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Capillary hemangiomatosis like‐lesions in lung biopsies from children with congenital heart defects
Author(s) -
Aiello Vera Demarchi,
Thomaz Ana Maria,
Pozzan Geanete,
Lopes Antonio Augusto
Publication year - 2014
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.22889
Subject(s) - medicine , pulmonary hypertension , lung , lung biopsy , lesion , biopsy , heart disease , lung disease , respiratory disease , pathology , cardiology , radiology
Summary 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. Pediatr Pulmonol. 2014; 49:E82–E85. © 2013 Wiley Periodicals, Inc.