Aiello Vera Demarchi, Thomaz Ana Maria, Pozzan Geanete, Lopes Antonio Augusto
Laboratory of Pathology, Heart Institute (InCor), São Paulo University Medical School, São Paulo, Brazil.
Pediatr Pulmonol. 2014 Mar;49(3):E82-5. doi: 10.1002/ppul.22889. Epub 2013 Sep 9.
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.
