1. Clinical and hemodynamic characteristics of the pediatric failing Fontan
- Author
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Erin L. Albers, Maryanne Chrisant, John C. Dykes, Doff B. McElhinney, Asma Khan, Kurt R. Schumacher, Daniel Bernstein, Rebecca K. Ameduri, Kenneth R. Knecht, Sharon Chen, Kathleen E. Simpson, Marc E. Richmond, Kevin P. Daly, David N. Rosenthal, Simon Urschel, Kimberly Y. Lin, Christopher S. Almond, and Jacob Simmonds
- Subjects
Pulmonary and Respiratory Medicine ,Heart Defects, Congenital ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Cardiac output ,Adolescent ,Waiting Lists ,Cardiac index ,Hemodynamics ,Fontan Procedure ,Primary outcome ,Risk Factors ,Internal medicine ,medicine ,Reduced systolic function ,Humans ,Treatment Failure ,Child ,Retrospective Studies ,Transplantation ,business.industry ,Age Factors ,medicine.disease ,Survival Rate ,surgical procedures, operative ,Heart failure ,Child, Preschool ,Cohort ,cardiovascular system ,Cardiology ,Heart Transplantation ,Surgery ,Female ,Fontan failure ,Cardiology and Cardiovascular Medicine ,business - Abstract
AIM To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant. METHODS In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with "normal" hearts (FNH). Primary outcome was waitlist and post-transplant mortality. RESULTS 177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI
- Published
- 2020