Arrhythmic Mitral Valve Prolapse and Sudden Cardiac

Correspondence Disclosures

Letter by Providencia and Lambiase Regarding Article, “Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death”

None. Rui Providencia, MD, PhD Barts Hart Centre Barts Health NHS Trust London, United Kingdom

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To the Editor: Basso and colleagues1 elegantly describe a well-circumscribed entity combining mitral valve prolapse (MVP) with left ventricular fibrosis and ventricular arrhythmia causing 7% of all sudden cardiac deaths (SCDs) in young adults from the Veneto region. Malignant MVP affects a tiny minority of patients with MVP, and it is responsible for a very small absolute number of all SCDs in young adults. Assuming that the annual incidence of SCD in this age group ranges between 1.21 (in young athletes) and 3.76 (general population aged 12–35) per 100 000,2 malignant MVP may be causing 0.85 to 2.63 deaths per 1 000 000 person-years. Based on the prevalence of MVP in the general population, which is 2% to 3%,3 we can expect only 1 of every 7600 to 35 400 young adults with this abnormality to sustain a fatal event per year. Therefore, what are the implications of these findings when assessing a patient with MVP? Assuming that we may have to perform a cardiac MRI, treadmill exercise test, and serial Holter monitoring, should we request these tests in every single patient with this condition? The expected cost of this strategy to identify a high-risk patient and implant a prophylactic implantable cardioverter defibrillator would be $2.3 to $10.6 million per life saved if we decide to screen only patients referred to clinic after being clinically diagnosed with MVP; however, it would ascend to $277 to $281 million if we start using echocardiogram as the standard screening test and proceed to cardiac MRI, treadmill exercise test, and Holter monitoring when a MVP is diagnosed. Are we prepared to deal with this exponential increase in costs, referrals, and false positives? A better, more cost-effective alternative could entail a widespread program of cardiopulmonary resuscitation training, external automatic defibrillators operated by lay rescuers, coupled with rapid-response paramedics for all SCD events in the population,4 and specialized clinics for screening relatives of SCD victims as practiced by Tan et al.5 The cost of secondary prevention measures has been estimated to be $1.5 to $3.3 million per life saved (ie,5–10 times cheaper than the ECG-based strategy).4 The description of this rare manifestation of MVP may be a warning of the weaknesses of current screening strategies, but concentration on more effective SCD secondary prevention strategies in the first instance will be more likely to prevent such SCDs with lower resource and cost implications.

Pier D. Lambiase, PhD, FRCP, FHRS Barts Hart Centre Barts Health NHS Trust London, United Kingdom Institute of Cardiovascular Science University College of London London, United Kingdom

References 1. Basso C, Perazzolo Marra M, Rizzo S, De Lazzari M, Giorgi B, Cipriani A, Frigo AC, Rigato I, Migliore F, Pilichou K, Bertaglia E, Cacciavillani L, Bauce B, Corrado D, Thiene G, Iliceto S. Arrhythmic mitral valve prolapse and sudden cardiac death. Circulation. 2015;132:556–566. doi: 10.1161/CIRCULATIONAHA.115.016291. 2. Holst AG, Winkel BG, Theilade J, Kristensen IB, Thomsen JL, Ottesen GL, Svendsen JH, Haunsø S, Prescott E, Tfelt-Hansen J. Incidence and etiology of sports-related sudden cardiac death in Denmark–implications for preparticipation screening. Heart Rhythm. 2010;7:1365–1371. doi: 10.1016/j.hrthm.2010.05.021. 3. Delling FN, Vasan RS. Epidemiology and pathophysiology of mitral valve prolapse: new insights into disease progression, genetics, and molecular basis. Circulation. 2014;129:2158–2170. doi: 10.1161/ CIRCULATIONAHA.113.006702. 4. Hazinski MF, Markenson D, Neish S, Gerardi M, Hootman J, Nichol G, Taras H, Hickey R, OConnor R, Potts J, van der Jagt E, Berger S, Schexnayder S, Garson A Jr, Doherty A, Smith S; American Heart Association; American Academy of Pediatrics; American College of Emergency Physicians; American National Red Cross; National Association of School Nurses; National Association of State EMS Directors; National Association of EMS Physicians; National Association of Emergency Medical Technicians; Program for School Preparedness and Planning; National Center for Disaster Preparedness; Columbia University Mailman School of Public Health. Response to cardiac arrest and selected life-threatening medical emergencies: the medical emergency response plan for schools: a statement for healthcare providers, policymakers, school administrators, and community leaders. Circulation. 2004;109:278–291. doi: 10.1161/01.CIR.0000109486.45545.AD. 5. Tan HL, Hofman N, van Langen IM, van der Wal AC, Wilde AA. Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives. Circulation. 2005;112:207– 213. doi: 10.1161/CIRCULATIONAHA.104.522581.

Sources of Funding This work was funded by the British Heart Foundation and University College of London Hospitals Biomedical Research Centre, funded by the National Institute for Health Research.

(Circulation. 2016;133:e459. DOI: 10.1161/CIRCULATIONAHA.115.018760.) © 2016 American Heart Association, Inc. Circulation is available at http://circ.ahajournals.org

DOI: 10.1161/CIRCULATIONAHA.115.018760

e459

Letter by Providencia and Lambiase Regarding Article, ''Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death'' Rui Providencia and Pier D. Lambiase

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Circulation. 2016;133:e459 doi: 10.1161/CIRCULATIONAHA.115.018760 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 2016 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539

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