Abstract
Exercise function is well characterized in adults with hypertrophic cardiomyopathy (HCM); however, there is a paucity of data in children and young adults with HCM. Here we sought to characterize exercise function in young people with HCM, understand limitations in exercise function by correlating exercise function parameters with echocardiogram parameters and identify prognostic value of exercise parameters. We performed a retrospective, single-center cohort study characterizing exercise function in patients < 26 years old with HCM undergoing cardiopulmonary exercise testing (CPET). Patients with syndromic HCM or submaximal effort were excluded. We compared exercise function in this cohort to population normal values and measured changes in exercise function over time. We correlated exercise function parameters with echocardiographic parameters and investigated the relationship between exercise test parameters and a clinical composite outcome comprised of significant ventricular arrhythmia, death, or heart transplantation. We identified 229 CPETs performed by 117 patients (mean age at time of first CPET 15.6 ± 3.2 years). Mean %-predicted peak VO2, O2 pulse, and peak heart rate were statistically significantly depressed compared to population normal values and exercise function gradually worsened over time. Abnormal exercise testing correlated closely with echocardiographic indices of diastolic dysfunction. There was a trend toward increased incidence of poor clinical outcome in patients with abnormal exercise function. While adverse clinical outcomes were rare, normal exercise function appears to be a marker of low risk for adverse clinical outcomes in this population.
Similar content being viewed by others
References
Ommen SR, Mital S, Burke MA et al (2021) 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American college of cardiology/American heart association joint committee on clinical practice guidelines. J Thorac Cardiovasc Surg S0022–5223(21):00591–00592. https://doi.org/10.1016/j.jtcvs.2021.04.001
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(4):785–789. https://doi.org/10.1161/01.cir.92.4.785
Maron BJ, Mathenge R, Casey SA, Poliac LC, Longe TF (1999) Clinical profile of hypertrophic cardiomyopathy identified de novo in rural communities. J Am Coll Cardiol 33(6):1590–1595. https://doi.org/10.1016/s0735-1097(99)00039-x
Zou Y, Song L, Wang Z et al (2004) Prevalence of idiopathic hypertrophic cardiomyopathy in China: a population-based echocardiographic analysis of 8080 adults. Am J Med 116(1):14–18. https://doi.org/10.1016/j.amjmed.2003.05.009
Morita H, Larson MG, Barr SC, Vasan RS, O’Donnell CJ, Hirschhorn JN, Levy D, Corey D, Seidman CE, Seidman JG, Benjamin EJ (2006) Single-gene mutations and increased left ventricular wall thickness in the community: the Framingham heart study. Circulation 113(23):2697–2705. https://doi.org/10.1161/CIRCULATIONAHA.105.593558
Colan SD (2010) Hypertrophic cardiomyopathy in childhood. Heart Fail Clin 6(4):433–444. https://doi.org/10.1016/j.hfc.2010.05.004
Panza JA, Petrone RK, Fananapazir L, Maron BJ (1992) Utility of continuous wave doppler echocardiography in the noninvasive assessment of left ventricular outflow tract pressure gradient in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 19(1):91–99. https://doi.org/10.1016/0735-1097(92)90057-t
Maron BJ, Maron MS (2016) The remarkable 50 years of imaging in HCM and how it has changed diagnosis and management: from M-mode echocardiography to CMR. ACC Cardiovasc Imaging 9(7):858–872. https://doi.org/10.1016/j.jcmg.2016.05.003
Pridie RB, Oakley CM (1970) Mechanism of mitral regurgitation in hypertrophic obstructive cardiomyopathy. Br Heart J 32(2):203–208. https://doi.org/10.1136/hrt.32.2.203
Lele SS, Thomson HL, Seo H, Belenkie I, McKenna WJ, Frenneaux MP (1995) Exercise capacity in hypertrophic cardiomyopathy: role of stroke volume limitation, heart rate, and diastolic filling characteristics. Circulation 92:2886–2894. https://doi.org/10.1161/01.cir.92.10.2886
Sharma S, Elliott P, Whyte G, Jones S, Mahon N, Whipp B, McKenna WJ (2000) Utility of cardiopulmonary exercise in the assessment of clinical determinants of functional capacity in hypertrophic cardiomyopathy. Am J Cardiol 86:162–168. https://doi.org/10.1016/s0002-9149(00)00854-7
Magrì D, Agostoni P, Cauti FM et al (2014) Determinants of peak oxygen uptake in patients with hypertrophic cardiomyopathy: a single-center study. Intern Emerg Med 9:293–302. https://doi.org/10.1007/s11739-012-0866-x
Efthimiadis GK, Giannakoulas G, Parcharidou DG et al (2011) Chronotropic incompetence and its relation to exercise intolerance in hypertrophic cardiomyopathy. Int J Cardiol 153:179–184. https://doi.org/10.1016/j.ijcard.2010.08.026
Luo HC, Dimaano VL, Kembro JM et al (2015) Exercise heart rates in patients with hypertrophic cardiomyopathy. Am J Cardiol 115:1144–1450. https://doi.org/10.1016/j.amjcard.2015.01.548
Le VV, Perez MV, Wheeler MT, Myers J, Schnittger I, Ashley EA (2009) Mechanisms of exercise intolerance in patients with hypertrophic cardiomyopathy. Am Heart J 158(3):e27–e34. https://doi.org/10.1016/j.ahj.2009.06.006
Dumont CA, Monserrat L, Peteiro J, Soler R, Rodgriguez E, Bouzas A, Fernandez X, Perez R, Bouzas B, Castro-Beiras A (2007) Relation of left ventricular chamber stiffness at rest to exercise capacity in hypertrophic cardiomyopathy. Am J Cardiol 99:1454–1457. https://doi.org/10.1016/j.amjcard.2006.12.077
Sachdev V, Shizukuda Y, Brenneman CL, Birdsall CW, Waclawiw MA, Arai AE, Mohiddin SA, Tipodi D, Fananapazir L, Plehn JF (2005) Left atrial volumetric remodeling is predictive of functional capacity in nonobstructive hypertrophic cardiomyopathy. Am Heart 149:730–736. https://doi.org/10.1016/j.ahj.2004.07.017
Finocchiaro G, Haddad F, Knowles JW et al (2015) Cardiopulmonary responses and prognosis in hypertrophic cardiomyopathy: a potential role for comprehensive noninvasive hemodynamic assessment. JACC Heart Fail 3:408–418. https://doi.org/10.1016/j.jchf.2014.11.011
Coats CJ, Rantell K, Bartnik A, Patel A, Mist B, McKenna WJ, Elliot PM (2015) Cardiopulmonary exercise testing and prognosis in hypertrophic cardiomyopathy. Circ Heart Fail 8(6):1022–1031. https://doi.org/10.1161/CIRCHEARTFAILURE.114.002248
Magri D, Limongelli G, Re F et al (2016) Cardiopulmonary exercise test and sudden cardiac death risk in hypertrophic cardiomyopathy. Heart 102(8):602–609. https://doi.org/10.1136/heartjnl-2015-308453
Magri D, Re F, Limongelli G et al (2016) Heart failure progression in hypertrophic cardiomyopathy- possible insights from cardiopulmonary exercise testing. Circ J 80(10):2204–2211. https://doi.org/10.1253/circj.CJ-16-0432
Desai MY, Bhonsale A, Patel P, Naji P, Smedira NG, Thamilarasan M, Lytle BW, Lever HM (2014) Exercise echocardiography in asymptomatic HCM: exercise capacity and not LV outflow gradient predicts long-term outcomes. JACC Cardiovasc Imaging 7(1):26–36. https://doi.org/10.1016/j.jcmg.2013.08.010
Sorajja P, Allison T, Hayes C, Nishimura RA, Lam CS, Ommen SR (2012) Prognostic utility of metabolic exercise testing in minimally symptomatic patients with obstructive hypertrophic cardiomyopathy. Am J Cardiol 109:1494–1498. https://doi.org/10.1016/j.amjcard.2012.01.363
Finocchiaro G, Haddad F, Knowles JW et al (2015) Cardiopulmonary responses and prognosis in hypertrophic cardiomyopathy: a potential role for comprehensive noninvasive hemodynamic assessment. J Am Coll Cardiol HF 3(5):408–418. https://doi.org/10.1016/j.jchf.2014.11.011
Masri A, Pierson LM, Smedira NG, Agarwal S, Lytle BW, Naji P, Thamilarasan M, Lever HM, Cho LS, Desai MY (2015) Predictors of long-term outcomes in patients with hypertrophic cardiomyopathy undergoing cardiopulmonary stress testing and echocardiography. Am Heart J 169(5):684-692.e1. https://doi.org/10.1016/j.ahj.2015.02.006
Olivotto I, Maron BJ, Montereggi A, Mazzuoli F, Dolara A, Cecchi F (1999) Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy. J Am Coll Cardiol 33:2044–2051. https://doi.org/10.1016/s0735-1097(99)00094-7
Sadoul N, Prasad K, Elliott PM, Bannerjee S, Frenneaux MP, McKenna WJ (1997) Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation 96(9):2987–2991. https://doi.org/10.1161/01.cir.96.9.2987
Frenneaux MP, Counihan PJ, Caforio AL, Chikamori T, McKenna WJ (1990) Abnormal blood pressure response during exercise in hypertrophic cardiomyopathy. Circulation 82(6):1995–2002. https://doi.org/10.1161/01.cir.82.6.1995
Nagata M, Shimizu M, Ino H, Yamaguchi M, Hayashi K, Taki J, Mabuchi H (2003) Hemodynamic changes and prognosis in patients with hypertrophic cardiomyopathy and abnormal blood pressure responses during exercise. Clin Cardiol 26(2):71–76. https://doi.org/10.1002/clc.4960260206
Maron MS, Olivotto I, Zenovich AG, Link MS, Pandian NG, Kuvin JT, Nistri S, Cecchi F, Udelson JE, Maron BJ (2006) Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation 114(21):2232–2239. https://doi.org/10.1161/CIRCULATIONAHA.106.644682
Rowin EJ, Maron BJ, Olivotto I, Maron MS (2017) Role of exercise testing in hypertrophic cardiomyopathy. JACC Cardiovasc Imaging 10(11):1374–1386. https://doi.org/10.1016/j.jcmg.2017.07.016
Yasui K, Shibata T, Nishizawa T et al (2001) Response of the stroke volume and blood pressure of young patients with nonobstructive hypertrophic cardiomyopathy to exercise. Jpn Circ J 65(4):300–304. https://doi.org/10.1253/jcj.65.300
Barry OM, Gauvreau K, Rhodes J, Reichman JR, Bourette L, Curran T, O’Neill J, Pymm JL, Alexander ME (2018) Incidence and predictors of clinically important and dangerous arrhythmic events during exercise tests in pediatric and congenital heart disease patients. JACC Clin Electrophysiol 4(10):1319–1327. https://doi.org/10.1016/j.jacep.2018.05.018
El Assaad I, Gauvreau K, Rizwan R, Margossian R, Colan S, Chen MH (2020) Value of exercise stress echocardiography in children with hypertrophic cardiomyopathy. J Am Soc Echocardiogr 33(7):888-894.e2. https://doi.org/10.1016/j.echo.2020.01.020
Funding
No specific funding/support.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors have no conflicts/disclosures of interest relative to this article including no relationships with industry.
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
Przybylski, R., Fischer, I.R., Gauvreau, K. et al. Assessment of Exercise Function in Children and Young Adults with Hypertrophic Cardiomyopathy and Correlation with Transthoracic Echocardiographic Parameters. Pediatr Cardiol 43, 1037–1045 (2022). https://doi.org/10.1007/s00246-022-02822-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-022-02822-2