Fahimeh Azizinik, Behnaz Moradi, Razieh Mohammadi-Dashtaki, Shima Karbasi, Nima Broomand Lomer, Mohammad Mohammadi, Mobin Fathy, Maryam Rahmannia, Hamed Ghorani, Maedeh Mohaghegh, Fatemeh Shakki Katouli, Parham Talebi-Boroujeni
PURPOSE: Cardiac rhabdomyoma, the most common fetal cardiac tumor, is frequently associated with tuberous sclerosis complex (TSC). This study examined pre- and postnatal factors affecting mortality in fetal cardiac rhabdomyoma (FCR) and explored links between TSC and clinical features. METHODS: A systematic review was conducted in July 2023 using Medline, Web of Science, Embase, and Cochrane Library to identify reported FCR cases. Analyses were performed on two sample groups (A and B). RESULTS: Neonatal mortality was significantly associated with hydrops (p < 0.001), tumor progression (p < 0.001), fetal heart failure (p < 0.001), and fetal growth restriction (FGR) (p = 0.021). Hydrops was the strongest predictor of mortality (Sample A: RR 17.2, p = 0.027; Sample B: RR 12.2, p = 0.006). Among 683 cases, 73% were diagnosed with TSC. Multiple tumors increased the likelihood of TSC (Sample A: RR 4.8, p < 0.0001; Sample B: RR 3, p = 0.015). In postnatal factors, preterm delivery (p < 0.001), cardiac arrhythmia (p = 0.001), and heart failure (p < 0.001) were also linked to mortality. CONCLUSIONS: Hydrops is the strongest mortality predictor in FCR. Multiple tumors are associated with TSC. In prenatal factors, tumor progression, fetal heart failure, and FGR were associated with increased mortality.