Prenatal Diagnosis of Vascular Ring: Evaluation of Fetal Diagnosis and Postnatal Outcomes. 2022

Safwat Aly, and Koyelle Papneja, and Wadi Mawad, and Mike Seed, and Edgar Jaeggi, and Shi-Joon Yoo
Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

The impact of fetal echocardiography on the diagnosis and outcomes of vascular ring has not been well examined. We hypothesized that prenatal detection of vascular ring has improved over time and that prenatal diagnosis of vascular ring is associated with earlier intervention and favorable outcomes. This is a single-center, retrospective study of the evolution and outcomes of prenatal diagnosis of vascular ring from 2000 to 2020. We compared clinical presentation, timing of surgical intervention, and outcomes between the prenatally and postnatally diagnosed cases during the same study period. A total of 170 patients were included: 50 with prenatal and 120 with postnatal diagnosis of vascular ring. Prenatal diagnoses included 42 patients (84%) with right aortic arch (RAA), aberrant left subclavian artery (ALSCA), and a left-sided ductus arteriosus and eight (16%) patients with double aortic arch (DAA). The postnatal cohort consisted mainly of 90 patients (75%) with DAA and 22 (18%) with RAA-ALSCA. None of the postnatally diagnosed cases had undergone a fetal echocardiogram. Numbers (percentage) of prenatally diagnosed cases of vascular ring compared with the postnatal cases improved from 4/31 (13%), to 10/29 (34%), to 14/25 (56%), and to 22/35 (69%), respectively, during 2000-2005, 2005-10, 2010-15, and 2015-20 (P = .032). Vascular ring was an isolated abnormality in 84% and 85% of the prenatal and postnatal cohorts, respectively. Compared with the prenatal cohort, postnatally diagnosed patients with an isolated vascular ring were more frequently symptomatic (66% vs 48%, P < .03) and underwent cross-sectional imaging (69% vs 44%, P = .009) and surgery more frequently (79% vs 48%, P = .003). Surgery was performed at a later patient age (18 [2-147] months vs 4.8 [0.5-42] months, P = .01) and was more often associated with residual symptoms (27/81 [33%] vs 1/20 [5%], P = .01) in the postnatal cohort than in the prenatal cohort. The diagnosis of vascular ring by fetal echocardiography has improved over time. A significantly higher incidence of RAA-ALSCA in the prenatal compared with the postnatal cohort suggests that patients with this form of vascular ring often do not present to medical attention with significant symptoms postnatally. Prenatal diagnosis of vascular ring was associated with a lower incidence of symptoms, less frequent use of cross-sectional imaging, earlier age at surgical intervention, and lower likelihood of residual symptoms.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011296 Prenatal Diagnosis Determination of the nature of a pathological condition or disease in the postimplantation EMBRYO; FETUS; or pregnant female before birth. Diagnosis, Prenatal,Fetal Diagnosis,Fetal Imaging,Fetal Screening,Intrauterine Diagnosis,Antenatal Diagnosis,Antenatal Screening,Diagnosis, Antenatal,Diagnosis, Intrauterine,Prenatal Screening,Antenatal Diagnoses,Antenatal Screenings,Diagnosis, Fetal,Fetal Diagnoses,Fetal Imagings,Fetal Screenings,Imaging, Fetal,Intrauterine Diagnoses,Prenatal Diagnoses,Prenatal Screenings,Screening, Antenatal,Screening, Fetal,Screening, Prenatal
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000073872 Vascular Ring Congenital vascular malformation in which the AORTA arch and its branches encircle the TRACHEA and ESOPHAGUS. Signs and symptoms include DYSPNEA; RESPIRATORY SOUNDS, especially with eating, DYSPHAGIA, persistent cough, and GASTROESOPHAGEAL REFLUX or may be asymptomatic. Two most common types are double aortic arch and right aortic arch. It may be associated with other anomalies (e.g., DIGEORGE SYNDROME). Double Aortic Arch,Right Aortic Arch Syndrome,Right Aortic Arch with Left Ligamentum Arteriosum,Aortic Arch, Double,Vascular Rings
D001013 Aorta, Thoracic The portion of the descending aorta proceeding from the arch of the aorta and extending to the DIAPHRAGM, eventually connecting to the ABDOMINAL AORTA. Aorta, Ascending,Aorta, Descending,Aortic Arch,Aortic Root,Arch of the Aorta,Descending Aorta,Sinotubular Junction,Ascending Aorta,Thoracic Aorta,Aortic Roots,Arch, Aortic,Ascending Aortas,Junction, Sinotubular,Root, Aortic,Sinotubular Junctions
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective
D016216 Ultrasonography, Prenatal The visualization of tissues during pregnancy through recording of the echoes of ultrasonic waves directed into the body. The procedure may be applied with reference to the mother or the fetus and with reference to organs or the detection of maternal or fetal disease. Fetal Ultrasonography,Prenatal Diagnosis, Ultrasonic,Ultrasonography, Fetal,Diagnosis, Prenatal Ultrasonic,Diagnosis, Ultrasonic Prenatal,Prenatal Ultrasonic Diagnosis,Prenatal Ultrasonography,Ultrasonic Diagnosis, Prenatal,Ultrasonic Prenatal Diagnosis,Diagnoses, Prenatal Ultrasonic,Diagnoses, Ultrasonic Prenatal,Prenatal Diagnoses, Ultrasonic,Prenatal Ultrasonic Diagnoses,Ultrasonic Diagnoses, Prenatal,Ultrasonic Prenatal Diagnoses

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