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Ann Thorac Surg 2009;88:558-564. doi:10.1016/j.athoracsur.2009.03.085
© 2009 The Society of Thoracic Surgeons
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Right arrow Congenital - cyanotic
Right arrow Transplantation - heart

Original Articles: Pediatric Cardiac

Evaluating Failing Fontans for Heart Transplantation: Predictors of Death

Eric R. Griffiths, MDa, Aditya K. Kaza, MDa, Moritz C. Wyler von Ballmoos, MDa, Hugo Loyola, BSa, Anne Marie Valente, MDb, Elizabeth D. Blume, MDb, Pedro del Nido, MDa,*

a Department of Cardiac Surgery Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts
b Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts

Accepted for publication March 26, 2009.

* Address correspondence to Dr del Nido, Department of Cardiac Surgery, Children's Hospital Boston, 300 Longwood Ave, Boston, MA 02115 (Email: pedro.delnido{at}cardio.chboston.org).

Presented at the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.

Background: Late complications of the Fontan operation represent a significant management challenge. Failing Fontan patients have two modes of presentation: impaired ventricular function (IVF) and those with preserved ventricular function (PVF) but with failing Fontan physiology (protein-losing enteropathy [PLE] and plastic bronchitis [PB]). This study evaluated whether failing Fontan patients referred for heart transplantation had a different outcome based on the mode of presentation.

Methods: The medical records of all Fontan patients evaluated for heart transplantation at a single institution from 1994 to 2008 were retrospectively reviewed. Demographic, hemodynamic, and laboratory data were collected. Patients were stratified into an IVF or PVF group by echocardiographic criteria. Descriptive statistics and Kaplan-Meier analysis were used for hypothesis testing.

Results: Thirty-four Fontan patients were evaluated for heart transplantation. According to echo description of systolic function, 18 were categorized as IVF and 16 as PVF. The IVF group had a significantly lower cardiac index and venous oxygen saturation, and significantly higher systemic vascular resistance vs the PVF group (p < 0.05). PLE or PB was present in 13 PVF patients and none in the IVF group. Twenty patients underwent transplantation, with similar rates amongst the IVF and PVF groups. Within 1 year from evaluation, 2 IVG patients and 7 PVF patients had died (p = 0.052).

Conclusions: Failing Fontan patients with PVF have decreased overall survival independent of whether they underwent transplantation. This trend indicates a need to improve the management and timing for transplantation amongst this population.






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