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

Area of Science:

• Cardiology
• Fetal Medicine
• Pediatric Surgery

Background:

• Double-inlet left ventricle (DILV) is a complex congenital heart defect.
• Prenatal diagnosis and management are crucial for affected fetuses.
• Tertiary care centers play a vital role in managing DILV cases.

Purpose of the Study:

• To review the experience of a tertiary care center in managing fetuses with DILV.
• To assess fetal echocardiography findings, prenatal course, and postnatal outcomes.
• To evaluate the effectiveness of current management strategies for DILV.

Main Methods:

• Retrospective study of DILV cases diagnosed prenatally between 2015 and 2023.
• Inclusion of patients from a maternal-fetal medicine department.
• Evaluation of prenatal diagnosis, course, postnatal management, and outcomes.

Main Results:

• 33 cases of DILV were confirmed postnatally.
• Genetic abnormalities were found in 8% of tested fetuses.
• Pulmonary artery obstruction (30.3%) and aortic arch abnormalities (33.3%) were common.
• Survival rate was 66.6% at a median follow-up of 12 months.
• 24 of 27 live births (81.8%) required univentricular palliation.

Conclusions:

• DILV is often not associated with chromosomal anomalies unless other defects are present.
• Univentricular repair is feasible in most DILV cases.
• Restrictive bulboventricular foramen and severe aortic obstruction are key factors in poor prognosis.
• Long-term survival after univentricular repair is achievable but requires ongoing monitoring and interventions.