Cardiac Findings in the Fetus with Cerebral Arteriovenous Malformation Are Associated with Adverse Outcome

Abstract

Objectives: To assess cardiac sequelae of fetal cerebral arteriovenous malformations (CAVMs) and evaluate any association with outcomes.

Methods: We retrospectively analyzed cardiac structure and function in fetuses with CAVMs who underwent fetal echocardiography (October 1999 to August 2015, n = 11), and compared them with normal controls.

Results: The median gestational age was 36 weeks (range 18-38). Common abnormal findings included dilated superior vena cava (100%) and right atrium (82%), reduced middle cerebral artery pulsatility index (86%), tricuspid regurgitation (82%), and right ventricular (RV) dysfunction (64%). Hydrops was present in 1 fetus, who did not survive. The median cardiothoracic ratio (CTR) was 0.36 (0.29-0.45, n = 10); the median combined cardiac output indexed to estimated fetal weight (iCCO) was 565 ml/kg/min (379-1,565, n = 7). Of the 11 fetuses, 1 patient elected for termination, and 5 suffered neonatal demise. Comparing survivors (n = 5) and nonsurvivors (n = 6), a larger tricuspid valve (TV) z-score (p = 0.009) and RV dysfunction (p = 0.015) were associated with nonsurvival, and nonsurvivors had a higher iCCO than controls (990 vs. 550 ml/kg/min, p = 0.035). A larger difference between the TV and mitral valve z-scores (surrogate for RV dilation, p = 0.052), and CTR >0.38 (p = 0.0762) tended towards nonsurvival.

Conclusion: CAVMs impose volume load on the fetal circulation, mainly affecting right heart structures. Increased right heart dilation and dysfunction are associated with nonsurvival.