Prevalence, determinants, and prognostic significance of exercise-induced pulmonary hypertension in patients with hypertrophic cardiomyopathy
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Exercise-induced pulmonary hypertension (EIPH) is associated with worse outcomes in patients with heart failure or valvular heart disease. However, little is known regarding the implications of EIPH in hypertrophic cardiomyopathy (HCM) patients. We retrospectively reviewed data of consecutive HCM patients who underwent clinically indicated exercise echocardiography using a semi-supine bicycle ergometer at our hospital. EIPH was defined as pulmonary artery systolic pressure ≥ 60 mmHg during exercise. The incidences of HCM-related mortality and HCM-related morbidity during follow-up period were evaluated. Of 42 patients (mean age 59 ± 21 years; 4 with resting obstruction, 19 with provoked obstruction, and 19 without obstruction), 16 (38%) developed EIPH. Patients with EIPH had significantly longer resting E wave deceleration time (271 ± 116 vs. 213 ± 66 ms; P = 0.04), higher resting pulmonary artery systolic pressure (35 ± 6 vs. 31 ± 5 mmHg; P = 0.04), and higher B-type natriuretic peptide level (283 [222, 465] vs. 142 [54, 423] pg/ml; P = 0.04) than those without EIPH. Kaplan–Meier curve analysis demonstrated that EIPH was significantly associated with HCM-related morbidity (log-rank; P = 0.01). In Cox regression analysis, EIPH was a significant predictor of HCM-related morbidity (hazard ratio: 5.98, 95% confidence interval 1.36–41.07; P = 0.02). In conclusion, EIPH was documented in about one-third of HCM patients. EIPH was a significant predictor of HCM-related morbidity in patients with HCM.
KeywordsExercise-induced pulmonary hypertension Hypertrophic cardiomyopathy Exercise echocardiography
We sincerely appreciate the help of the sonographer (Aiko Koda and Hitomi Nishimura), and medical staff in the National Cerebral and Cardiovascular Center.
This research did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.
Compliance with ethical standards
Conflict of interest
The authors declare that there is no conflict of interest.
- 1.Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M (2016) 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 37(1):67–119. https://doi.org/10.1093/eurheartj/ehv317 CrossRefGoogle Scholar
- 2.Vachiery JL, Adir Y, Barbera JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galie N, Ghio S, Gibbs JS, Martinez F, Semigran M, Simonneau G, Wells A, Seeger W (2013) Pulmonary hypertension due to left heart diseases. J Am Coll Cardiol 62(25 Suppl):D100–D108. https://doi.org/10.1016/j.jacc.2013.10.033 CrossRefPubMedGoogle Scholar
- 4.Shim CY, Kim SA, Choi D, Yang WI, Kim JM, Moon SH, Lee HJ, Park S, Choi EY, Chung N, Ha JW (2011) Clinical outcomes of exercise-induced pulmonary hypertension in subjects with preserved left ventricular ejection fraction: implication of an increase in left ventricular filling pressure during exercise. Heart 97(17):1417–1424. https://doi.org/10.1136/hrt.2010.220467 CrossRefPubMedGoogle Scholar
- 7.Lancellotti P, Magne J, Donal E, O’Connor K, Dulgheru R, Rosca M, Pierard LA (2012) Determinants and prognostic significance of exercise pulmonary hypertension in asymptomatic severe aortic stenosis. Circulation 126(7):851–859. https://doi.org/10.1161/CIRCULATIONAHA.111.088427 CrossRefPubMedGoogle Scholar
- 8.Covella M, Rowin EJ, Hill NS, Preston IR, Milan A, Opotowsky AR, Maron BJ, Maron MS, Maron BA (2017) Mechanism of progressive heart failure and significance of pulmonary hypertension in obstructive hypertrophic cardiomyopathy. Circ Heart Fail 10(4):e003689. https://doi.org/10.1161/CIRCHEARTFAILURE.116.003689 CrossRefPubMedPubMedCentralGoogle Scholar
- 9.Musumeci MB, Mastromarino V, Casenghi M, Tini G, Francia P, Maruotti A, Romaniello A, Magri D, Lillo R, Adduci C, Volpe M, Autore C (2017) Pulmonary hypertension and clinical correlates in hypertrophic cardiomyopathy. Int J Cardiol 248:326–332. https://doi.org/10.1016/j.ijcard.2017.07.010 CrossRefPubMedGoogle Scholar
- 10.Ong KC, Geske JB, Hebl VB, Nishimura RA, Schaff HV, Ackerman MJ, Klarich KW, Siontis KC, Coutinho T, Dearani JA, Ommen SR, Gersh BJ (2016) Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy. Eur Heart J Cardiovasc Imaging 17(6):604–610. https://doi.org/10.1093/ehjci/jew024 CrossRefPubMedGoogle Scholar
- 12.Finocchiaro G, Knowles JW, Pavlovic A, Perez M, Magavern E, Sinagra G, Haddad F, Ashley EA (2014) Prevalence and clinical correlates of right ventricular dysfunction in patients with hypertrophic cardiomyopathy. Am J Cardiol 113(2):361–367. https://doi.org/10.1016/j.amjcard.2013.09.045 CrossRefPubMedGoogle Scholar
- 13.Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, Flachskampf FA, Foster E, Goldstein SA, Kuznetsova T, Lancellotti P, Muraru D, Picard MH, Rietzschel ER, Rudski L, Spencer KT, Tsang W, Voigt JU (2015) Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr 28(1):1–39 e14. https://doi.org/10.1016/j.echo.2014.10.003 CrossRefGoogle Scholar
- 14.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. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 58 (25):e212–e260. https://doi.org/10.1016/j.jacc.2011.06.011 CrossRefPubMedGoogle Scholar
- 15.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(39):2733–2779. https://doi.org/10.1093/eurheartj/ehu284 CrossRefPubMedGoogle Scholar
- 17.Lim AY, Kim C, Park SJ, Choi JO, Lee SC, Park SW (2017) Clinical characteristics and determinants of exercise-induced pulmonary hypertension in patients with preserved left ventricular ejection fraction. Eur Heart J Cardiovasc Imaging 18(3):276–283. https://doi.org/10.1093/ehjci/jew199 CrossRefPubMedGoogle Scholar
- 18.Pellikka PA, Nagueh SF, Elhendy AA, Kuehl CA, Sawada SG (2007) American Society of Echocardiography recommendations for performance, interpretation, and application of stress echocardiography. J Am Soc Echocardiogr 20(9):1021–1041. https://doi.org/10.1016/j.echo.2007.07.003 CrossRefPubMedGoogle Scholar
- 19.Lancellotti P, Pellikka PA, Budts W, Chaudhry FA, Donal E, Dulgheru R, Edvardsen T, Garbi M, Ha JW, Kane GC, Kreeger J, Mertens L, Pibarot P, Picano E, Ryan T, Tsutsui JM, Varga A (2016) The clinical use of stress echocardiography in non-ischaemic heart disease: recommendations from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. Eur Heart J Cardiovasc Imaging 17(11):1191–1229. https://doi.org/10.1093/ehjci/jew190 CrossRefGoogle Scholar
- 21.Kamijima R, Suzuki K, Izumo M, Kuwata S, Mizukoshi K, Takai M, Kou S, Hayashi A, Kida K, Harada T, Akashi YJ (2017) Predictors of exercise-induced pulmonary hypertension in patients with asymptomatic degenerative mitral regurgitation: mechanistic insights from 2D speckle-tracking echocardiography. Sci Rep 7:40008. https://doi.org/10.1038/srep40008 CrossRefPubMedPubMedCentralGoogle Scholar
- 22.Suzuki K, Izumo M, Yoneyama K, Mizukoshi K, Kamijima R, Kou S, Takai M, Kida K, Watanabe S, Omiya K, Nobuoka S, Akashi YJ (2015) Influence of exercise-induced pulmonary hypertension on exercise capacity in asymptomatic degenerative mitral regurgitation. J Cardiol 66(3):246–252. https://doi.org/10.1016/j.jjcc.2014.11.005 CrossRefPubMedGoogle Scholar
- 23.Nagueh SF, Smiseth OA, Appleton CP, Byrd BF III, Dokainish H, Edvardsen T, Flachskampf FA, Gillebert TC, Klein AL, Lancellotti P, Marino P, Oh JK, Alexandru Popescu B, Waggoner AD (2016) Recommendations for the evaluation of left ventricular diastolic function by echocardiography: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 17(12):1321–1360. https://doi.org/10.1093/ehjci/jew082 CrossRefPubMedGoogle Scholar
- 24.Cardim N, Galderisi M, Edvardsen T, Plein S, Popescu BA, D’Andrea A, Bruder O, Cosyns B, Davin L, Donal E, Freitas A, Habib G, Kitsiou A, Petersen SE, Schroeder S, Lancellotti P, Camici P, Dulgheru R, Hagendorff A, Lombardi M, Muraru D, Sicari R (2015) Role of multimodality cardiac imaging in the management of patients with hypertrophic cardiomyopathy: an expert consensus of the European Association of Cardiovascular Imaging Endorsed by the Saudi Heart Association. Eur Heart J Cardiovasc Imaging 16(3):280. https://doi.org/10.1093/ehjci/jeu291 CrossRefPubMedGoogle Scholar
- 25.Nishimura RA, Appleton CP, Redfield MM, Ilstrup DM, Holmes DR, Tajik AJ (1996) Noninvasive doppler echocardiographic evaluation of left ventricular filling pressures in patients with cardiomyopathies: a simultaneous doppler echocardiographic and cardiac catheterization study. J Am Coll Cardiol 28(5):1226–1233. https://doi.org/10.1016/s0735-1097(96)00315-4 CrossRefPubMedGoogle Scholar
- 27.Chen YZ, Duan FJ, Yuan JS, Hu FH, Cui JG, Yang WX, Zhang Y, Wang H, Qiao SB (2016) Effects of alcohol septal ablation on left ventricular diastolic filling patterns in obstructive hypertrophic cardiomyopathy. Heart Vessels 31(5):744–751. https://doi.org/10.1007/s00380-015-0656-2 CrossRefPubMedGoogle Scholar
- 28.Jassal DS, Neilan TG, Fifer MA, Palacios IF, Lowry PA, Vlahakes GJ, Picard MH, Yoerger DM (2006) Sustained improvement in left ventricular diastolic function after alcohol septal ablation for hypertrophic obstructive cardiomyopathy. Eur Heart J 27(15):1805–1810. https://doi.org/10.1093/eurheartj/ehl106 CrossRefPubMedGoogle Scholar