Abstract 1923: Medical Characteristics and Midterm Outcome: 100 Consecutive Fontan Conversions with Arrhythmia Surgery
Introduction Fontan conversion with arrhythmia surgery has provided a unique opportunity to assess medical characteristics of patients (pts) with failing Fontan circulations, and to identify high-risk pts.
Methods A retrospective review of charts was performed to delineate clinical characteristics of the first 100 consecutive pts undergoing Fontan converison with arrhythmia surgery at Children’s Memorial Hospital between 1994 and 2006.
Results Patients’ ages ranged from 2.6–47 yrs (median 21.4 yrs). Mean postoperative interval following initial Fontan surgery was 15.1 yrs. Anatomic diagnosis was single left ventricle in 85, single right ventricle in 11, and ambiguous ventricle in 4 pts. Indications for surgery were exercise intolerance in 99 pts, associated with refractory arrhythmia in 95 pts. NYHA classification among pts was IV (21), III (55), and I-II (24). Mean cardiac index was 2.6 ± 0.8 L/min/m2, with mean oxygen consumption during exercise testing 18 ml/kg/min. Medical co-morbidities prior to surgery included depression in 42%, thrombocytopenia in 23%, and thyroid disorders in 7%. Prior strokes were present in 9 pts; right atrial thrombi were noted in 18. Protein-losing enteropathy (PLE) was present in 2 pts. Arrhythmia mechanism was right atrial macro-reentry in 33%, and atrial fibrillation or left atrial reentry tachycardia in 62%. Repair of associated hemodynamic problems included atrioventricular (AV) valve repair (11) or replacement (3); aortic valve replacement in 1, and aortic root replacement in 1. Early mortality (1%) occurred in 1 patient, with reoperation for bleeding in 1 pt. Freedom from death or transplant was 94% at 1 yr and 89% at 5 yrs. Freedom from SVT recurrence was 94% at 1 yr and 88% at 5 yrs. NYHA classification improved in 90%, with improvement in platelet count and depression. Risk factors for death or cardiac transplantation by multivariate analysis included PLE, AV valve repair, and prolonged cardiopulmonary bypass time.
Conclusion Depression, thrombocytopenia, stroke, and thyroid disease are common co-morbidities in Fontan pts. Patients with PLE and severe AV valve dysfunction should be considered for transplantation. Intervention prior to the development of severe AV valvar regurgitation or PLE improves outcome.