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Familial Pulmonary Capillary Hemangiomatosis Resulting in Primary Pulmonary Hypertension

David Langleben, MD; James M. Heneghan, MD; Adrian P. Batten, MD; Nai-San Wang, MD; Naomi Fitch, MD, PhD; Robert D. Schlesinger, MD; Albert Guerraty, MD; and Jean L. Rouleau, MD
[+] Article, Author, and Disclosure Information

Grant Support: Partial support by the Sir Mortimer B. Jewish General Hospital Cardiology Research Fund and the Women's Auxiliary. Dr. Langleben is a Research Scholar of the Canadian Heart Foundation.

Requests for Reprints: Dr. David Langleben, Room A-707, The Sir Mortimer B. Davis Jewish General Hospital, 3755 Chemin de la Côte Ste-Catherine, Montreal, QC, H3T 1E2, Canada.

Current Author Addresses: Drs. Langleben and Schlesinger: Division of Cardiology, Department of Medicine and Lady Davis Institute, The Sir Mortimer B. Davis Jewish General Hospital, Montreal, QC H3T 1E2.

Drs. Heneghan, Batten, and Wang: Department of Pathology, Royal Victoria Hospital, Montreal, QC H3A 1A1.

Dr. Fitch: Division of Genetics, Department of Medicine and Lady Davis Institute, The Sir Mortimer B. Jewish General Hospital, Montreal, QC H3T 1E2.

Dr. Guerraty: Department of Cardiovascular Surgery, Royal Victoria Hospital, Montreal, QC H3A 1A1.

Dr. Rouleau: Department of Cardiology, Hôpital du Sacre-Coeur, Montreal, QC H4J 1C5.

© 1988 American College of PhysiciansAmerican College of Physicians

Ann Intern Med. 1988;109(2):106-109. doi:10.7326/0003-4819-109-2-106
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We describe the first cases of familial pulmonary capillary hemangiomatosis, a disorder in which capillaries in the lungs proliferate. Three siblings died from primary pulmonary hypertension. One developed pulmonary congestion preterminally after vasodilator treatment. The inheritance pattern seems autosomal recessive. Lung specimens obtained in two siblings showed extensive pulmonary capillary hemangiomatosis, with normal capillaries proliferating into veins and alveoli. Including our patients, four of the nine patients with pulmonary capillary hemangiomatosis have presented with the clinical picture of primary pulmonary hypertension. Thus, pulmonary capillary hemangiomatosis should be considered as a histologic pattern of primary pulmonary hypertension. Most other cases of pulmonary capillary hemangiomatosis have been similar to pulmonary venoocclusive disease. Recently, disorders involving the proliferation of cytologically normal capillaries have been termed angiogenic diseases. Pulmonary capillary hemangiomatosis may be an angiogenic disease.





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