The Present and Future
JACC State-of-the-Art Review
Chronic Thromboembolic Pulmonary Hypertension: Evolving Therapeutic Approaches for Operable and Inoperable Disease

https://doi.org/10.1016/j.jacc.2018.04.009Get rights and content
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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH), a rare consequence of an acute pulmonary embolism, is a disease that is underdiagnosed, and surgical pulmonary thromboendarterectomy (PTE) remains the preferred therapy. However, determination of operability is multifactorial and can be challenging. There is growing excitement for the percutaneous treatment of inoperable CTEPH with data from multiple centers around the world showing the clinical feasibility of balloon pulmonary angioplasty. Riociguat remains the only approved medical therapy for CTEPH patients deemed inoperable or with persistent pulmonary hypertension after PTE. We recommend that expert multidisciplinary CTEPH teams be developed at individual institutions. Additionally, optimal and standardized techniques for balloon pulmonary angioplasty need to be developed along with dedicated interventional equipment and appropriate training standards. In the meantime, the percutaneous revascularization option is appropriate for patients deemed inoperable in combination with targeted medical therapy, or those who have failed to benefit from surgery.

Key Words

balloon pulmonary angioplasty
BPA
chronic thromboembolic pulmonary hypertension
CTEPH
PTE
pulmonary thromboendarterectomy

Abbreviations and Acronyms

BPA
balloon pulmonary angioplasty
CBCT
cone beam computed tomography
CT
computed tomography
CTED
chronic thromboembolic disease
CTEPH
chronic thromboembolic pulmonary hypertension
LAO
left anterior oblique
mPAP
mean pulmonary artery pressure
PAH
pulmonary arterial hypertension
Pd/Pa
mean distal to proximal arterial pressure ratio
PTE
pulmonary thromboendarterectomy
PVR
pulmonary vascular resistance
RPE
reperfusion pulmonary edema
UC
University of California
V/Q
ventilation/perfusion

Cited by (0)

Dr. Kim has been a consultant for Actelion, Bayer, and Merck; and has served on the Speakers Bureau for Bayer. Dr. Poch has been a consultant; and has served on the Speakers Bureau for Bayer. Dr. Auger has served as an advisory board member (uncompensated) for Bayer’s CTEPH Image Expert Panel; and has received research funding from Bayer for the CTEPH registry. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Listen to this manuscript's audio summary by JACC Editor-in-Chief Dr. Valentin Fuster.