A Population-Based Study of Cardiac Malformations and Outcomes Associated With Dextrocardia

The incidence of dextrocardia and its associated cardiac and noncardiac malformations is not known. There is inadequate information about outcomes to counsel parents about prognosis. A retrospective review of all diagnoses of dextrocardia due to embryologic development at a tertiary care hospital from 1985 to 2001 was performed. Eighty-one cases were identified (48 antenatally). The incidence of dextrocardia was estimated to be 1 in 12,019 pregnancies. Twenty-seven cases were situs solitus, 30 situs inversus, and 24 situs ambiguous or isomerism. Cardiac malformations were found in 26 of 27 cases of situs solitus, 7 of 30 cases of situs inversus, and 24 of 24 cases of isomerism. Noncardiac malformations were identified in 10 of 27 cases of situs solitus, 6 of 30 cases of situs inversus, and 14 of 24 cases of isomerism. Twelve pregnancies were terminated, 3 fetuses were stillborn, and 2 women chose compassionate care. All terminated fetuses were diagnosed with dextrocardia before termination, and all had >1 cardiac anomaly; 7 also had noncardiac anomalies. There were 43 subjects in the intention-to-treat group (20 situs solitus, 10 solitus inversus, 13 isomerism). Thirty-two had ≥1 cardiac operation, and 21 had ≥3. Thirty-nine subjects were alive at most recent follow-up. In conclusion, the incidence of dextrocardia was 1 in 12,019 pregnancies. In conclusion, in our cohort, the numbers of cases of situs solitus, situs inversus, and isomerism were similar. Cardiac and noncardiac malformations were most common in the isomerism group. Cardiac malformations were often complex in the situs solitus and isomerism groups. Ninety-one percent of those in the intention-to-treat cohort were alive at follow-up.