Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a specific type of pulmonary hypertension (PH) and the major component of Group 4 pulmonary hypertension (PH). It is caused by pulmonary vasculature obstruction that leads to a progressive increase in pulmonary vascular resistance and, ultimately, to failure of the right ventricle. Pulmonary thromboendarterectomy (PEA) is the only definitive therapy, so a timely diagnosis and early referral to a specialized PEA center to determine candidacy is prudent for a favorable outcome. Percutaneous balloon pulmonary angioplasty (BPA) has a potential role in patients unsuitable for PEA. Medical therapy with riociguat is the only PH-specific medical therapy currently approved for the treatment of inoperable or persistent CTEPH. This review article aims to revisit CTEPH succinctly with a review of prevailing literature.
Highlights
If the V/Q scan is suggestive of Chronic thromboembolic pulmonary hypertension (CTEPH), additional testing is required with right heart catheterization (RHC) and pulmonary angiography
Lifelong anticoagulation is recommended for all patients with CTEPH [1,2]
Balloon Angioplasty (BPA) is mainly used in patients with inoperable CTEPH [40,41]
Summary
The 6th world symposium on pulmonary hypertension (PH) in 2018 has defined PH as an elevated mean pulmonary artery pressure (mPAP) ≥ 20 mmHg at rest, pulmonary capillary wedge pressure < 15 mmHg, and pulmonary vascular resistance (PVR) ≥ 3 Wood. PH can be further subdivided into 5 groups (Table 1). Chronic thromboembolic pulmonary hypertension (CTEPH) belongs to Group 4 of the PH classification. CTEPH is defined as an obstruction of the pulmonary arterial vasculature secondary to single or recurrent episodes of pulmonary embolism (PE) [2]. It is a distinct and infrequently diagnosed entity of PH that is progressive and can be fatal if left untreated
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