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Circulation Journal
Vol. 76 (2012) No. 1 p. 195-203



Pediatric Cardiology and Adult Congenital Heart Disease

Background: The serial hemodynamics and predictors of long-term, good Fontan survivors remain unknown. Methods and Results: Two hundred one patients who had undergone a Fontan operation before September 1998 were reviewed to compare their long-term clinical status with serial hemodynamics. During a mean follow-up of 18.7 years, 47 (30.1%) of the 156 survivors had no clinical events that required an unscheduled hospitalization. Of those, 18 survivors exhibited good serial hemodynamics and the respective mean values of central venous pressure (CVP, mmHg), cardiac index (CI, L·min-1·m2), and ventricular ejection fraction (EF, %) before and 1, 5, 10, and 15 years after the operation were as follows: 3.8, 9.0, 11.3, 10.6, and 10.1 (CVP); 3.6, 3.1, 2.8, 2.6, and 2.6, and 69 (CI); 57, 56, 58, 54, and 53 (EF). Serial values of CVP, ventricular end-diastolic pressure (EDP) and the grade of atrioventricular valve regurgitation (AVVR) were lower and the peak oxygen uptake was greater in the good patients (P<0.05 for all). A 1-year postoperative lower CVP and no history of AVVR repair during the Fontan operation independently predicted the good patients (P<0.05). Conclusions: A lower CVP and EDP, better atrioventricular valvular function, and greater exercise capacity characterize good Fontan survivors and an early postoperative low CVP without an AVVR repair predicts good survivors. (Circ J 2012; 76: 195-203)


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