Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function

Abstract

The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines on the diagnosis and treatment of pulmonary hypertension (PH) includes a multimodal approach of combinations of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapies to target major vessel pulmonary vascular lesions, and microvasculopathy. Today, BPA of >1700 patients has been reported in the literature from centers in Asia, the US, and also Europe; many more patients have been treated outside literature reports. As BPA becomes part of routine care of patients with CTEPH, benchmarks for safe and effective care delivery become increasingly important. In light of this development, the ESC Working Group on Pulmonary Circulation and Right Ventricular Function has decided to publish a document that helps standardize BPA to meet the need of uniformity in patient selection, procedural planning, technical approach, materials and devices, treatment goals, complications including their management, and patient follow-up, thus complementing the guidelines. Delphi methodology was utilized for statements that were not evidence based. First, an anatomical nomenclature and a description of vascular lesions are provided. Second, treatment goals and definitions of complete BPA are outlined. Third, definitions of complications are presented which may be the basis for a standardized reporting in studies involving BPA. The document is intended to serve as a companion to the official ESC/ERS guidelines.

Keywords: Balloon pulmonary angioplasty; Chronic thromboembolic pulmonary disease; Chronic thromboembolic pulmonary hypertension.

Graphical Abstract

Immediate and long-term effect of balloon pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension. The anterior–posterior projection radiographs on the left show a patient example where left A8 was crossed with a wire (panel A), dilated with a 2.0-mm balloon (panel B), and reopened, with immediate filling of a second, bifurcating pulmonary artery (panel C). The bar graph on the right shows PVR at baseline and at 6 months in the absence of concomitant medication with pulmonary vasodilators in two recent randomized controlled trials (MR BPA and RACE). BPA, balloon pulmonary angioplasty; PVR, pulmonary vascular resistance; RCT, randomized controlled trial; FUP, follow-up.

Figure 1

Anatomical classification of the pulmonary vascular tree, and segment names. Anatomy is in accordance with a computed tomogram of a healthy Danish volunteer. Segments are color coded. Panel A: anterior–posterior projection. Panel B: left anterior oblique (LAO) 60° projection. Segment A7 on the left side is variable; if present, it typically occurs in a common branch with A8.

Figure 2

Vascular lesions of CTEPH. Lesions are common at bifurcations. Except for tortuous lesions that affect vessels < 2 mm in diameter and pouches that are predominantly proximal, all lesions may occur at any level of vessel size.

Figure 3

The principle of BPA. The top of the figure shows the setting of BPA, with a guiding catheter telescoped through an 80 or 90 cm sheath. The bottom of the figure shows cross sections of a vessel before BPA with the obstructing webs (left), with the balloon inflated (middle), and the final result (right). In contrast to coronary intervention, the balloon tears and compresses intraluminal webs and bands without injuring the medial layer of the vessel wall.

Figure 4

Building blocks of a BPA program.

Figure 5

Grades of injury after BPA. Time is displayed on the x-axis and clinical appearance, pathology, and imaging on the y-axis. Three hours after the start of the BPA procedure (asterisk) is arbitrary to represent the peri-procedural time frame that may vary.