title: Capillary hemangiomatosis like-lesions in lung biopsies from children with congenital heart defects.
abstract: The morphological findings in the lung vessels of children with pulmonary arterial hypertension associated with uncorrected congenital cardiac shunts may be qualitatively and quantitatively assessed by lung biopsy in selected cases. Among 297 lung biopsies collected over a period of 11 years two cases were detected presenting the typical findings of pulmonary capillary hemangiomatosis, a rare disorder usually associated with pulmonary veno-occlusive disease. We report the clinical and histological findings and discuss about the impact of this unexpected and not-previously described associated lesion on the prognosis and on the therapeutic strategy in patients with pulmonary hypertension associated with congenital heart disease.
authors.affiliation: Laboratory of Pathology, Heart Institute (InCor), São Paulo University Medical School, São Paulo, Brazil.
authors.forename: Vera Demarchi
authors.initials: VD
authors.lastname: Aiello
authors.forename: Ana Maria
authors.initials: AM
authors.lastname: Thomaz
authors.forename: Geanete
authors.initials: G
authors.lastname: Pozzan
authors.forename: Antonio Augusto
authors.initials: AA
authors.lastname: Lopes
mesh_terms: D002675:Child, Preschool; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications; D006345:Heart Septal Defects, Ventricular / Q000150:complications; D018324:Hemangioma, Capillary / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D008168:Lung / Q000000981:diagnostic imaging* / Q000473:pathology; D008175:Lung Neoplasms / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D011877:Radionuclide Imaging
keywords: congenital heart defects; pulmonary capillary hemangiomatosis; pulmonary hypertension; pulmonary vascular disease