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 |
|
This article has been cited by other articles:
|
|
|
|
 
K. Dimopoulos and G. Giannakoulas
Chapter 9 Eisenmenger complex
OCL Pulmonary Hypertension,
December 1, 2011;
1(1):
med-9780199572632-chapter - med-9780199572632-chapter.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
Endorsed by the Association for European Paediatri, Authors/Task Force Members, H. Baumgartner, P. Bonhoeffer, N. M. S. De Groot, F. de Haan, J. E. Deanfield, N. Galie, M. A. Gatzoulis, C. Gohlke-Baerwolf, et al.
ESC Guidelines for the management of grown-up congenital heart disease (new version 2010): The Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC)
Eur. Heart J.,
December 1, 2010;
31(23):
2915 - 2957.
[Full Text]
[PDF]
|
|
|
|
|
|
|
The Task Force for the Diagnosis and Treatment of, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al.
Guidelines for the diagnosis and treatment of pulmonary hypertension
Eur. Respir. J.,
December 1, 2009;
34(6):
1219 - 1263.
[Full Text]
[PDF]
|
|
|
|
|
|
|
Authors/Task Force Members, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J.-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al.
Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)
Eur. Heart J.,
October 2, 2009;
30(20):
2493 - 2537.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Beghetti and N. Galie
Eisenmenger Syndrome: A Clinical Perspective in a New Therapeutic Era of Pulmonary Arterial Hypertension
J. Am. Coll. Cardiol.,
March 3, 2009;
53(9):
733 - 740.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. Bonderman, J. Jakowitsch, B. Redwan, H. Bergmeister, M.-K. Renner, H. Panzenbock, C. Adlbrecht, A. Georgopoulos, W. Klepetko, M. Kneussl, et al.
Role for Staphylococci in Misguided Thrombus Resolution of Chronic Thromboembolic Pulmonary Hypertension
Arterioscler Thromb Vasc Biol,
April 1, 2008;
28(4):
678 - 684.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
