Abstract
Hypertrophic cardiomyopathy (HCM) with severe diastolic dysfunction is a major cause of heart failure and sudden cardiac death (SCD) associated with lethal arrhythmia. Although various risk factors for cardiac events have been reported in HCM patients, previous studies have reported that some HCM patients exhibit either no risk or a low risk of SCD experienced cardiac events. The mid-diastolic transmitral flow velocity curve (mitral L-wave) is an echocardiographic index of left ventricular compliance, and it has been reported as one of the parameters of advanced diastolic dysfunction assessed noninvasively. However, little is known about the association between the mitral L-wave and long-term clinical outcomes in HCM patients without SCD risk factors. Between July 2005 and February 2016, 112 patients were diagnosed with HCM and 96 patients without risk factors were enrolled. After excluding 3 patients whom we could not detect L-wave more than once, 93 patients (mean age 57.7 ± 13.1 years, 33 females) were divided into the following two groups, according to the presence or absence of the mitral L-wave: Group L (+) (with the mitral L-wave) and Group L (−) (without the mitral L-wave). The correlations between the mitral L-wave and rates of cardiac events were investigated. The mitral L-wave was present in 14 (15.1%) patients [Group L]. During the follow-up period [4.7 (2.9–7.5) years], patients experienced 7 cardiac events. Kaplan–Meier survival analysis showed that the event-free rate was significantly lower in Group L (+) than in Group L (−) (log-rank P = 0.002). Additionally, in multivariate analysis, L-wave positivity was identified as independent predictors of cardiac events. Existence of the mitral L-wave can predict cardiac events, even in HCM patients without SCD risk factors.
Similar content being viewed by others
References
Maron BJ, Maron MS (2013) Hypertrophic cardiomyopathy. Lancet 381:242–255
Maron BJ, Gardin JM, Flack JM, Gidding SS, Kurosaki TT, Bild DE (1995) Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults. Circulation 92:785–789
American College of Cardiology Foundation/American Heart Association Task Force on P, American Association for Thoracic S, American Society of E, American Society of Nuclear C, Heart Failure Society of A, Heart Rhythm S, Society for Cardiovascular A, Interventions, Society of Thoracic S, Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (2011) 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Thorac Cardiovasc Surg 142:e153–203
Grossman W (1991) Diastolic dysfunction in congestive heart failure. N Engl J Med 325:1557–1564
Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH, Spirito P, Ten Cate FJ, Wigle ED, Vogel RA, Abrams J, Bates ER, Brodie BR, Danias PG, Gregoratos G, Hlatky MA, Hochman JS, Kaul S, Lichtenberg RC, Lindner JR, O’rourke RA, Pohost GM, Schofield RS, Tracy CM, Winters WL, Klein WW, Priori SG, Alonso-Garcia A, Blomström-Lundqvist C, De Backer G, Deckers J, Flather M, Hradec J, Oto A, Parkhomenko A, Silber S, Torbicki A (2003) American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy. J Am Coll Cardiol 42:1687–1713
Maron BJ (2002) Hypertrophic cardiomyopathy: a systematic review. JAMA 287:1308–1320
Maron BJ (2010) Contemporary insights and strategies for risk stratification and prevention of sudden death in hypertrophic cardiomyopathy. Circulation 121:445–456
Spirito P, Seidman CE, McKenna WJ, Maron BJ (1997) The management of hypertrophic cardiomyopathy. N Engl J Med 336:775–785
Elliott PM, Poloniecki J, Dickie S, Sharma S, Monserrat L, Varnava A, Mahon NG, McKenna WJ (2000) Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol 36:2212–2218
JCS Joint Working Group (2016) Guidelines for diagnosis and treatment of patients with hypertrophic cardiomyopathy (JCS 2012)—digest version. Circ J 80:753–774
Authors/ Task Force m, Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, Hagege AA, Lafont A, Limongelli G, Mahrholdt H, McKenna WJ, Mogensen J, Nihoyannopoulos P, Nistri S, Pieper PG, Pieske B, Rapezzi C, Rutten FH, Tillmanns C, Watkins H (2014) 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 35:2733–2779
Christiaans I, van Engelen K, van Langen IM, Birnie E, Bonsel GJ, Elliott PM, Wilde AA (2010) Risk stratification for sudden cardiac death in hypertrophic cardiomyopathy: systematic review of clinical risk markers. Europace 12:313–321
O'Mahony C, Tome-Esteban M, Lambiase PD, Pantazis A, Dickie S, McKenna WJ, Elliott PM (2013) A validation study of the 2003 American College of Cardiology/European Society of Cardiology and 2011 American College of Cardiology Foundation/American Heart Association risk stratification and treatment algorithms for sudden cardiac death in patients with hypertrophic cardiomyopathy. Heart 99:534–541
Keren G, Meisner JS, Sherez J, Yellin EL, Laniado S (1986) Interrelationship of mid-diastolic mitral valve motion, pulmonary venous flow, and transmitral flow. Circulation 74:36–44
Ha JW, Oh JK, Redfield MM, Ujino K, Seward JB, Tajik AJ (2004) Triphasic mitral inflow velocity with middiastolic filling: clinical implications and associated echocardiographic findings. J Am Soc Echocardiogr 17:428–431
Lam CS, Han L, Ha JW, Oh JK, Ling LH (2005) The mitral L wave: a marker of pseudonormal filling and predictor of heart failure in patients with left ventricular hypertrophy. J Am Soc Echocardiogr 18:336–341
Kim SA, Son J, Shim CY, Choi EY, Ha JW (2017) Long-term outcome of patients with triphasic mitral flow with a mid-diastolic L wave: prognostic role of left atrial volume and N-terminal pro-brain natriuretic peptide. Int J Cardiovasc Imaging 33:1377–1384
Masai K, Mano T, Goda A, Sugahara M, Daimon A, Asakura M, Ishihara M, Masuyama T (2018) Correlates and prognostic values of appearance of L wave in heart failure patients with preserved vs Reduced Ejection Fraction. Circ J 82:2311–2316
Nagueh SF, Bierig SM, Budoff MJ, Desai M, Dilsizian V, Eidem B, Goldstein SA, Hung J, Maron MS, Ommen SR, Woo A, Echocardiography ASo, Cardiology ASoN, Resonance SfCM, Tomography SoCC (2011) American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with hypertrophic cardiomyopathy: Endorsed by the American Society of Nuclear Cardiology, Society for Cardiovascular Magnetic Resonance, and Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr 24:473–498
Maron BJ, Rowin EJ, Casey SA, Link MS, Lesser JR, Chan RH, Garberich RF, Udelson JE, Maron MS (2015) Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies. J Am Coll Cardiol 65:1915–1928
O'Mahony C, Jichi F, Pavlou M, Monserrat L, Anastasakis A, Rapezzi C, Biagini E, Gimeno JR, Limongelli G, McKenna WJ, Omar RZ, Elliott PM, Hypertrophic Cardiomyopathy Outcomes I (2014) A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J 35:2010–2020
Spirito P, Autore C, Formisano F, Assenza GE, Biagini E, Haas TS, Bongioanni S, Semsarian C, Devoto E, Musumeci B, Lai F, Yeates L, Conte MR, Rapezzi C, Boni L, Maron BJ (2014) Risk of sudden death and outcome in patients with hypertrophic cardiomyopathy with benign presentation and without risk factors. Am J Cardiol 113:1550–1555
Adabag AS, Maron BJ, Appelbaum E, Harrigan CJ, Buros JL, Gibson CM, Lesser JR, Hanna CA, Udelson JE, Manning WJ, Maron MS (2008) Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance. J Am Coll Cardiol 51:1369–1374
Chan RH, Maron BJ, Olivotto I, Pencina MJ, Assenza GE, Haas T, Lesser JR, Gruner C, Crean AM, Rakowski H, Udelson JE, Rowin E, Lombardi M, Cecchi F, Tomberli B, Spirito P, Formisano F, Biagini E, Rapezzi C, De Cecco CN, Autore C, Cook EF, Hong SN, Gibson CM, Manning WJ, Appelbaum E, Maron MS (2014) Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation 130:484–495
Ha JW, Ahn JA, Moon JY, Suh HS, Kang SM, Rim SJ, Jang Y, Chung N, Shim WH, Cho SY (2006) Triphasic mitral inflow velocity with mid-diastolic flow: the presence of mid-diastolic mitral annular velocity indicates advanced diastolic dysfunction. Eur J Echocardiogr 7:16–21
Lam CS, Han L, Oh JK, Yang H, Ling LH (2008) The mitral annular middiastolic velocity curve: functional correlates and clinical significance in patients with left ventricular hypertrophy. J Am Soc Echocardiogr 21:165–170
Geske JB, Sorajja P, Nishimura RA, Ommen SR (2007) Evaluation of left ventricular filling pressures by Doppler echocardiography in patients with hypertrophic cardiomyopathy: correlation with direct left atrial pressure measurement at cardiac catheterization. Circulation 116:2702–2708
Frommelt PC, Pelech AN, Frommelt MA (2003) Diastolic dysfunction in an unusual case of cardiomyopathy in a child: insights from Doppler and Doppler tissue imaging analysis. J Am Soc Echocardiogr 16:176–181
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare no competing financial interest.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Sugiura, Y., Morimoto, R., Aoki, S. et al. Prognostic impact of mitral L-wave in patients with hypertrophic cardiomyopathy without risk factors for sudden cardiac death. Heart Vessels 34, 2002–2010 (2019). https://doi.org/10.1007/s00380-019-01440-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00380-019-01440-y