title: Occlusive venopathy phenotype in hereditary pulmonary arterial hypertension.
abstract: A 33-year-old male with severe hereditary pulmonary arterial hypertension had a confirmed diagnosis of occlusive venopathy and microvasculopathy. He remained stable for three and a half years on oral sildenafil, 75 mg t.i.d. (six-minute walked distance of 375 m vs 105 m at baseline), but required addition of bosentan (125 mg b.i.d.), subsequently. Despite the fatal outcome at five years post-diagnosis, the observations suggest a potential usefulness of vasodilators as a bridge for lung transplant in selected cases with significant venous/capillary involvement. The occurrence of veno-occlusive and capillary lesions in the familial form of pulmonary arterial hypertension reinforces the difficulties with the current classification of the disease.
authors: Instituto do Coração, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
authors: Sonia Meiken
authors: SM
authors: Franchi
authors: Vera D
authors: VD
authors: Aiello
authors: Antonio Augusto
authors: AA
authors: Lopes
mesh_terms: D000328:Adult; D001706:Biopsy; D065627:Familial Primary Pulmonary Hypertension; D017809:Fatal Outcome; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008168:Lung / Q000473:pathology*; D008297:Male; D010641:Phenotype; D010879:Piperazines / Q000008:administration & dosage; D011668:Pulmonary Veno-Occlusive Disease / Q000473:pathology*; D011687:Purines / Q000008:administration & dosage; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage; D014665:Vasodilator Agents / Q000008:administration & dosage
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate