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Chronic Thromboembolic Pulmonary Hypertension-Management Strategies and Outcomes.

Authors
  • Ranka, Sagar1
  • Mohananey, Divyanshu2
  • Agarwal, Nikhil3
  • Verma, Beni Rai4
  • Villablanca, Pedro5
  • Mewhort, Holly E6
  • Ramakrishna, Harish7
  • 1 Department of Cardiovascular Medicine, Kansas University Medical Center, Kansas City, KS.
  • 2 Department of Cardiovascular Medicine, Medical College of Wisconsin, Milwaukee, WI.
  • 3 Division of Cardiovascular Medicine, University of Buffalo, Buffalo, NY.
  • 4 Department of Cardiology, Cleveland Clinic, Cleveland, OH.
  • 5 Interventional Cardiology, Henry Ford Health System, Detroit, MI.
  • 6 Cardiovascular Surgery, Mayo Clinic, Rochester, MN.
  • 7 Division of Cardiovascular and Thoracic Anesthesiology, Department of Anesthesia and Perioperative Medicine, Mayo Clinic, Rochester, MN. Electronic address: [email protected]
Type
Published Article
Journal
Journal of cardiothoracic and vascular anesthesia
Publication Date
Sep 01, 2020
Volume
34
Issue
9
Pages
2513–2523
Identifiers
DOI: 10.1053/j.jvca.2019.11.019
PMID: 31883688
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is rare but complex pathophysiological disease with hallmark features of chronic thrombotic mechanical obstruction, right ventricular dysfunction, and secondary pulmonary arteriopathy. It increasingly is being understood that chronic infection/inflammation, abnormal fibrinolysis, and cytokines play an important role in pathogenesis such that only a subset of patients with pulmonary embolism develop CTEPH. Diagnosis remains challenging given the lack of early clinical signs and overlap with other cardiopulmonary conditions. Pulmonary endarterectomy is the surgical procedure of choice with good postoperative survival and functional outcomes, especially when done at high-volume centers with a multidisciplinary approach. There has been a resurgence of balloon pulmonary angioplasty (BPA) as salvage therapy for inoperable CTEPH or in its newfound hybrid role for persistent postoperative pulmonary hypertension with excellent 1-year and 3-year survival. Use of riociguat has shown promising improvements in functional outcomes up to 2 years after initiation. Endothelin receptor antagonists serve a supplemental role postoperatively or in inoperable CTEPH. The role of drug therapy preoperatively or in tandem with BPA is currently under investigation. Copyright © 2019. Published by Elsevier Inc.

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