Title:Chronic Thromboembolic Pulmonary Hypertension
Volume: 20
Issue: 3
Author(s): Emory Buck, Sean Kearns, Talal Dahhan, Kishan S. Parikh, Richard A. Krasuski and Sudarshan Rajagopal*
Affiliation:
- Division of Cardiology, Department of Medicine, Duke University School of Medicine, Durham, NC
27710, USA
Keywords:
Chronic thromboembolic pulmonary hypertension, chronic thromboembolic pulmonary disease, balloon pulmonary angioplasty, pulmonary endarterectomy, RHC, PA.
Abstract: While the majority of patients have complete resolution of their acute pulmonary embolism
(PE) after an adequate course of anticoagulation, some patients remain symptomatic with
evidence of chronic PE. Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Chronic
Thromboembolic Pulmonary Disease (CTEPD) are terms that describe symptomatic patients
with chronic thromboembolic occlusions of the pulmonary arteries with or without pulmonary hypertension,
respectively. Here, we review the definitions, epidemiology, pathobiology, diagnosis
and management of CTEPH. The chronic PE in CTEPH is essentially a scar in the pulmonary vasculature
and is accompanied by a pulmonary arteriolar vasculopathy. Ventilation-perfusion scanning
is the most sensitive screening test for CTEPH, and diagnosis must be confirmed by right
heart catheterization (RHC). Treatment decisions require a multidisciplinary team and guidance
from additional imaging, usually CT or pulmonary angiography. While pulmonary endarterectomy
(PEA) to remove the chronic PE surgically is still the first-line treatment for appropriate candidates,
there is an expanding role for balloon pulmonary angioplasty (BPA) and medical treatment,
as well as multimodality treatment approaches that incorporate all of those options. New imaging
modalities and treatment strategies hold the promise to improve our care and management of
CTEPH patients in the future.
