Chronic thromboembolic pulmonary hypertension: a review of risk factors, management and current challenges

ABSTRACT

Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) is an under-diagnosed complication of acute pulmonary embolism (PE), whereby there is incomplete resolution of pulmonary emboli, which then become organized into obstructive fibrotic material with an associated small vessel vasculopathy. CTEPH is diagnosed by abnormal lung perfusion despite 3-months of oral anticoagulation with persistent pulmonary artery thromboembolic/ fibrotic material confirmed on cross sectional imaging and pulmonary hypertension on right heart catheterization. Without treatment the prognosis is bleak, but there are now three treatment modalities that are effective in treating patients with CTEPH: pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA) and medical therapy.

Areas Covered

We provide a comprehensive review of the literature with particular emphasis on the epidemiology, pathophysiology, diagnosis, and the established and emerging treatments of CTEPH.

Expert opinion

It is important to recognize that CTEPH is complication of an acute PE as there are effective and sometimes curative treatments available. In European and North American practice, PEA surgery remains the cornerstone of therapy in patients with CTEPH and accessible disease without significant comorbidity. For those patients with a disease burden that is inaccessible or fails to justify the risk of major surgery, initiating medical therapy and considering BPA is appropriate. Those with persistent pulmonary hypertension after PEA should also receive medical therapy and be considered for BPA or re-do endarterectomy. The available therapies are not necessarily mutually exclusive, and the treatment decision is often subjective and individualized, based on the local available expertise. Further research is needed to understand the optimal treatment strategy for patients and in particular to identify ‘responders’ and assess the synergies between these three treatment strategies.

KEYWORDS:

• CTEPH - chronic thromboembolic pulmonary hypertension
• PEA - pulmonary endarterectomy
• BPA - balloon pulmonary angioplasty

Article Highlights

• CTEPH is an under-recognised and potentially reversible cause of pulmonary hypertension.

• A history of venous thromboembolism strongly favours a diagnosis of CTEPH.

• A ventilation perfusion (V/Q) scan is the preferred initial screening test for CTEPH.

• Patients should receive life-long vitamin K antagonist (warfarin) or direct oral anticoagulant (DOAC).

• A multi-disciplinary team (MDT) assessment should consider the three treatment modalities: pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA) or medical therapy.

• Treatment consideration is often subjective and the choice offered will depend on the local expertise available.

• Optimising treatment selection for patients with CTEPH by assessing predictors of response and further work on the synergy between the three treatments available is needed to further improve outcomes of patients with CTEPH.

Declarations of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.