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40-Year Follow-Up After the Fontan Operation

2015; Elsevier BV; Volume: 66; Issue: 15 Linguagem: Inglês

10.1016/j.jacc.2015.07.065

1558-3597

Kavitha N. Pundi, Jonathan N. Johnson, Joseph A. Dearani, Krishna Pundi, Zhuo Li, Cynthia A. Hinck, S Dahl, Bryan C. Cannon, Patrick O’Leary, David J. Driscoll, Frank Cetta,

Cardiac Valve Diseases and Treatments

There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation. This study sought to determine the long-term outcome of all patients who had a Fontan operation at the Mayo Clinic. Records of all patients who had a modified Fontan operation between 1973 and 2012 were reviewed. A follow-up questionnaire was mailed to all patients alive at the time of the study. Overall, 10-, 20-, and 30-year survival for 1,052 patients was 74%, 61%, and 43%, respectively. Factors associated with decreased overall or late survival in multivariate analysis included pre-operative diuretic use, longer cardiopulmonary bypass time, operation prior to 1991, atrioventricular valve (AVV) replacement at the time of Fontan operation, elevated post-bypass Fontan (>20 mm Hg) or left atrial (>13 mm Hg) pressures, prolonged chest tube drainage (>21 days), post-operative ventricular arrhythmias, renal insufficiency, and development of protein-losing enteropathy (PLE). Pre-operative and intraoperative sinus rhythm were associated with improved survival. Long-term survival was similar for patients regardless of ventricular morphology. The most common reoperations were pacemaker insertion/revision in 212 patients (20%), Fontan revision/conversion in 117 patients (11%), and AVV repair/replacement in 66 patients (5%). Clinically significant late atrial or ventricular arrhythmias occurred in 468 patients (44%). Ninety-five patients (9%) developed PLE, and 5-, 10-, and 20-year survival after diagnosis of PLE was 50%, 35%, and 19%, respectively. As the surgical techniques for the Fontan operation have changed over the last 40 years, survival has improved. However, development of PLE and arrhythmias and the need for reoperation during long-term follow-up pose significant management challenges.