Double Outlet Right Ventricle (DORV) in Fetuses: Prenatal Diagnosis, Associated Findings, and Prognosis - A Single-Center Study

PROGNO AND MANAGEMENT IN FETUSES WITH DOUBLE OUTPUT RIGHT VENTRICLE (DORV)

Abstract

Aim: The Double Outlet Right Ventricle (DORV) presents unique challenges in prenatal diagnosis and postnatal management as a complex congenital heart defect. This article illustrates the prenatal characteristics, accompanying anomalies, and prognosis of fetuses diagnosed with DORV, as well as the postnatal period management in our clinic.
Material and Method: A retrospective analysis of all fetuses
diagnosed prenatally with DORV over a 6-year period in a single tertiary referral center was conducted using records from prenatal, pediatric, and pediatric cardiovascular surgery departments. All additional prenatal findings were evaluated and correlated with the outcomes. The accuracy of the prenatal diagnosis was assessed.
Results: Prenatal diagnosis was made for 153 DORV cases.
The average gestational age at the time of initial diagnosis was 20+6 weeks (range 14-30w). Five cases were excluded due to  unavailability. DORV diagnosis was confirmed in 96.6% of the live births. Major additional cardiac anomalies were found in 134 (93.7%) of the fetuses with DORV, extracardiac anomalies in 91 (63.6%), and chromosomal or syndromal anomalies in 32 (22.3%). Lateralization disorders were observed in 32 (22.2%) fetuses. 46 pregnancies were medically terminated (32.1%), with 8 (5.6%) intrauterine and 19 (13.2%) postnatal deaths. Among the survivors, the average follow-up duration was 46 months (range 3-79). Out of the 83 children who underwent postnatal surgery, 64
(77.1%) continue to live without any complications. Fetuses with pulmonary atresia and stenosis showed a poor prognosis after surgery.
Conclusion: DORV is a rare cardiac anomaly that can be
diagnosed with high precision prenatally, and it’s often complex. DORV is frequently associated with major additional anomalies, leading to high rates of intrauterine and postnatal losses due to terminations or refusals of postnatal treatment. The prognosis is favorable without additional anomalies, but most of the children will have a single ventricle palliation.