Abstract

Few studies investigated the long-term outcome of patients with univentricular heart and common atrioventricular valve. We retrospectively analysed the medical files of all patients univentricular heart with common atrioventricular valve in the setting of heterotaxy or with unbalanced atrioventricular septal defect preventing biventricular repair. 117 patients were identified during the study period. 89/117 had a postnatal diagnosis. 28/117 patients never underwent surgery, 25/117 underwent one palliation surgery (Blalock-Taussig-shunt (BTS)/ pulmonary banding), and finally, 61/117 patients entered a sequential cavopulmonary connection program: 37/61 had partial cavopulmonary connection at the time of data analysis while 24/61 had total cavopulmonary connection (TCPC). The average age at TCPC was 7.6 years +/-4 years [1.7-16 years]. Three patients were eventually transplanted. The overall mortality was 59% (69/117): 65% and 30% in heterotaxy and in patients with unbalanced atrioventricular septal defect respectively. Mortality was 85% (24/28) in the subgroup of patients who never underwent surgery, 93% in the subgroup of patients who had a BTS, and 89% after pulmonary banding. In the subgroup planed to have TCPC, 49% died after partial cavopulmonary connection and survival rate was 71.6% [50.7-100] in patients who had TCPC. The long-term outcome of univentricular hearts with common atrioventricular valve treated in a tertiary referral center showed a high mortality rate. Patients with this kind of complex congenital heart disease should undergo the sequential univentricular program without delay in order to obtain better long-term survival.

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