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(Chest. 2002;121:286-289.)
© 2002 American College of Chest Physicians

Unilateral Chronic Thromboembolic Pulmonary Disease Associated With Combined Inherited Thrombophilia*

Klaus Laczika, MD; Irene Martha Lang, MD; Peter Quehenberger, MD; Christine Mannhalter, MD; Manfred Muhm, MD; Walter Klepetko, MD and Paul Alexander Kyrle, MD

* From the Intensive Care Unit (Dr. Laczika) and the Division of Hematology (Dr. Kyrle), the Department of Internal Medicine I, the Department of Internal Medicine II (Dr. Lang), Division of Cardiology, the Clinical Institute of Laboratory Medicine (Drs. Quehenberger and Mannhalter), Divisions of Hematology and Molecular Biology, the Department of Cardiothoracic/Vascular Anesthesia (Dr. Muhm), and the Department of Cardiothoracic Surgery (Dr. Klepetko), Vienna University Hospital, Vienna, Austria.

Correspondence to: Klaus Laczika, MD, Department of Internal Medicine I, Division of Critical Care Waehringer Guertel 18–20 A-1090 Vienna, Austria; e-mail: Klaus.Laczika{at}akh-wien.ac.at

Chronic thromboembolic pulmonary hypertension (CTEPH) is considered to be an extreme variant of pulmonary thromboembolism. The underlying mechanisms for the failure of thrombus resolution are still unclear. In looking for inherited thrombophilia, an association with a lupus anticoagulant has been described repeatedly, and single cases of anticoagulant deficiencies (ie, antithrombin [AT], protein C, and protein S) have been reported. We describe a young patient with type I AT deficiency, the heterozygous prothrombin G20210A mutation, and unilateral chronic thromboembolic pulmonary disease presenting after a single thrombotic event. Pulmonary vascular patency was restored successfully by surgical pulmonary thromboendarterectomy. This case is unique because unilateral CTEPH is extremely uncommon, and it illustrates the severe clinical sequelae of the cosegregation of inherited thrombophilic defects. (CHEST 2002; 121:286–289)

Key Words: compound inherited thrombophilia • unilateral thromboembolic pulmonary hypertension






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