Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.

TitleAsymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.
Publication TypeJournal Article
Year of Publication2018
AuthorsO'Byrne ML, Cross RR, Martin GR
JournalCardiol Young
Volume28
Issue2
Pagination329-333
Date Published2018 Feb
ISSN1467-1107
Abstract

An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called "scimitar vein" without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.

DOI10.1017/S1047951117001822
Alternate JournalCardiol Young
PubMed ID28847332
Grant ListK23 HL130420 / HL / NHLBI NIH HHS / United States