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State of the art review
Cardiac MRI in the assessment of chronic thromboembolic pulmonary hypertension and response to treatment
  1. Aleksandra Bartnik1,2,3,
  2. Joanna Pepke-Zaba4,
  3. Katherine Bunclark4,
  4. Alessandro Ruggiero2,
  5. D Jenkins1,
  6. J Taghavi1,
  7. Steven Tsui1,
  8. Nicholas Screaton2,
  9. L D’Errico2,
  10. Jonathan Weir-McCall2,3
  1. 1 Cardiothoracic Surgery, Royal Papworth Hospital, Cambridge, UK
  2. 2 Radiology, Papworth Hospital NHS Foundation Trust, Cambridge, UK
  3. 3 Radiology, University of Cambridge, Cambridge, UK
  4. 4 PVDU, Papworth Hospital NHS Foundation Trust, Cambridge, UK
  1. Correspondence to Aleksandra Bartnik, Cardiothoracic surgery, Royal Papworth Hospital, Cambridge CB2 9DS, UK; o.bartnik{at}ucl.ac.uk; Dr Jonathan Weir-McCall; jweirmccall{at}gmail.com

https://doi.org/10.1136/thorax-2022-219716

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    Chronic thromboembolic pulmonary disease (CTEPD) arises in patients with prior pulmonary embolism who do not clear the obstructive material from the pulmonary circulation and may experience persistently raised pulmonary pressures (chronic thromboembolic pulmonary hypertension, CTEPH) and right heart failure. CTEPH affects between 1% and 9% of patients following a single episode of pulmonary embolism.1 The diagnosis of CTEPD requires characteristic radiological appearances, at least 3 months of anticoagulation therapy, and the diagnosis of concomitant pulmonary hypertension requires mean pulmonary artery pressure (mPAP)>20 mm Hg and pulmonary vascular resistance (PVR)>2 Wood units with a pulmonary capillary wedge pressure≤15 mm Hg.2 3 The literature included in this review is based on the previous threshold of mPAP>25 mm Hg for the diagnosis of CTEPH4

    The role of cardiovascular MR in CTEPH

    Surgical pulmonary endarterectomy (PEA) has offered a breakthrough by improving the 3-year survival to 89% vs 70% with medical management (figure 1).5 Nonetheless, PEA is still associated with 21% 5-year mortality, 51% residual pulmonary hypertension (mPAP>25 mm Hg), poor functional response in 15% and 20% of patients requiring pulmonary vasodilators within 2 years of surgery.6 Therefore, there is a clear need for improved phenotyping of patients considered for PEA to identify those requiring aggressive follow-up and medical therapy. Balloon pulmonary angioplasty (BPA) has been used successfully in patients who are not surgical candidates with clinical and haemodynamic improvement.7–11 Around 7% of patients undergoing PEA have CTEPD without pulmonary hypertension and experience significant improvement in exercise tolerance. However, surgical selection in this group is challenging. The decision-making can be aided by exercise right heart catheterisation (RHC) demonstrating a raise in pulmonary pressures disproportionate to cardiac output, and cardiopulmonary exercise testing showing reduced ventilatory efficiency.12 However, strong evidence base for indications for PEA in these patients is lacking. Detailed assessment of the right ventricle (RV) could help …

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    Footnotes

    • Twitter @jweirmccall

    • Contributors All authors had substantial contribution to the manuscript according to ICMJE guidelines. AB performed the overall review of the literature, wrote the main body of the article and produced the central figure, JP-Z performed the overall review of the literature, advised on the accuracy and clinical relevance of the information, KB performed the review of literature on the left ventricular assessment and wrote the related section of the manuscript, AR provided expert opinion on the lung perfusion and provided relevant figure for the manuscript, DJ, ST and JT ensured accuracy of the information related to the surgical management and provided expert opinion on the relevance to surgical management, NS and LD’E ensured accuracy of the information related to different CMR techniques discussed in the literature, JW-M performed overall review of the literature and provided oversight for the writing of all aspects of the manuscript.

    • Funding This work was supported by JP Moulton Charity Trust grant number 01/19. JW-M is supported by the NIHR Cambridge Biomedical Research Centre grant number BRC-1215-20014.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.