title: Pulmonary veno-occlusive disease: Two children with gradual disease progression.
abstract: Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis are rare forms of pulmonary vascular disease. We report two cases of affected children who had evidence of pulmonary hypertension 3-5 years before developing radiographic findings of pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis. Both patients experienced a moderate decrease in pulmonary arterial pressure during acute vasodilator testing. Both patients experienced an improvement in six-minute walk performance without an increase in pulmonary edema when treated with targeted therapy for pulmonary hypertension. In some patients, pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis may progress slowly over a period of months to years. A favorable acute vasodilator response may identify patients who will tolerate, and demonstrate transient clinical improvement with, medical therapy.
authors.affiliation: University of Utah Department of Pediatrics, 81 North Mario Capecchi Drive, Salt Lake City, UT 84113, USA.
authors.forename: Ronald W
authors.initials: RW
authors.lastname: Day
authors.affiliation: University of Utah Department of Pathology, 15 North Medical Drive, Suite 1100, Salt Lake City, UT 84132, USA.
authors.forename: Parker W
authors.initials: PW
authors.lastname: Clement
authors.affiliation: University of Utah Department of Pediatrics, 81 North Mario Capecchi Drive, Salt Lake City, UT 84113, USA.
authors.forename: Aimee O
authors.initials: AO
authors.lastname: Hersh
authors.affiliation: Vascular Biology Program of Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.
authors.forename: Susan M
authors.initials: SM
authors.lastname: Connors
authors.affiliation: ARUP Institute for Clinical and Experimental Pathology, 500 Chipeta Way, Salt Lake City, UT 84108, USA.
authors.forename: Kelli L
authors.initials: KL
authors.lastname: Sumner
authors.affiliation: ARUP Institute for Clinical and Experimental Pathology, 500 Chipeta Way, Salt Lake City, UT 84108, USA.
authors.forename: D Hunter
authors.initials: DH
authors.lastname: Best
authors.affiliation: University of Utah Department of Pathology, 15 North Medical Drive, Suite 1100, Salt Lake City, UT 84132, USA.
authors.forename: Mouied
authors.initials: M
authors.lastname: Alashari
keywords: PCH, Pulmonary Capillary Hemangiomatosis; PVOD, pulmonary veno-occlusive disease; Pulmonary arterial hypertension; Pulmonary capillary hemangiomatosis; Pulmonary veno-occlusive disease