Prenatal diagnosis of vascular ring: evaluation of fetal diagnosis and postnatal outcomes

Abstract Funding Acknowledgements Type of funding sources: None. Background The performance of fetal echocardiogram in diagnosing vascular ring (VR) and its impact on postnatal outcomes has not been well examined. Methods We reviewed all patients with VR diagnosis from 2000-2020. Results 50 patients with antenatal diagnosis of VR; 42(84%) with right aortic arch aberrant left subclavian artery and left-sided ductus arteriosus ((RAA-ALS) and 8(16%) with double aortic arch (DAA) were compared to 120 patients with postnatal diagnosis; 90(75%) with DAA, 22(18%) with RAA-ALS (Table 1). Prenatal diagnosis of VR increased over study period; 4 vs 31, 10 vs 29, 14 vs 25, 22 vs 35 between 2000-2005, 2005-2010, 2010-2015, and 2015-2020 respectively, p< 0.01. Prenatal diagnosis of VR was associated with less symptoms [26(52%) vs 72(60%), p < 0.003] and less cross-sectional imaging [22(44%) vs 82(69%), p = 0.0001]. Postnatal diagnosis of VR was associated with more surgical interventions (102(85%) vs 28(56%), p = 0.002), later surgical repair (22.9 ± 35 vs 12.6 ± 10.2 months, p < 0.01), more postoperative complications [8/102 (7.8%) vs 1/28 (3.5%), p = 0.04] and more residual symptoms (2/28(7%) vs 30/102(29%), p = 0.001 respectively]. Conclusion Prenatal diagnosis of VR has evolved over time. RAA-ALS versus DAA were dominant in the prenatally and postnatally diagnosed cohorts respectively. Prenatal diagnosis of VR was associated less symptoms, less cross-sectional imaging, earlier age of surgical intervention and less residual symptoms. Table 1 Prenatal Diagnosis (N = 50) Postnatal Diagnosis (N = 120) p-value Gestational Age (weeks) 39 ± 3 38 ± 2 0.9 Male (%) 31 (62%) 69(58%) 0.8 Subtype of vascular ring: Double aortic arch RAA-ALSA Others 8 (16%) 42 (80 %) 0 (0 %) 90 (72%) 22 (17%) 8 (11%) 0.002 0.01 Associated intracardiac CHD: VSD TOF DORV AVSD Coarctation of aorta Others 11 (22%) 1 1 3 0 3 3 24 (20%) 5 7 2 2 3 5 0.8 Associated genetic diagnosis: 22q11 deletion Trisomy 21 Others 9 (18%) 6 3 0 32 (26%) 29 3 0 0.1 Symptomatic presentation: Respiratory Gastrointestinal 26 (52 %) 15 11 72 (60 %) 57 15 <0.03 Age at first symptoms (month) 3.2 ± 2.8 21.6 ± 37 0.01 Diagnostic modalities: Fetal echocardiogram Postnatal echocardiogram Barium swallow Bronchoscopy CT scan MRI 50 (100%) 50 (100%) 2 (4%) 3 (6%) 18 (36 %) 4 (8%) 0 (0%) 120 (100%) 23 (25 %) 18 (15 %) 65 (54%) 7 (14%) Surgical repair 28 (56%) 102 (85%) 0.02 Age at surgical repair (month) 12.6 ± 10.2 22.9 ± 35 <0.01 Postoperative complications 1 (3%) 8 (7%) 0.03 Residual symptoms 2 (1%) 30 (25%) 0.001 Age at most recent follow up (mo) 26 ± 7 60 ± 7 0.02 Table 1: Comparison of patients with prenatal versus postnatal diagnosis of vascular ring