Clinical Research
Congenital Heart Disease
Pulmonary Arterial Thrombosis in Eisenmenger Syndrome Is Associated With Biventricular Dysfunction and Decreased Pulmonary Flow Velocity

https://doi.org/10.1016/j.jacc.2007.04.056Get rights and content
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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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Supported by a grant from the Royal Brompton Hospital Clinical Research Committee. Salary support for Dr. Broberg was provided by the Waring Trust, Royal Brompton Hospital, and an unrestricted grant from Actelion, United Kingdom. Prof. Gatzoulis and the Royal Brompton Adult Congenital Heart Program have received support from the British Heart Foundation, London, England.