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.