Circulation. 2007;115:e632-e634
doi: 10.1161/CIRCULATIONAHA.107.695312
(Circulation. 2007;115:e632-e634.)
© 2007 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Pulmonary Artery Thrombosis and Hemoptysis in Eisenmenger Syndrome
Annette Schophuus Jensen, MD;
Kasper Iversen, MD;
Niels G. Vejlstrup, MD, PhD;
Lars Sondergaard, MD, MDSc
From the Department of Cardiology, Rigshospitalet, Copenhagen, Denmark.
Correspondence to Dr Annette Schophuus Jensen, Department of Cardiology B 2014, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark. E-mail asjensen{at}dadlnet.dk
A 37-year-old man with Eisenmenger syndrome caused by a large ventricular septum defect was admitted to the hospital with small-volume hemoptysis, cough, and left-sided chest pain for several weeks. For several years he had been classified as functional-class II, his oxygen saturation was in the 
85%, hemoglobin was 11.0 mmol/L, and echocardiography visualized a mild tricuspid regurgitation with a gradient of 103 mm Hg (maximum). He had experienced several episodes of minor hemoptysis in the past. There was no previous history of arrhythmias or thromboembolic events.
On admission, his oxygen saturation was 82% and temperature 38.5°C. Blood samples revealed a slightly elevated C-reactive protein level of 16 mg/L (reference <10 mg/L) but normal white blood cell count. D-dimer was markedly elevated at 7.3 mg/L (reference <0.5 mg/L). Platelet count was normal. Chest x-ray showed massive dilated pulmonary arteries (Figure 1) confirmed by spiral computed tomography, which in addition revealed in situ circumferential mural thrombosis with calcification (Figure 2). Furthermore, in the left lung 2 minor infarcts were detected.
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Figure 2. Computed tomography pulmonary angiography reveals giant pulmonary arteries with mural thrombosis and calcifications.
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Because hemoptysis may be caused by pulmonary infarction secondary to thrombosis,1 warfarin treatment was instigated with the goal of international normalized ratio 2.0 to 3.0. Within days the hemoptysis stopped, and the patient recovered to his previous functional status. Warfarin treatment was continued after discharge. However, 2 years later he died suddenly as a result of a massive hemoptysis.
Hemoptysis occurs in the majority of patients with Eisenmenger syndrome2 and has been reported as the cause of death in 11% to 29% of patients with Eisenmenger syndrome.2,3 Furthermore, pulmonary artery thrombosis is found in 21% to 29% of patients with Eisenmenger syndrome.4,5 The coexistence of hemoptysis and pulmonary thrombosis poses a difficult clinical dilemma. Although anticoagulation seems logical for both prevention and treatment of thrombosis, it may be a double-edged sword that increases the risk of fatal bleeding. Because necropsy was not performed, dissection of the pulmonary aneurysm cannot be ruled out as the cause of the fatal bleeding. However, even in pulmonary artery dissection, successful urgent heart-lung transplantation has been reported.6 Thus there is not enough data on the role of anticoagulation in the case of pulmonary artery thrombosis and hemoptysis, a topic that clearly needs to be addressed in clinical trials.
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Disclosures
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None.
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References
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- Wood P. The Eisenmenger syndrome or pulmonary hypertension with reverse central shunt. BMJ. 1958; 2: 701–709, 755–762.[Medline]
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- Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, Stone S. Eisenmenger syndrome: factors relating to deterioration and death. Eur Heart J. 1998; 19: 1845–1855.[Abstract/Free Full Text]
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- Silversides CK, Granton JT, Konen E, Hart MA, Webb GD, Therrien J. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol. 2003; 42: 1982–1987.[Abstract/Free Full Text]
- Tonder N, Kober L, Hassager C. Pulmonary artery dissection in a patient with Eisenmenger syndrome treated with heart and lung transplantation. Eur J Echocardiogr. 2004; 5: 228–230.[CrossRef][Medline]
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Circulation 2007 115: 3039.
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