Off-label use of PDE-5 inhibitors and endothelin receptor antagonists may provide clinical benefit in patients with severe thromboembolic pulmonary hypertension who are unfit for surgical thrombendarterectomy.
We present a case of severe thromboembolic pulmonary hypertension in a patient with history of recurrent deep vein thrombosis and pulmonary restrictive disease due to pulmonary and vertebral tuberculosis in young adulthood. He was considered not eligible in the National Program for Primary Pulmonary Hypertension, being referred for thoracic surgery, but he was considered unfit for thrombendarterectomy. Despite guidelines, we administered him specific medical therapy (phosphodiesterase-5 inhibitors and endothelin receptor antagonists). His clinical evolution was satisfactory, with increasing effort tolerance and decreasing need for ambulatory oxigenotherapy.
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Nedelcu et al. (2017) studied this question.
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