Chronic Thromboembolic Pulmonary Hypertension-Management Strategies and Outcomes

Sagar Ranka, Divyanshu Mohananey, Nikhil Agarwal, Beni Rai Verma, Pedro Villablanca, Holly E. Mewhort, Harish Ramakrishna

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)2513-2523
Number of pages11
JournalJournal of Cardiothoracic and Vascular Anesthesia
Issue number9
StatePublished - Sep 2020


  • balloon pulmonary angioplasty
  • chronic thromboembolic pulmonary hypertension
  • review
  • riociguat

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Anesthesiology and Pain Medicine


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