Overview of the management of chronic thromboembolic pulmonary arterial hypertension

CTEPH: chronic thromboembolic pulmonary arterial hypertension; PVR: pulmonary vascular resistance; CTED: chronic thromboembolic pulmonary disease; PH: pulmonary hypertension; PEA: pulmonary artery endarterectomy.

* If the diagnosis of CTEPH is not confirmed, patients should be referred back to the PH center for further evaluation.

¶ CTED is a complication of chronic thromboembolism. Patients with CTED have postembolic exercise intolerance and evidence of chronic thromboembolic disease in the absence of resting PH. Whether it is a precursor to CTEPH is unknown.

Δ The response to exercise is measured variably in different centers. Some centers measure it using cycle ergometry while others measure the response while lifting weights or performing another type of exercise.

◊ Surgical management of CTEPH is known as PEA. Most patients undergoing evaluation for PEA have severe hemodynamic abnormalities at rest that almost invariably warrant surgery (eg, PVR of 600 to 1200 dynes per second per cm^–5 [7.5 to 15 Wood units]). Patients with moderate hemodynamic impairment at rest (eg, PVR between 300 and 600 dynes per second per cm^–5 [3.75 and 7.5 Wood units]) still warrant consideration of surgery given the potential for both progression of the hemodynamic abnormality and development of an irreversible vasculopathy. In those with a PVR <300 dynes per second per cm^–5 [<3.75 Wood units]), the benefit is less certain but surgery is still considered if the patient is willing to accept the risk and has symptoms that justify it.