10-year success story in CTEPH treatment: breaking the myth of rareness

Unlike acute pulmonary embolism (PE), the resolution of thrombi is ineffective in chronic thromboembolic pulmonary hypertension (CTEPH), leading to reorganisation and fibrotic changes within the pulmonary arteries. The authors report the case of a man in his 60s with polycythemia vera, under warfarin, following an acute PE. He was admitted a year later with right heart failure and haemodynamic instability. Acute over chronic PE caused this severe presentation, confirmed by right heart catheterisation and pulmonary scintigraphy. The challenging diagnosis and management involved transfer to a centre specialised in pulmonary vascular disease. Normalisation of functional and haemodynamic parameters, sustained in 10-year follow-up, was achieved with anticoagulation and triple therapy with prostanoids as a bridge to pulmonary thromboendarterectomy. Targeted medical therapy, not standard at that time, was crucial to recovering conditions for transfer. An individualised approach, integrating multidisciplinary pulmonary hypertension expertise, provides the basis for the best care for CTEPH.