ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2019) 65 P364 | DOI: 10.1530/endoabs.65.P364

Retrospective analysis of pulmonary venous drainage in 90 patients with Turner syndrome demonstrates abnormalities are common; Is it time to review the guidelines?

Alexander Stockenhuber1, Raj Soundarajan1, Saul Myerson2, Andrew Kelion3, Helen Turner4 & Elizabeth Orchard1

1Adult Congenital Heart Disease, Oxford University Hospital NHS Trust, Oxford, UK; 2Centre for Clinical Magnetic Resonance Research, Oxford University Hospital NHS Trust, Oxford, UK; 3Oxford Cardiac Imaging Centre, Oxford University NHS Foundation Trust, Oxford, UK; 4Department of Endocrinology, Oxford University Hospital NHS Foundation Trust, Oxford, UK

Turner syndrome is a common chromosomal disorder affecting 1 in 2500 life female births. Turner syndrome is associated with congenital cardiovascular malformations of the aortic arch, systemic and pulmonary venous return with reported incidences ranging from 23 to 45%. These vascular malformations cause significant morbidity and mortality with increased incidence of aortic pathology, right heart strain and pulmonary hypertension as a result. In this investigation we retrospectively reviewed CT-chest and C-MR imaging studies of patients with known Turner syndrome who are under regular follow up in the Oxford Turner Syndrome clinic from January 2010 to August 2018. CT scans and C-MR studies were analyzed by a senior clinician for abnormalities in particular anomalous pulmonary return. Out of 102 Turner patients under regular follow up 90 had undergone either C-MR imaging or a CT-chest since their original diagnosis. 22 out of these 90 patients (24.4%) were found to have abnormal venous connections. Three patients (3.3%) were found to have isolated anomalous left upper pulmonary venous return, seven patients (7.7%) were found to have isolated right upper pulmonary venous return while 3 patients (3.3%) were found to have bilateral anomalous upper pulmonary venous return. 3 patients (3.3%) were found to have anomalous azygous connections, 5 patients (5.5%) had left sided SVCs and 1 patient ( 1.1%) had an interrupted IVC. Current international guidelines for the management of Turner syndrome patients suggest transthoracic echocardiography and C-MR imaging at the time of diagnosis but specific imaging of the pulmonary vasculature is not recommended. We suggest that initial cardiovascular imaging should include detailed imaging of the pulmonary veins as abnormalities may lead to right heart dilation and subsequent failure. Thus CT angiography or targeted C-MR venogram to assess for PAVD could be performed in addition to assessment of aortic arch anatomy.

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