About 2-4% of all patients with pulmonary embolism do not resolve their acute thrombus (blood clot). They then enter a chronic phase where the vascular pressures in the lung elevates, putting a strain on the right ventricle and eventually causing it to fail. Many patients with chronic thromboembolic pulmonary hypertension (CTEPH) are unaware they had an acute pulmonary embolism. CTEPH is a common cause of unexplained pulmonary hypertension (elevated vascular pressure in the lungs).
Common CTEPH symptoms include chest pain, palpitations, syncope and shortness of breath. Patients often have signs of right heart failure, including leg swelling. Any patient whose symptoms do not resolve within three months of an acute pulmonary embolism needs to be checked for CTEPH by a heart doctor.
The initial screening test to diagnose chronic thromboembolic pulmonary hypertension is an echocardiogram to look for evidence of elevated lung pressures and right heart failure. Any patient with unexplained pulmonary hypertension should undergo a VQ scan. If the scan shows normal perfusion, CTEPH is ruled out. If the VQ scan is positive, patients should receive a CT angiogram and a right heart catheterization.
Chronic thromboembolic pulmonary hypertension treatment requires care from a multidisciplinary team. The entire team reviews each patient to attain consensus before management. CTEPH is surgically curable. In patients who have favorable anatomy, a procedure called pulmonary thromboendarterectomy (PTE) can reduce pressures and reverse the patient’s hypertension symptoms. Patients who have residual hypertension after surgery, and those who are not candidates for surgery, are managed by pulmonary hypertension specialists. There are now medications designed specifically to treat patients with chronic thromboembolic pulmonary hypertension.