Author + information
- Received September 26, 2017
- Revision received November 27, 2017
- Accepted December 19, 2017
- Published online February 26, 2018.
- Mark Dennis, MBBS Honsa,b,
- Diana Zannino, MScc,
- Karin du Plessis, PhDc,
- Andrew Bullock, MBBSd,
- Patrick J.S. Disney, MBBSe,
- Dorothy J. Radford, MBBS, MDf,
- Tim Hornung, MDg,
- Leeanne Grigg, MBBSh,
- Rachael Cordina, MBBS, PhDa,b,
- Yves d’Udekem, MD, PhDc,i and
- David S. Celermajer, MBBS, PhD, DSca,b,∗ ()
- aSydney Medical School, University of Sydney, Camperdown, Sydney, Australia
- bDepartment of Cardiology, Royal Prince Alfred Hospital, Missenden Road, Sydney, Australia
- cMurdoch Children’s Research Institute, Melbourne, Australia
- dChildren’s Cardiac Centre, Princess Margaret Hospital for Children, Perth, Australia
- eDepartment of Cardiovascular Services, Royal Adelaide Hospital, Adelaide, Australia
- fAdult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Australia
- gGreen Lane Paediatric and Congenital Cardiac Service, Starship Children’s Hospital Auckland, Auckland, New Zealand
- hDepartment of Cardiology, The Royal Melbourne Hospital, Melbourne, Australia
- iDepartments of Cardiac Surgery and Cardiology, Royal Children’s Hospital, Melbourne, Australia
- ↵∗Address for correspondence:
Dr. David S. Celermajer, Cardiology Department, Royal Prince Alfred Hospital, Missenden Road, Camperdown, New South Wales 2050, Australia.
Background Long-term outcomes of Fontan patients who survive to age ≥16 years have not been well characterized. The Australian and New Zealand Fontan Registry (ANZFR) provides a unique opportunity to understand survival and complication rates in Fontan patients who transition to adult congenital heart disease centers.
Objectives This study sought to describe the survival and complications of adult patients who have had a Fontan procedure.
Methods The study analyzed outcomes in patients ≥16 years of age who were prospectively enrolled in the ANZFR.
Results Data from all 683 adult survivors from the ANZFR were analyzed. Mortality status was confirmed from the National Death Index. There were 201 atriopulmonary (AP) connections and 482 total cavopulmonary connections (249 lateral tunnels and 233 extracardiac conduits). For these subjects, the survival rate at age 30 years was 90% (95% CI: 87% to 93%), and it was 80% (95% CI: 75% to 87%) at 40 years of age. Survival at age 30 years was significantly worse for the patients with AP connections (p = 0.03). At latest follow-up, only 53% of patients were in New York Heart Association functional class I. After the age of 16 years, 136 (20%) had experienced at least 1 new arrhythmia, 42 (6%) required a permanent pacemaker, 45 (7%) had a thromboembolic event, and 135 (21%) required a surgical reintervention. Only 41% (95% CI: 33% to 51%) of Fontan patients were free of serious adverse events at 40 years of age.
Conclusions This comprehensively followed cohort showed that a variety of morbid complications is common in Fontan adults, and that there is a substantial incidence of premature death, particularly in patients with AP connections.
This work was supported by a National Health and Medical Research Council (NHMRC) Partnership Grant (1076849). Dr. d’Udekem is a Clinician Practitioner Fellow of the National Health and Medical Research Council (NHMRC) (1082186); and has received consulting fees from Merck Sharp & Dohme and Actelion. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received September 26, 2017.
- Revision received November 27, 2017.
- Accepted December 19, 2017.
- 2018 American College of Cardiology Foundation
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