Korean Circ J.  2012 Nov;42(11):772-775. 10.4070/kcj.2012.42.11.772.

Pulmonary Arterial Thrombosis in a Patient With an Atrial Septal Defect and Eisenmenger Syndrome

• Lee CW
• Huang SS
• Huang PH

• KMID: 2224978
• DOI: http://doi.org/10.4070/kcj.2012.42.11.772

Abstract

Pulmonary hypertension is characterized by elevated pulmonary arterial pressure and secondary right ventricular failure. A thromboembolic occlusion of the proximal or distal pulmonary vasculature results in chronic thromboembolic pulmonary hypertension. We report an uncommon case that presented to our hospital with symptoms of dyspnea on exertion over 2 years. The patient had been treated for profound pulmonary thrombosis and right ventricular failure with adequate anticoagulation and sildenafil. Our echocardiography disclosed a large atrial septal defect with severe pulmonary hypertension and right ventricular failure. A diagnosis of Eisenmenger syndrome with pulmonary artery thrombosis was made. Although Eisenmenger syndrome with pulmonary thrombosis is well described in western societies, a huge pulmonary thrombosis is seldom reported in eastern countries. Profound pulmonary thrombosis may obfuscate the actual diagnosis of pulmonary artery hypertension with underlying congenital heart disease. A physical examination and echocardiography are essential in patients with pulmonary hypertension.

Keyword

Pulmonary embolism; Eisenmenger syndrome

MeSH Terms

Arterial Pressure
Dyspnea
Echocardiography
Eisenmenger Complex
Heart Diseases
Heart Septal Defects, Atrial
Humans
Hypertension
Hypertension, Pulmonary
Physical Examination
Piperazines
Pulmonary Artery
Pulmonary Embolism
Purines
Sulfones
Thrombosis
Sildenafil Citrate
Piperazines
Purines
Sulfones

Figure

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  Fig. 1 Pulmonary arterial thrombosis in atrial septal defect with Eisenmenger syndrome. A: chest radiography revealed marked enlargement and lobulated contour of the hilar shadows of bilateral pulmonary arteries. B: electrocardiogram revealed right axis deviation and bi-ventricular hypertrophy with strain pattern. C: thoracic computed tomographic (CT) scan showed thrombus in the aneurysmal dilatation of proximal pulmonary arteries. D: the transthoracic echocardiography showed a large secundum type atrial septal defect (ASD).

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