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Cardiopulmonary exercise test and sudden cardiac death risk in hypertrophic cardiomyopathy
  1. Damiano Magrì1,
  2. Giuseppe Limongelli2,
  3. Federica Re3,
  4. Piergiuseppe Agostoni4,5,
  5. Elisabetta Zachara3,
  6. Michele Correale6,
  7. Vittoria Mastromarino1,
  8. Caterina Santolamazza1,
  9. Matteo Casenghi1,
  10. Giuseppe Pacileo2,
  11. Fabio Valente2,
  12. Beatrice Musumeci1,
  13. Antonello Maruotti7,8,
  14. Massimo Volpe1,9,
  15. Camillo Autore1
  1. 1Department of Clinical and Molecular Medicine, University of Rome “La Sapienza”, Rome, Italy
  2. 2Cardiologia SUN, Monaldi Hospital, II University of Naples, Naples, Italy
  3. 3Cardiology Division, Cardiac Arrhythmia Center and Cardiomyopathies Unit, San Camillo-Forlanini Hospital, Rome, Italy
  4. 4Centro Cardiologico Monzino, IRCCS, Milan, Italy
  5. 5Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
  6. 6Department of Cardiology, University of Foggia, Foggia, Italy
  7. 7Department of Scienze Economiche, politiche e delle lingue moderne, Libera Università SS Maria Assunta, Rome, Italy
  8. 8Centre for Innovation and Leadership in Health Sciences, University of Southampton, Southampton, UK
  9. 9IRCCS, Neuromed, Pozzilli (IS), Italy
  1. Correspondence to Professor Damiano Magrì, Department of Clinical and Molecular Medicine, “Sapienza” University, Rome, Italy; Cardiology Unit, Sant'Andrea Hospital, Via di Grottarossa 1037, Rome 00189, Italy; damiano.magri{at}


Background In hypertrophic cardiomyopathy (HCM), most of the factors associated with the risk of sudden cardiac death (SCD) are also involved in the pathophysiology of exercise limitation. The present multicentre study investigated possible ability of cardiopulmonary exercise test in improving contemporary strategies for SCD risk stratification.

Methods A total of 623 consecutive outpatients with HCM, from five tertiary Italian HCM centres, were recruited and prospectively followed, between September 2007 and April 2015. The study composite end point was SCD, aborted SCD and appropriate implantable cardioverter defibrillator (ICD) interventions.

Results During a median follow-up of 3.7 years (25th–75th centile: 2.2–5.1 years), 25 patients reached the end point at 5 years (3 SCD, 4 aborted SCD, 18 appropriate ICD interventions). At multivariate analysis, ventilation versus carbon dioxide relation during exercise (VE/VCO2 slope) remains independently associated to the study end point either when challenged with the 2011 American College of Cardiology Foundation/American Heart Association guidelines-derived score (C index 0.748) or with the 2014 European Society of Cardiology guidelines-derived score (C index 0.750). A VE/VCO2 slope cut-off value of 31 showed the best accuracy in predicting the SCD end point within the entire HCM study cohort (sensitivity 64%, specificity 72%, area under the curve 0.72).

Conclusions Our data suggest that the VE/VCO2 slope might improve SCD risk stratification, particularly in those HCM categories classified at low-intermediate SCD risk according to contemporary guidelines. There is a need for further larger studies, possibly on independent cohorts, to confirm our preliminary findings.

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