Clinical Updates on the Diagnosis and Management of Chronic Thromboembolic Pulmonary Hypertension

Abstract

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a known sequela after acute pulmonary embolism (PE). It is a debilitating disease, and potentially fatal if left untreated. This review provides a clinically relevant overview of the disease and discusses the usefulness and limitations of the various investigational and treatment options. Methods: A PubMed search on articles relevant to pulmonary embolism, pulmonary hypertension, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, and balloon pulmonary angioplasty were performed. A total of 68 articles were found to be relevant and were reviewed. Results: CTEPH occurs as a result of non-resolution of thrombotic material, with subsequent fibrosis and scarring of the pulmonary arteries. Risk factors have been identified, but the underlying mechanisms have yet to be fully elucidated. The cardinal symptom of CTEPH is dyspnoea on exertion, but the diagnosis is often challenging due to lack of awareness. The ventilation/perfusion scan is recommended for screening for CTEPH, with other modalities (eg. dual energy computed tomography pulmonary angiography) also being utilised in expert centres. Conventional pulmonary angiography with right heart catherisation is important in the final diagnosis of CTEPH. Conclusion: Operability assessment by a multidisciplinary team is crucial for the management of CTEPH, as pulmonary endarterectomy (PEA) remains the guideline recommended treatment and has the best chance of cure. For inoperable patients or those with residual disease post-PEA, medical therapy or balloon pulmonary angioplasty are potential treatment options. Keywords: Balloon pulmonary angioplasty, Chronic thromboembolic pulmonary hypertension, Pulmonary embolism, Pulmonary endarterectomy, Pulmonary hypertension

Pulmonary Embolism (PE) to Chronic Thromboembolic Pulmonary Disease (CTEPD): Findings from a Survey of UK Physicians

“…Diagnostic algorithms for CTEPH recommend an echocardiographic assessment of the probability of PH, assessment of lung perfusion (e.g., using ventilation/perfusion (V/Q) lung scintigraphy) and cardiopulmonary exercise testing (CPET) [ 1 , 8 , 9 , 10 ]. However, CTEPH awareness is low among PE-treating physicians [ 11 , 12 , 13 ], and CTEPH often goes undiagnosed for over a year [ 14 ], negatively impacting patient prognosis, with higher pulmonary artery pressures and an increased risk of death [ 15 ].…”