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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Pulmonary Arterial Thrombosis in Eisenmenger Syndrome Is Associated With Biventricular Dysfunction and Decreased Pulmonary Flow Velocity

Craig S. Broberg, MD^_*,,_*, Masuo Ujita, MD, Sanjay Prasad, MD, Wei Li, MD, PhD^_*, Michael Rubens, FRCR, Bridget E. Bax, PhD^||, Simon J. Davidson, FIBMS CSci^¶, Beatriz Bouzas, MD^_*, J. Simon R. Gibbs, MD^#, John Burman, MD^¶ and Michael A. Gatzoulis, MD, PhD^_*

^_* Adult Congenital Heart Disease Centre, Royal Brompton Hospital and National Heart Lung Institute, Imperial College School of Medicine, London, England
Division of Cardiology, Oregon Health and Sciences University, Portland, Oregon
Department of Radiology, Royal Brompton Hospital and National Heart Lung Institute, Imperial College School of Medicine, London, England
Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital and National Heart Lung Institute, Imperial College School of Medicine, London, England
^|| Child Health, Department of Clinical Developmental Sciences, St. George’s, University of London, London, England
^¶ Department of Haematology, Royal Brompton Hospital and National Heart Lung Institute, Imperial College School of Medicine, London, England
^# Hammersmith Hospital, London, England.

Manuscript received January 3, 2007; revised manuscript received April 10, 2007, accepted April 15, 2007.

^_* Reprint requests and correspondence: Dr. Craig S. Broberg, UHN 62, Division of Cardiology, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Portland, Oregon 97239. (Email: brobergc{at}ohsu.edu).

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.

Abbreviations and Acronyms   ANP = atrial natriuretic peptide   BNP = brain natriuretic peptide   CI = confidence interval   CMR = cardiovascular magnetic resonance   CTPA = computed tomography pulmonary angiogram   LPA = left pulmonary artery   LV = left ventricle/ventricular   MPA = main pulmonary artery   OR = odds ratio   PA = pulmonary artery   RPA = right pulmonary artery   RV = right ventricle/ventricular   RVOT = right ventricular outflow tract   VSD = ventricular septal defect

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