228 - Prenatally Diagnosed Vascular Rings – Circling Back to Primary Care Pediatrics

Poster Number: 228
Publication Number: 228.221

Nicolle M. Ceneri, Children's National Medical Center, WASHINGTON, DC, United States; Michelle Udine, Children's National Health System, Washington, DC, United States; Stephanie R. Lacey, Children's National Health System, Richmond, VA, United States; Mary Donofrio, Children's National Health System, Washingon, DC, United States

Background: Recent clinical experience suggests an increase in prenatal diagnosis of vascular rings(VR). Correlation with postnatal symptoms and outcomes has not been investigated. This poses counseling and management dilemmas for fetal/pediatric cardiologists and pediatricians.

Objective: To analyze short term outcomes of prenatally diagnosed VR including postnatal diagnosis, development of symptoms, need for surgery and persistence of symptoms after surgery.

Design/Methods: Single-center, retrospective review of patients with a fetal diagnosis of isolated VR from 2010-2021. Postnatal evaluation included echocardiography +/- cardiac magnetic resonance imaging(cMRI) or computed tomography(CT). Symptoms, surgical interventions and outcomes were recorded.

Results: Fifty fetuses were identified with concern for VR due to right aortic arch(RAA) or double aortic arch(DAA). Postnatal diagnoses included RAA with aberrant left subclavian artery(ALSCA) in 71%(n=36), DAA in 18%(n=9) and RAA with no VR in 10%(n=5). 74% had cMRI(n=37) or CT(n=1). Fetal echocardiography and cMRI/CT diagnoses were concordant in 81%(30/37); 4 had discrepant type of VR and 3 had inconclusive arch anatomy prenatally that was defined postnatally. For those with RAA and cMRI/CT performed, cMRI identified ALSCA and Kommerell’s diverticulum consistent with VR in 94%(30/32); 2 had RAA with mirror image branching and no VR. Airway narrowing was noted in 5/37(14%). Two newborns (4%) required delivery room resuscitation with resolution of symptoms not attributable to VR. Symptoms attributed to the VR were observed in 31%(15/45) with median onset at 42 days. Of those symptomatic, 40%(6/15) had respiratory and 80%(12/15) had gastrointestinal symptoms. Surgical repair was performed in 22%(10/45) at median age of 99(34-370) days; 90%(9/10) were symptomatic and one with DAA was asymptomatic at 2-month follow-up. Median follow-up was 68 days (7 days-7 years); 33% (15/45) had follow up at 6-months. At last follow up, 40%(4/10) surgical patients remained symptomatic with stridor(n=2) and feeding difficulties(n=2).Conclusion(s): Patients with prenatally diagnosed VR are stable in the delivery room and do not require subspecialty care at birth. Fetal echocardiography accurately identifies a potential VR, with postnatal cMRI/CT providing anatomic detail and limited airway assessment. Postnatal cMRI/CT should be utilized to confirm arch anatomy if not delineated by echocardiography and for surgical planning. Symptoms attributed to VR may not improve with surgery; assessment and monitoring of symptoms by pediatricians is integral to the care of these patients.
Nicolle Ceneri CV - APA AWARD SUBMISSIONCeneri Nicolle_CV for PAS_2022 Jan.pdf