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Case Report

Occlusive venopathy phenotype in hereditary pulmonary arterial hypertension

Sonia Meiken Franchi et al. Vera D. Aiello Antonio Augusto Lopes

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.

hereditary pulmonary arterial hypertension
microvasculopathy
Pulmonary Hypertension
pulmonary occlusive venopathy
sildenafil

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Occlusive venopathy phenotype in hereditary pulmonary arterial hypertension

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