations (2%). Freedom from death and heart transplantation at 10, 20 and 35 years post Fontan surgery were 94% (95% CI 93-95%), 87% (95 %CI 85-90%) and 66% (95 %CI 57-78%) respectively. Being male, having an atriopulmonary Fontan, pre-Fontan atrioventricular valve intervention, or prolonged pleural effusions post Fontan were predictive of late death or heart transplantation. However, time-dependent variables such as the development of atrial arrhythmia, protein/losing enteropathy or late ventricular dysfunction were stronger predictors of the same outcome. Patients who developed a time-dependent risk factor had a freedom from death and heart transplantation rate of 54% (95 %CI 43-66) at 15 years and 44% (95 %CI 33-57) at 25 years post Fontan. However, 95% (95 %CI 91-99) of patients without any of the identified risk factors were free from death or heart transplantation rate at 25 years post Fontan. CONCLUSION: In conclusion, the occurrence of post-operative complications such as PLE, arrhythmia and ventricular dysfunction will likely precede the late demise of these patients.
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    Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position Statement
    Zentner, D ; Celermajer, DS ; Gentles, T ; d'Udekem, Y ; Ayer, J ; Blue, GM ; Bridgman, C ; Burchill, L ; Cheung, M ; Cordina, R ; Culnane, E ; Davis, A ; du Plessis, K ; Eagleson, K ; Finucane, K ; Frank, B ; Greenway, S ; Grigg, L ; Hardikar, W ; Hornung, T ; Hynson, J ; Iyengar, AJ ; James, P ; Justo, R ; Kalman, J ; Kasparian, N ; Le, B ; Marshall, K ; Mathew, J ; McGiffin, D ; McGuire, M ; Monagle, P ; Moore, B ; Neilsen, J ; O'Connor, B ; O'Donnell, C ; Pflaumer, A ; Rice, K ; Sholler, G ; Skinner, JR ; Sood, S ; Ward, J ; Weintraub, R ; Wilson, T ; Wilson, W ; Winlaw, D ; Wood, A (ELSEVIER SCIENCE INC, 2020-01)
    The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.