Common Phenotype in Patients With Mitral Valve Prolapse Who Experienced Sudden Cardiac Death
2018; Lippincott Williams & Wilkins; Volume: 138; Issue: 10 Linguagem: Francês
10.1161/circulationaha.118.033488
ISSN1524-4539
AutoresJérôme Hourdain, Marie‐Annick Clavel, Jean‐Claude Deharo, Samuel J. Asirvatham, Jean-François Aviérinos, Gilbert Habib, Frédéric Franceschi, Vincent Probst, Nicolas Sadoul, Raphaël P. Martins, Christophe Leclercq, Michel Chauvin, Jean Luc Pasquié, Philippe Maury, Gabriel Laurent, Michael J. Ackerman, David O. Hodge, Maurice Enriquez‐Sarano,
Tópico(s)Infective Endocarditis Diagnosis and Management
ResumoHomeCirculationVol. 138, No. 10Common Phenotype in Patients With Mitral Valve Prolapse Who Experienced Sudden Cardiac Death Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBCommon Phenotype in Patients With Mitral Valve Prolapse Who Experienced Sudden Cardiac Death Jérôme Hourdain, MD, Marie Annick Clavel, DVM, PhD, Jean-Claude Deharo, MD, Samuel Asirvatham, MD, Jean François Avierinos, MD, Gilbert Habib, MD, Frederic Franceschi, MD, PhD, Vincent Probst, MD, PhD, Nicolas Sadoul, MD, Raphael Martins, MD, PhD, Christophe Leclercq, MD, PhD, Michel Chauvin, MD, Jean Luc Pasquie, MD, PhD, Philippe Maury, MD, Gabriel Laurent, MD, PhD, Michael Ackerman, MD, PhD, David O. Hodge, MS and Maurice Enriquez-Sarano, MD Jérôme HourdainJérôme Hourdain Jérôme Hourdain, MD, Hopital de la Timone, 264 Rue Saint Pierre, Marseille 13385, France. Email E-mail Address: [email protected] Centre Hospitalier Universitaire la Timone, Marseille, France (J.H., J.-C.D., J.F.A., G.H., F.F.) , Marie Annick ClavelMarie Annick Clavel Mayo Clinic, Rochester, MN (M.A.C., S.A., M.A., D.O.H., M.E.-S.) , Jean-Claude DeharoJean-Claude Deharo Centre Hospitalier Universitaire la Timone, Marseille, France (J.H., J.-C.D., J.F.A., G.H., F.F.) , Samuel AsirvathamSamuel Asirvatham Mayo Clinic, Rochester, MN (M.A.C., S.A., M.A., D.O.H., M.E.-S.) , Jean François AvierinosJean François Avierinos Centre Hospitalier Universitaire la Timone, Marseille, France (J.H., J.-C.D., J.F.A., G.H., F.F.) , Gilbert HabibGilbert Habib Centre Hospitalier Universitaire la Timone, Marseille, France (J.H., J.-C.D., J.F.A., G.H., F.F.) , Frederic FranceschiFrederic Franceschi Centre Hospitalier Universitaire la Timone, Marseille, France (J.H., J.-C.D., J.F.A., G.H., F.F.) , Vincent ProbstVincent Probst Centre Hospitalier Universitaire Nord-Laennec, Nantes, France (V.P.) , Nicolas SadoulNicolas Sadoul Centre Hospitalier Universitaire Brabois, Vandoeuve-lès-Nancy, France (N.S.) , Raphael MartinsRaphael Martins Centre Hospitalier Universitaire Pontchaillou, Rennes, France (R.M., C.L.) , Christophe LeclercqChristophe Leclercq Centre Hospitalier Universitaire Pontchaillou, Rennes, France (R.M., C.L.) , Michel ChauvinMichel Chauvin Hôpital Civil, Strasbourg, France (M.C.) , Jean Luc PasquieJean Luc Pasquie Centre Hospitalier Universitaire Arnaud de Villeneuve, Montpellier, France (J.L.P.) , Philippe MauryPhilippe Maury Centre Hospitalier Universitaire Rangueil, Toulouse, France (P.M.) , Gabriel LaurentGabriel Laurent Centre Hospitalier Universitaire Bocage, Dijon, France (G.L.). , Michael AckermanMichael Ackerman Mayo Clinic, Rochester, MN (M.A.C., S.A., M.A., D.O.H., M.E.-S.) , David O. HodgeDavid O. Hodge Mayo Clinic, Rochester, MN (M.A.C., S.A., M.A., D.O.H., M.E.-S.) and Maurice Enriquez-SaranoMaurice Enriquez-Sarano Mayo Clinic, Rochester, MN (M.A.C., S.A., M.A., D.O.H., M.E.-S.) Originally published4 Sep 2018https://doi.org/10.1161/CIRCULATIONAHA.118.033488Circulation. 2018;138:1067–1069Mitral valve prolapse (MVP) is a prevalent valvular condition with heterogeneous outcomes.1,2 Excess mortality is associated with moderate-to-severe mitral regurgitation (MR) and reduced left ventricular ejection fraction.2 Whereas MVP is considered benign in patients without such risk factors,1,2 some reports have described ventricular arrhythmias and sudden death (SD) in apparently uncomplicated MVP.3,4 Moreover, MVP was identified in 42% of patients who experienced idiopathic out-of-hospital cardiac arrest.3The aim of this study was to collect exhaustive clinical and laboratory characteristics of patients with MVP who survived SD without any other obvious explanation.This international study involved 9 tertiary centers, 8 in France and 1 in the United States. From the database of adults implanted with a cardioverter-defibrilIator between 1996 and 2014 at each center, we included all the patients after surviving documented ventricular fibrillation with no detectable structural or electric cause other than MVP. We analyzed clinical, ECG, and echocardiographic characteristics collected before SD when available, 3 months after SD (63.6±4.0% versus 60.9±6.4%; P=0.98). Similarly, there was no change in the prevalence of PVCs of any origin (P=0.26) and of PVCs from posterior papillary muscle origin (P=0.14).Over the mean follow-up of 110.7±105.6 months, 15 patients received ≥1 shock, with a median of 3 (range, 1−16) appropriate shocks per patient. Nine patients received inappropriate shocks; 1 had an atrial fibrillation history. The patients who received appropriate shocks showed no differences over the period from SD to last follow-up in terms of left ventricle ejection fraction (52±15% versus 58±7%, P=0.68), left ventricle end-diastolic diameter (61±5 mm versus 59±4 mm, P=0.89), left ventricle end-systolic diameter (42±4 mm versus 40±2 mm, P=0.59), and MR grade (2 [2–2] versus 2 [2–4], P=0.09).We report the largest international case series of resuscitated patients with SD in whom MVP was the only detectable cause. Sudden death cannot be explained by a natural history of severe MR because it occurs in patients with a normal left ventricular ejection fraction and, for most, no, mild, or mild-to-moderate MR.2 We went beyond the often mentioned and separately described thickened bileaflet prolapse,3,4 inadequate to identify patients at risk of SD because of its high prevalence in MVP. We reported a "severe myxomatous mitral prolapse disease" characterized by the above-mentioned features combined with mitral annular disjunction, an abnormality recently associated with left ventricular late enhancement in the papillary muscles and inferobasal wall on cardiovascular magnetic resonance imaging.4Our patients demonstrated frequent and polymorphic ventricular arrhythmias originating primarily from the posterior papillary muscle with typical prolonged duration,5 known to represent ventricular arrhythmia triggers. Extrasystole prematurity, involved in idiopathic ventricular fibrillation, was unimpressive. In contrast to a previous publication,3 right/left outflow-tract PVCs, known to be mostly benign, were not predominant in our patients. Long-term follow-up demonstrated life-threatening arrhythmia recurrences, showing that cardiac arrest is not an isolated event. The stability over time of the entire phenotype confirmed that these findings are not consequent to SD.Our conclusions are drawn from a small sample, but, to our knowledge, this is the largest reported cohort of patients who have MVP with SD and are still alive. At the inclusion, no data were published on the relationship between arrhythmic MVP and myocardial scar on cardiovascular magnetic resonance, explaining that it is absent or inhomogeneous in many of our cases, precluding discussion of this important consideration.Our findings could guide further research to assess a multiparametric approach of SD risk stratification in patients with MVP, allowing identification of higher-risk patients and reassurance for those with a low-risk MVP phenotype.DisclosuresNone.Footnotes*Drs Hourdain and Clavel contributed equally and are joint first authors.https://www.ahajournals.org/journal/circData sharing: The data that support the findings of this study are available from the corresponding author on reasonable request.Jérôme Hourdain, MD, Hopital de la Timone, 264 Rue Saint Pierre, Marseille 13385, France. Email hourdain.[email protected]frReferences1. Freed LA, Levy D, Levine RA, Larson MG, Evans JC, Fuller DL, Lehman B, Benjamin EJ. Prevalence and clinical outcome of mitral-valve prolapse.N Engl J Med. 1999; 341:1–7. doi: 10.1056/NEJM199907013410101CrossrefMedlineGoogle Scholar2. Avierinos JF, Gersh BJ, Melton LJ, Bailey KR, Shub C, Nishimura RA, Tajik AJ, Enriquez-Sarano M. Natural history of asymptomatic mitral valve prolapse in the community.Circulation. 2002; 106:1355–1361.LinkGoogle Scholar3. Sriram CS, Syed FF, Ferguson ME, Johnson JN, Enriquez-Sarano M, Cetta F, Cannon BC, Asirvatham SJ, Ackerman MJ. Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest.J Am Coll Cardiol. 2013; 62:222–230. doi: 10.1016/j.jacc.2013.02.060CrossrefMedlineGoogle Scholar4. 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. 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