We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy.

Patients with a pre or postnatal diagnosis of DORV from 2000 to 2007 were identified and classified into 3 subgroups: subaortic ventricular septal defect (VSD) and normal great artery (GA) arrangement (=VSD type), tetralogy of Fallot type, and transposition of the GA type (=TGA type). Patients with heterotaxy, atrioventricular septal defect, valve atresia, and ventricular hypoplasia were excluded. Complex postnatal care was defined as prematurity, need for prostaglandins, surgical repair <2 months, or univentricular palliation. Risk factors for complex postnatal care and demise were sought in multivariable logistic regression models. One hundred fort-five patients were included (93 prenatal, 52 postnatal).

There were 24 pregnancy terminations and 7 in utero deaths. Fetal demise was associated with abnormal karyotype (odds ratio [OR] 1.9, P = .01), any tricuspid valve regurgitation (OR 10.6, P = .01), and hydrops (OR 23.8, P = .02). Of 114 liveborn patients, 23 were tetralogy-type, 67 VSD-type, and 24 TGA-type patients. Postnatal survival of liveborn patients at 1 year was similar in pre- versus postnatally diagnosed patients (84% vs 85%). Abnormal GA relationship (OR 2.9, P = .02), subpulmonary VSD (OR 6.0, P = .001), unobstructed pulmonary blood flow at birth (OR 2.8, P = .05), and aortic coarctation (OR 9.0, P = .007) were associated with suboptimal postsurgical outcomes.

Double outlet right ventricle, even without heterotaxy, is associated with complex postnatal care and high risk of early demise. Morphologic subtype, irrespective of pre- or postnatal diagnosis, is a major determinant of outcome.