Navigating the Spectrum of Double-İnlet Left Ventricle Presentations: Contemporary Retrospective Cohort Study

Purpose: We presented the experience of a tertiary care center for maternal and fetal diseases and assessed the findings fetuses with double-inlet left ventricle (DILV) regarding fetal echocardiography, prenatal course including fetal growth and death, and postnatal outcome.

Methods: In this retrospective study, patients diagnosed with DILV via prenatal ultrasound in the maternal-fetal medicine department between 2015 and 2023 were included to evaluate important aspects of prenatal diagnosis and course, as well as postnatal management and outcome.

Results: There were 33 DILV cases prenatally diagnosed and postnatally confirmed. Genetic abnormality was detected in two (8%) of 25 fetuses that had undergone genetic tests. Pulmonary artery obstruction was observed in 10 (30.3%) fetuses; pulmonary stenosis was found in nine, and pulmonary atresia in one. Aortic arc abnormalities were found in 11 (33.3%) fetuses. The great arteries were malpositioned in the 29 (87.8%) fetuses. The median duration of follow-up for the infants was 12 (2-96) months, and 22 (66.6%) of 33 cases survived. Among the 27 (81.8%) live births, 24 infants required univentricular palliation.

Conclusions: Remarkable implications of this study were that unless extra-cardiac and genetic anomalies accompany DILV, the probability of a chromosomal anomaly is low; that univentricular repair could be performed in almost all cases; and that the major causes of poor prognosis may be related to the restrictive bulboventricular foramen and severe aortic obstruction. Although long-term survival rates after univentricular repair appear to be high, these patients require long-term follow-up and repeated surgical procedures.