Abstract

Chronic thromboembolic pulmonary hypertension occurs after one or more episodes of pulmonary embolism. A rigorous diagnostic approach makes it possible to identify this condition, to evaluate its functional and hemodynamic impact and to determine the degree of extension of the vascular damage. Surgical pulmonary endarterectomy in specialized centers remains the treatment of choice in certain cases. Other therapeutic modalities include transplantation, pulmonary angioplasty, and pharmacological treatments with pulmonary vasodilators. We report the case of OA aged 25, followed since the age of 10 for post-embolic PAH. She was hospitalized at this age in pediatrics for the first episode of pulmonary embolism. The initial etiological assessment was without abnormalities. One year after pulmonary embolism, pulmonary pressures were still elevated despite effective anticoagulation. The patient was started on sildenafil at the age of 11. The evolution was stable. At the age of 16, she was referred to us for care. Her cardiovascular assessment revealed suprasystemic PAH; the patient was then put on a combination of sintrom, sildenafil and bosentan. The thrombophilia assessment is without abnormalities. Currently, she is 25 years old, she is in NYHA functional class II on dual therapy, she has presented several right heart decompensations following non-compliance with treatment and dietary deviation. We discussed a surgical endarterectomy at her home but unfortunately the damage is distal and old. Through this case, we will review this entity of PAH by discussing the different modes of expression and the medical, surgical and interventional therapeutic management (pulmonary angiopalstia with a balloon).