Gabriella Captur1, Charlotte H Manisty2, Betty Raman3, Alberto Marchi4, Timothy C Wong5, Rina Ariga3, Anish Bhuva2, Elizabeth Ormondroyd6, Ilaria Lobascio7, Claudia Camaioni7, Savvas Loizos7, Jenade Bonsu-Ofori8, Aslan Turer9, Vlad G Zaha9, João B Augutsto2, Rhodri H Davies2, Andrew J Taylor10, Arthur Nasis11, Mouaz H Al-Mallah12, Sinitsyn Valentin13, Diego Perez de Arenaza14, Vimal Patel15, Mark Westwood7, Steffen E Petersen16, Chunming Li17, Lijun Tang18, Shiro Nakamori19, Reza Nezafat19, Raymond Y Kwong20, Carolyn Y Ho20, Alan G Fraser21, Hugh Watkins6, Perry M Elliott2, Stefan Neubauer3, Guy Lloyd2, Iacopo Olivotto4, Petros Nihoyannopoulos22, James C Moon23. 1. UCL MRC Unit for Lifelong Health and Ageing, University College London, London, United Kingdom; The Royal Free Hospital, Centre for Inherited Heart Muscle Conditions, Cardiology Department, London, United Kingdom; UCL Institute of Cardiovascular Science, University College London, London, United Kingdom. 2. UCL Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Center, St. Bartholomew's Hospital, West Smithfield, London, United Kingdom. 3. University of Oxford Centre for Clinical Magnetic Resonance Research, Division of Cardiovascular Medicine, Radcliffe Department of Medicine, Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, Oxford, United Kingdom. 4. Cardiomyopathy Unit and Genetic Unit, Careggi University Hospital, Florence, Italy. 5. Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA; UPMC Cardiovascular Magnetic Resonance Center, Heart and Vascular Institute, Pittsburgh, Pennsylvania, USA. 6. Division of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, Oxford, United Kingdom. 7. Barts Heart Center, St. Bartholomew's Hospital, West Smithfield, London, United Kingdom. 8. UCL Medical School, Bloomsbury Campus, University College London, London, United Kingdom. 9. Cardiology Division, Department of Internal Medicine, and the University of Texas Southwestern Medical Center, Dallas, Texas, USA. 10. Department of Cardiovascular Medicine, Alfred Hospital, Melbourne, Australia; Baker Heart and Diabetes Institute, Melbourne, Australia; Department of Medicine, Monash University, Melbourne, Australia. 11. Monash Cardiovascular Research Centre, MonashHEART, Monash University, Clayton, Australia. 12. King Abdulaziz Cardiac Center (Riyadh), National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia. 13. Lomonosov Moscow State University, Department of Multidisciplinary Clinical Studies, Federal Center of Treatment and Rehabilitation, Moscow, Russia. 14. Cardiology Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina. 15. UCL Institute of Cardiovascular Science, University College London, London, United Kingdom. 16. Barts Heart Center, St. Bartholomew's Hospital, West Smithfield, London, United Kingdom; William Harvey Research Institute, NIHR Barts Biomedical Research Centre, Queen Mary University of London, London, United Kingdom. 17. Department of Radiology, University of Pennsylvania, Philadelphia, Pennsylvania, USA; School of Electronic Engineering, University of Electronic Science and Technology of China, Chengdu, China. 18. The First Affiliated Hospital of Nanjing Medical University, Nanjing, China. 19. Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA. 20. Cardiovascular Division, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA. 21. School of Medicine, Cardiff University, Cardiff, United Kingdom. 22. Imperial College London, National Heart & Lung Institute, The Hammersmith Hospital, London, United Kingdom. 23. UCL Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Center, St. Bartholomew's Hospital, West Smithfield, London, United Kingdom. Electronic address: j.moon@ucl.ac.uk.
Abstract
OBJECTIVES: The aim of this study was to define the variability of maximal wall thickness (MWT) measurements across modalities and predict its impact on care in patients with hypertrophic cardiomyopathy (HCM). BACKGROUND: Left ventricular MWT measured by echocardiography or cardiovascular magnetic resonance (CMR) contributes to the diagnosis of HCM, stratifies risk, and guides key decisions, including whether to place an implantable cardioverter-defibrillator (ICD). METHODS: A 20-center global network provided paired echocardiographic and CMR data sets from patients with HCM, from which 17 paired data sets of the highest quality were selected. These were presented as 7 randomly ordered pairs (at 6 cardiac conferences) to experienced readers who report HCM imaging in their daily practice, and their MWT caliper measurements were captured. The impact of measurement variability on ICD insertion decisions was estimated in 769 separately recruited multicenter patients with HCM using the European Society of Cardiology algorithm for 5-year risk for sudden cardiac death. RESULTS: MWT analysis was completed by 70 readers (from 6 continents; 91% with >5 years' experience). Seventy-nine percent and 68% scored echocardiographic and CMR image quality as excellent. For both modalities (echocardiographic and then CMR results), intramodality inter-reader MWT percentage variability was large (range -59% to 117% [SD ±20%] and -61% to 52% [SD ±11%], respectively). Agreement between modalities was low (SE of measurement 4.8 mm; 95% CI 4.3 mm-5.2 mm; r = 0.56 [modest correlation]). In the multicenter HCM cohort, this estimated echocardiographic MWT percentage variability (±20%) applied to the European Society of Cardiology algorithm reclassified risk in 19.5% of patients, which would have led to inappropriate ICD decision making in 1 in 7 patients with HCM (8.7% would have had ICD placement recommended despite potential low risk, and 6.8% would not have had ICD placement recommended despite intermediate or high risk). CONCLUSIONS: Using the best available images and experienced readers, MWT as a biomarker in HCM has a high degree of inter-reader variability and should be applied with caution as part of decision making for ICD insertion. Better standardization efforts in HCM recommendations by current governing societies are needed to improve clinical decision making in patients with HCM.
OBJECTIVES: The aim of this study was to define the variability of maximal wall thickness (MWT) measurements across modalities and predict its impact on care in patients with hypertrophic cardiomyopathy (HCM). BACKGROUND: Left ventricular MWT measured by echocardiography or cardiovascular magnetic resonance (CMR) contributes to the diagnosis of HCM, stratifies risk, and guides key decisions, including whether to place an implantable cardioverter-defibrillator (ICD). METHODS: A 20-center global network provided paired echocardiographic and CMR data sets from patients with HCM, from which 17 paired data sets of the highest quality were selected. These were presented as 7 randomly ordered pairs (at 6 cardiac conferences) to experienced readers who report HCM imaging in their daily practice, and their MWT caliper measurements were captured. The impact of measurement variability on ICD insertion decisions was estimated in 769 separately recruited multicenter patients with HCM using the European Society of Cardiology algorithm for 5-year risk for sudden cardiac death. RESULTS: MWT analysis was completed by 70 readers (from 6 continents; 91% with >5 years' experience). Seventy-nine percent and 68% scored echocardiographic and CMR image quality as excellent. For both modalities (echocardiographic and then CMR results), intramodality inter-reader MWT percentage variability was large (range -59% to 117% [SD ±20%] and -61% to 52% [SD ±11%], respectively). Agreement between modalities was low (SE of measurement 4.8 mm; 95% CI 4.3 mm-5.2 mm; r = 0.56 [modest correlation]). In the multicenter HCM cohort, this estimated echocardiographic MWT percentage variability (±20%) applied to the European Society of Cardiology algorithm reclassified risk in 19.5% of patients, which would have led to inappropriate ICD decision making in 1 in 7 patients with HCM (8.7% would have had ICD placement recommended despite potential low risk, and 6.8% would not have had ICD placement recommended despite intermediate or high risk). CONCLUSIONS: Using the best available images and experienced readers, MWT as a biomarker in HCM has a high degree of inter-reader variability and should be applied with caution as part of decision making for ICD insertion. Better standardization efforts in HCM recommendations by current governing societies are needed to improve clinical decision making in patients with HCM.
Authors: Yvonne Bewarder; Lucas Lauder; Saarraaken Kulenthiran; Ortwin Schäfer; Christian Ukena; Robert Percy Marshall; Pierre Hepp; Ulrich Laufs; Stephan Stöbe; Andreas Hagendorff; Michael Böhm; Felix Mahfoud; Sebastian Ewen Journal: Int J Cardiol Heart Vasc Date: 2022-05-06