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Pulmonary arterial thrombosis in Eisenmengrer syndrome is associated with biventricular dysfunction decreased pulmonary flow velocity

Broberg, CS; Ujita, M; Prasad, S; Li, W; Rubens, M; Bax, BE; Davidson, SJ; Bouzas, B; Gibbs, JS; Burman, J; et al. Broberg, CS; Ujita, M; Prasad, S; Li, W; Rubens, M; Bax, BE; Davidson, SJ; Bouzas, B; Gibbs, JS; Burman, J; Gatzoulis, MA (2007) Pulmonary arterial thrombosis in Eisenmengrer syndrome is associated with biventricular dysfunction decreased pulmonary flow velocity. JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 50 (7). 634 - 642. ISSN 0735-1097 https://doi.org/10.1016/j.jacc.2007.04.056
SGUL Authors: Bax, Bridget Elizabeth

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Abstract

Objectives: This study sought to determine what factors are associated with pulmonary artery thrombi in Eisenmenger patients. Background: Pulmonary artery thrombosis is common in Eisenmenger syndrome, although its underlying pathophysiology is poorly understood. Methods: Adult patients with Eisenmenger syndrome underwent computed tomography pulmonary angiography, cardiac magnetic resonance imaging, and echocardiography. Measurement of ventricular function, pulmonary artery size, and pulmonary artery blood flow were obtained. Hypercoagulability screening and platelet function assays were performed. Results: Of 55 consecutive patients, 11 (20%) had a detectable thrombus. These patients were older (p = 0.032), but did not differ in oxygen saturation, hemoglobin, or hematocrit from those without thrombus. Right ventricular ejection fraction by magnetic resonance imaging was lower in those with thrombus (0.41 ± 0.15 vs. 0.53 ± 0.13, p = 0.017), as was left ventricular ejection fraction (0.48 ± 0.12 vs. 0.60 ± 0.09, p = 0.002), a finding corroborated by tissue Doppler and increased brain natriuretic peptide. Those with thrombus also had a larger main pulmonary artery diameter (48 ± 14 mm vs. 38 ± 9 mm, p = 0.007) and a lower peak systolic velocity in the pulmonary artery (p = 0.003). There were no differences in clotting factors, platelet function, or bronchial arteries between groups. Logistic regression showed pulmonary artery velocity to be independently associated with thrombosis. Conclusions: Pulmonary arterial thrombosis among adults with Eisenmenger syndrome is common and relates to older age, biventricular dysfunction, and slow pulmonary artery blood flow rather than degree of cyanosis or coagulation abnormalities. Further work to define treatment efficacy is needed.

Item Type: Article
Additional Information: NOTICE: this is the author’s version of a work that was accepted for publication in the Journal of the American College of Cardiology. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control mechanisms may not be reflected in this document. Changes may have been made to this work since it was submitted for publication. A definitive version was subsequently published in the Journal of the American College of Cardiology [50, 7 (14 Aug 2007)] 10.1016/j.jacc.2007.04.056
Keywords: Adult, Age Factors, Blood Flow Velocity, Cross-Sectional Studies, Eisenmenger Complex, Female, Hemoptysis, Humans, Male, Middle Aged, Prospective Studies, Pulmonary Artery, Risk Factors, Thrombosis, Ventricular Function, Science & Technology, Life Sciences & Biomedicine, Cardiac & Cardiovascular Systems, Cardiovascular System & Cardiology, CONGENITAL HEART-DISEASE, CARDIOVASCULAR MAGNETIC-RESONANCE, ADULTS, HYPERTENSION, HEMOPTYSIS, PRESSURE, SURVIVAL, DEFECTS, ATRIAL, DEATH
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cell Sciences (INCCCS)
Journal or Publication Title: JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
ISSN: 0735-1097
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Dates:
DateEvent
14 August 2007Published
Web of Science ID: WOS:000249049300011
URI: http://openaccess.sgul.ac.uk/id/eprint/100456
Publisher's version: https://doi.org/10.1016/j.jacc.2007.04.056

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