Published online Jul 6, 2020. doi: 10.12998/wjcc.v8.i13.2679
Peer-review started: March 22, 2020
First decision: April 22, 2020
Revised: May 28, 2020
Accepted: June 10, 2020
Article in press: June 10, 2020
Published online: July 6, 2020
Processing time: 106 Days and 13.1 Hours
Chronic thromboembolic pulmonary hypertension (CTEPH) is a complex chronic disease in which pulmonary artery stenosis or obstruction caused by organized thrombus can lead to increased pulmonary artery pressure and pulmonary vascular resistance, ultimately triggering progressive right heart failure and death. Currently, its exact mechanism is not fully understood. Pulmonary endarterectomy (PEA) has immediate effects with low perioperative mortality and satisfactory prognosis in experienced expert centers for CTEPH patients with proximal lesions. Nevertheless, 37% of patients are deemed unsuitable for PEA surgery due to comorbidities and other factors, and nearly half of the operated patients have residual or recurrent pulmonary hypertension. Riociguat is the only approved drug for CTEPH, although its effect is limited. Balloon pulmonary angioplasty (BPA) is a promising alternative treatment for patients with CTEPH. After more than 30 years of development and refinements, emerging evidence has confirmed its role in patients with inoperable CTEPH or residual/recurrent pulmonary hypertension, with acceptable complications and comparable long-term prognosis to PEA. This review summarizes the pathophysiology of CTEPH, BPA history and development, therapeutic principles, indications and contraindications, interventional procedures, imaging modalities, efficacy and prognosis, complications and management, bridging and hybrid therapies, ongoing clinical trials and future prospects.
Core tip: Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious chronic disease with a poor prognosis if left untreated. Pulmonary endarterectomy (PEA) is the only curable treatment of choice for certain CTEPH patients with proximal vessel lesions. Balloon pulmonary angioplasty (BPA) is a promising alternative interventional option for patients with CTEPH, especially those with distal lesions or residual/recurrent pulmonary hypertension after PEA. Herein, we summarize the pathophysiology of CTEPH, the history and development of BPA, therapeutic principles, indications and contraindications, interventional procedures, imaging modalities, efficacy and prognosis, complications and management, bridging and hybrid therapies, ongoing clinical trials and future prospects.