Occlusive venopathy phenotype in hereditary pulmonary arterial hypertension
Arq Bras Cardiol. 2011 Jul;97(1):e8-10.
doi: 10.1590/s0066-782x2011000900019.
[Article in
English,
Portuguese,
Spanish]
Affiliation
- 1 Instituto do Coração, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
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.
MeSH terms
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Adult
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Biopsy
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Familial Primary Pulmonary Hypertension
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Fatal Outcome
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Humans
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Hypertension, Pulmonary / drug therapy
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Hypertension, Pulmonary / genetics
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Hypertension, Pulmonary / pathology*
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Lung / pathology*
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Male
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Phenotype
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Piperazines / administration & dosage
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Pulmonary Veno-Occlusive Disease / pathology*
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Purines / administration & dosage
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Sildenafil Citrate
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Sulfones / administration & dosage
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Vasodilator Agents / administration & dosage
Substances
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Piperazines
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Purines
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Sulfones
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Vasodilator Agents
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Sildenafil Citrate