Chronic Thromboembolic Pulmonary Hypertension in Hospitalized Patients: An Interprofessional Approach to Diagnosis, Pharmacologic Management, and Nursing Care

Background: Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare but life-threatening condition classified as Group 4 pulmonary hypertension. It arises from persistent obstruction of the pulmonary arteries by organized thromboembolic material, leading to increased pulmonary vascular resistance, progressive pulmonary hypertension, and right heart failure. CTEPH is a potential sequela of acute pulmonary embolism, though a significant number of patients have no prior embolic history. Aim: This article synthesizes the essential knowledge for the diagnosis and management of CTEPH, emphasizing the critical need for a systematic, interprofessional approach to improve patient outcomes. It aims to outline the pathophysiological mechanisms, diagnostic pathway, and comprehensive treatment strategies. Methods: A stepwise diagnostic evaluation is recommended for at-risk patients, beginning with transthoracic echocardiography and utilizing ventilation-perfusion (V/Q) scanning as a highly sensitive screening tool. Confirmation requires right heart catheterization for hemodynamic assessment and imaging via computed tomography pulmonary angiography (CTPA) or pulmonary angiography to define the extent and accessibility of thromboembolic obstructions. Results: Pulmonary endarterectomy (PEA) is the cornerstone curative treatment for operable patients, significantly improving survival and hemodynamics. For inoperable cases or those with residual hypertension, balloon pulmonary angioplasty (BPA) and targeted medical therapy (notably the soluble guanylate cyclase stimulator riociguat) are effective alternatives. Lifelong anticoagulation is mandatory for all patients to prevent disease recurrence and progression. Conclusion: CTEPH is a complex disorder whose management hinges on early diagnosis within a specialized multidisciplinary team to determine the optimal treatment pathway among surgical, interventional, and medical options.