Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is categorized as group IV in the WHO classification for pulmonary hypertension. The disease requires a very low index of suspicion for identification and needs a special diagnostic approach utilizing clinical, radiological, and hemodynamic tools. As CTEPH is potentially curable, all efforts should be consumed to reach the accurate diagnosis and subsequently evaluated for operability. Although pulmonary endarterectomy (PEA) is the only curative tool so far, recent updates concerning medical and interventional therapy have made significant advances in inoperable patients. In this review, we provide a detailed discussion on diagnostic algorithm, surgical operability criteria, PEA, and the medical therapy.