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Original research
Inline perfusion mapping provides insights into the disease mechanism in hypertrophic cardiomyopathy
  1. Claudia Camaioni1,
  2. Kristopher D Knott1,2,
  3. Joao B Augusto1,2,
  4. Andreas Seraphim1,2,
  5. Stefania Rosmini1,
  6. Fabrizio Ricci3,
  7. Redha Boubertakh1,4,
  8. Hui Xue5,
  9. Rebecca Hughes1,2,
  10. Gaby Captur1,2,
  11. Luis Rocha Lopes1,2,
  12. Louise Anne Elizabeth Brown6,
  13. Charlotte Manisty1,2,
  14. Steffen Erhard Petersen1,4,
  15. Sven Plein6,
  16. Peter Kellman5,
  17. Saidi A Mohiddin1,
  18. James C Moon1,2
  1. 1 Advanced Cardiac Imaging, Barts Health NHS Trust, London, UK
  2. 2 Institute of Cardiovascular Science, University College London, London, UK
  3. 3 University of Chieti-Pescara, Chieti, Italy
  4. 4 The William Harvey Research Institute, Queen Mary University of London, London, UK
  5. 5 National Institutes of Health, Bethesda, Maryland, USA
  6. 6 Department of Biomedical Imaging Science, University of Leeds, Leeds, UK
  1. Correspondence to Dr James C Moon, University College London, London WC1E 6BT, UK; j.moon{at}


Objective In patients with hypertrophic cardiomyopathy (HCM), the role of small vessel disease and myocardial perfusion remains incompletely understood and data on absolute myocardial blood flow (MBF, mL/g/min) are scarce. We measured MBF using cardiovascular magnetic resonance fully quantitative perfusion mapping to determine the relationship between perfusion, hypertrophy and late gadolinium enhancement (LGE) in HCM.

Methods 101 patients with HCM with unobstructed epicardial coronary arteries and 30 controls (with matched cardiovascular risk factors) underwent pixel-wise perfusion mapping during adenosine stress and rest. Stress, rest MBF and the myocardial perfusion reserve (MPR, ratio of stress to rest) were calculated globally and segmentally and then associated with segmental wall thickness and LGE.

Results In HCM, 79% had a perfusion defect on clinical read. Stress MBF and MPR were reduced compared with controls (mean±SD 1.63±0.60 vs 2.30±0.64 mL/g/min, p<0.0001 and 2.21±0.87 vs 2.90±0.90, p=0.0003, respectively). Globally, stress MBF fell with increasing indexed left ventricle mass (R2 for the model 0.186, p=0.036) and segmentally with increasing wall thickness and LGE (both p<0.0001). In 21% of patients with HCM, MBF was lower during stress than rest (MPR <1) in at least one myocardial segment, a phenomenon which was predominantly subendocardial. Apparently normal HCM segments (normal wall thickness, no LGE) had reduced stress MBF and MPR compared with controls (mean±SD 1.88±0.81 mL/g/min vs 2.32±0.78 mL/g/min, p<0.0001).

Conclusions Microvascular dysfunction is common in HCM and associated with hypertrophy and LGE. Perfusion can fall during vasodilator stress and is abnormal even in apparently normal myocardium suggesting it may be an early disease marker.

  • Hypertrophic cardiomyopathy
  • Advanced cardiac imaging
  • Cardiac magnetic resonance (CMR) imaging

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  • CC and KDK contributed equally.

  • Contributors CC and KDK contributed equally to this work. CC, KDK and JCM drafted the article, and contributed to the conception and design of the work and final approval of the version to be published. CC, KDK, JBA and AS contributed to data collection. CC, KDK, SR, FR, RB, HX and PK contributed to the data analysis and interpretation. HX, RH, GC, LRL, LAEB, CM, SEP, SP, PK and SAM contributed to the critical revision of the article.

  • Funding This study was supported by research grant from the European Association of Cardiovascular Imaging (to CC).

  • Competing interests SEP provides consultancy to Circle Cardiovascular Imaging (Calgary, Alberta, Canada).

  • Patient consent for publication Not required.

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

  • Data availability statement Data are available upon reasonable request.