The Virtual Hospital: ElectricPE: The Diagnosis of Pulmonary Embolism: Collagen Vascular Disease

ElectricPE: The Diagnosis of Pulmonary Embolism: Case Study 8

Collagen Vascular Disease

Jeffrey R. Galvin, M.D. and James J. Choi, B.S.
Peer Review Status: Internally Reviewed

This case is a 57 year old male with a long history of scleroderma who presented to this hospital with a history of severe chronic dyspnea that had worsened over the last 8 months. His lung disease had been attributed to his underlying collagen-vascular disease. One month prior to admission he had undergone a right heart catheterization and pulmonary angiogram that revealed pulmonary hypertension (PA pressure 85/41 mm Hg). The pulmonary angiogram reported normal vessels in the left lung. The vessels in the right lung were not imaged due to Òtechnical difficulties placing the catheter in the right lung.

The chest radiograph demonstrated an enlarged heart with a prominent right hilum that was felt to represent an enlarged right pulmonary artery.

A chest CT was requested to investigate the enlarged right hilum. Mediastinal windows from the chest CT revealed (A) right atrial enlargement and a thickened (B) right ventricular wall consistent with the patients known pulmonary hypertension. In addition, a large calcified clot (C) was identified filling the right pulmonary artery. A filling defect in the right atrium was also identified. (D)

Case Assessment
This is a 57 year old male with scleroderma and severe chronic dyspnea. It was assumed that his lung disorder was related to collagen vascular disease. A retrospective review of the patients history identified a sudden worsening in his dyspnea approximately one year prior to this admission. The diagnosis of pulmonary embolus was considered and then dismissed after a "low probability" lung scan.

1. It is often difficult to make the diagnosis of pulmonary embolus when the patient has another underlying disease that can also affect the lungs.

2. The ventilation/perfusion scan can be normal when there are large central clots. That, however, is a rare occurrence and in most cases a normal ventilation/perfusion is adequate to rule out the diagnosis.

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