Cardiovascular profile score (CVPS) and selected cardiac parameters in fetuses with Vein of Galen Malformation.

Abstract

Objectives: Vein of Galen Malformation (VGAM) is a rare congenital cerebrovascular anomaly. Early detection and monitoring of concurrent fetal heart failure in VGAM are crucial for improving outcomes. The study aims to evaluate heart anatomy, systolic and diastolic heart function, and indicators of heart failure in fetuses referred to a tertiary center due to VGAM detected in the second or third trimester.

Material and methods: This single-center retrospective study of echocardiographic data from five fetuses with VGAM examined between 2008 and 2023. Parameters analyzed included gestational age, reason for referral, cardiovascular profile score (CVPS), systolic and diastolic heart function and selected cardiac parameters RESULTS: Cardiomegaly as a sign of congestive heart failure was the main reason for referral most of the fetuses (4/5 - 80%) diagnosed later as VGAM in our center. Abnormal cerebral vessel flow was visualized in all cases, with normal umbilical arterial flow. Three fetuses were treated prenatally with digoxin due to congestive heart failure. Monophasic tricuspid valve inflow and decreased shortening fraction (SF) were observed in three fetuses (60%) at the time of diagnosis. CVPS scores ranged from 2 to 10, correlating with the severity of cardiac compromise. All five patients demised, three died in utero, and two shortly after birth.

Conclusions: This study emphasizes the severe cardiac implications of VGAM detected in utero and the need for early and comprehensive fetal assessment. Despite early diagnosis, outcomes remain poor, necessitating further research into effective prenatal treatments and management protocols to enhance survival for affected fetuses.