摘要
Elevated pulmonary vascular resistance may contribute to late Fontan circulati on failure but is difficult to assess in such patients. Our aims were to assess outcomes of patients with failed Fontan circulation after heart transplantation and to determine whether elevated pulmonary vascular resistance might have contr ibuted to the failure. Fifteen patients (14 Fontan circulations, 1 Kawashima cir culation) underwent transplantation. The most common indication was ventricular dysfunction(mean ventricular end-diastolic pressure 12.5mmHg). Patients with ea rly failures(n=4) required transplantation less than 1 year after the Fontan ope ration. Those with late failures(n=11) underwent transplantation at least 1 year after the Fontan operation. Mean age at transplantation was 11.6 years. Mean Fo ntan-transplantation intervalwas 7.4 years. Mean pulmonary arterial pressure, t ranspulmonary gradient, and pulmonary vascular resistance before and after trans plantation were assessed. Paired t tests of variable differences were used to co mpare variables. Survival was estimated by the Kaplan-Meier method. In-hospita lmortalitywas 7%. Therewere 2 late events(1 death, 1 retransplantation) related to compliance or rejection issues. Graft survivals were 93%, 82%, and 82%at 3, 5, and 7 years, respectively. Posttransplantation pulmonary vascular resistan ce was elevated(2.0Wood units m2) in 11 of 14 survivors past initial hospitaliza tion(mean 3.3 1.7Wood unitsm2). Only patients with early Fontan failures(3 of 4) had normal posttransplantation pulmonary vascular resistance. In paired compari sons, posttransplantation transpulmonary gradient was increased by a mean of 6.8 mm Hg(P . 0001) relative to pretransplantation value. Outcomes after heart tran splantation for failed Fontan circulation were good. Mild-to-moderate pulmonar y vascular disease was evident after heart transplantation for late failure. Ele vated pulmonary vascular resistance is a likely contributor to Fontan circulatio n failure.
Elevated pulmonary vascular resistance may contribute to late Fontan circulati on failure but is difficult to assess in such patients. Our aims were to assess outcomes of patients with failed Fontan circulation after heart transplantation and to determine whether elevated pulmonary vascular resistance might have contr ibuted to the failure. Fifteen patients (14 Fontan circulations, 1 Kawashima cir culation) underwent transplantation. The most common indication was ventricular dysfunction(mean ventricular end-diastolic pressure 12.5mmHg). Patients with ea rly failures(n=4) required transplantation less than 1 year after the Fontan ope ration. Those with late failures(n=11) underwent transplantation at least 1 year after the Fontan operation. Mean age at transplantation was 11.6 years. Mean Fo ntan-transplantation intervalwas 7.4 years. Mean pulmonary arterial pressure, t ranspulmonary gradient, and pulmonary vascular resistance before and after trans plantation were assessed. Paired t tests of variable differences were used to co mpare variables. Survival was estimated by the Kaplan-Meier method. In-hospita lmortalitywas 7%. Therewere 2 late events(1 death, 1 retransplantation) related to compliance or rejection issues. Graft survivals were 93%, 82%, and 82%at 3, 5, and 7 years, respectively. Posttransplantation pulmonary vascular resistan ce was elevated(2.0Wood units m2) in 11 of 14 survivors past initial hospitaliza tion(mean 3.3 1.7Wood unitsm2). Only patients with early Fontan failures(3 of 4) had normal posttransplantation pulmonary vascular resistance. In paired compari sons, posttransplantation transpulmonary gradient was increased by a mean of 6.8 mm Hg(P . 0001) relative to pretransplantation value. Outcomes after heart tran splantation for failed Fontan circulation were good. Mild-to-moderate pulmonar y vascular disease was evident after heart transplantation for late failure. Ele vated pulmonary vascular resistance is a likely contributor to Fontan circulatio n failure.
