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      Revisiting a Distinct Entity in Pulmonary Vascular Disease: Chronic Thromboembolic Pulmonary Hypertension (CTEPH).

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          Abstract

          Chronic thromboembolic pulmonary hypertension (CTEPH) is a specific type of pulmonary hypertension (PH) and the major component of Group 4 pulmonary hypertension (PH). It is caused by pulmonary vasculature obstruction that leads to a progressive increase in pulmonary vascular resistance and, ultimately, to failure of the right ventricle. Pulmonary thromboendarterectomy (PEA) is the only definitive therapy, so a timely diagnosis and early referral to a specialized PEA center to determine candidacy is prudent for a favorable outcome. Percutaneous balloon pulmonary angioplasty (BPA) has a potential role in patients unsuitable for PEA. Medical therapy with riociguat is the only PH-specific medical therapy currently approved for the treatment of inoperable or persistent CTEPH. This review article aims to revisit CTEPH succinctly with a review of prevailing literature.

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          Haemodynamic definitions and updated clinical classification of pulmonary hypertension

          Gerald Simonneau, David Montani, David Celermajer … (2019)
          Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3.3 mmHg. Two standard deviations above this mean value would suggest mPAP >20 mmHg as above the upper limit of normal (above the 97.5th percentile). This definition is no longer arbitrary, but based on a scientific approach. However, this abnormal elevation of mPAP is not sufficient to define pulmonary vascular disease as it can be due to an increase in cardiac output or pulmonary arterial wedge pressure. Thus, this 6th WSPH Task Force proposes to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg. Prospective trials are required to determine whether this PH population might benefit from specific management. Regarding clinical classification, the main Task Force changes were the inclusion in group 1 of a subgroup “pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers”, due to the specific prognostic and management of these patients, and a subgroup “PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement”, due to evidence suggesting a continuum between arterial, capillary and vein involvement in PAH.
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            Chronic thromboembolic pulmonary hypertension

            Nick H Kim, Marion Delcroix, Xavier Jaïs … (2019)
            Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.
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              Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.

              Marion Delcroix, Irene Lang, Joanna Pepke-Zaba … (2016)
              Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension.
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                Author and article information

                Journal
                Journal ID (iso-abbrev): Medicina (Kaunas)
                Title: Medicina (Kaunas, Lithuania)
                Publisher: MDPI AG
                ISSN (Electronic): 1648-9144
                ISSN (Print): 1010-660X
                Publication date (Electronic): Apr 07 2021
                Volume: 57
                Issue: 4
                Affiliations
                [1 ] Corpus Christi Medical Center, Department of Pulmonary Medicine, Corpus Christi, TX 78412, USA.
                [2 ] Department of Pulmonary Medicine, University of Texas Health Science Center, San Antonio, TX 78229, USA.
                Article
                Publisher Item ID: medicina57040355
                DOI: 10.3390/medicina57040355
                PMC ID: 8067524
                PubMed ID: 33916978
                SO-VID: c9e41c98-cce5-4808-a28e-e7d297828a4d
                History

                Keywords: computed tomography pulmonary angiography,chronic thromboembolic pulmonary hypertension,ventilation-perfusion lung scan,riociguat,pulmonary thromboendarterectomy,pulmonary embolism,pulmonary angiography,percutaneous balloon pulmonary angioplasty
                Data availability:
                Keywords: computed tomography pulmonary angiography, chronic thromboembolic pulmonary hypertension, ventilation-perfusion lung scan, riociguat, pulmonary thromboendarterectomy, pulmonary embolism, pulmonary angiography, percutaneous balloon pulmonary angioplasty

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