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[Chronic thromboembolic pulmonary hypertension: Recommendations of the Cologne Consensus Conference 2016]

H Wilkens, S Konstantinides, I Lang

    Deutsche Medizinische Wochenschrift (1946)
    |October 21, 2016
    PubMed

    Insights

    Chronic thromboembolic pulmonary hypertension (CTEPH) requires exclusion in all unexplained pulmonary hypertension cases. Surgical pulmonary endarterectomy (PEA) is the primary treatment, with drug therapy or balloon pulmonary angioplasty (BPA) for inoperable patients.

    Area of Science:

    • Cardiology
    • Pulmonology
    • Vascular Surgery

    Background:

    • The 2015 European Guidelines on Pulmonary Hypertension (PH) addressed multiple PH subgroups beyond pulmonary arterial hypertension (PAH).
    • A 2016 Consensus Conference convened to address practical implementation challenges of these guidelines.
    • A dedicated working group focused on chronic thromboembolic pulmonary hypertension (CTEPH).

    Purpose of the Study:

    • To summarize recommendations for the diagnosis and treatment of CTEPH.
    • To emphasize the importance of excluding CTEPH in patients with unexplained PH.
    • To outline treatment strategies including pulmonary endarterectomy (PEA), drug therapy, and balloon pulmonary angioplasty (BPA).

    Main Methods:

    • Review of current literature and expert consensus on CTEPH diagnosis and management.
    • Discussion of surgical (PEA) and non-surgical treatment options.
    • Consideration of emerging therapies like BPA and rare conditions like pulmonary artery sarcoma.

    Main Results:

    • CTEPH must be excluded in all patients with unexplained PH.
    • Surgical pulmonary endarterectomy (PEA) is the primary treatment, best performed in specialized centers.
    • Inoperable patients or those with persistent/recurrent CTEPH are candidates for targeted drug therapy or BPA, with limited experience for BPA.

    Conclusions:

    • The recommendations provide a framework for managing CTEPH based on current evidence and expert opinion.
    • Specialized multidisciplinary centers are crucial for optimal CTEPH treatment, including PEA and BPA.
    • Pulmonary artery sarcoma management may parallel PEA approaches.

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