Advertisement
Mayo Clinic Proceedings Home

Right Ventricular Enlargement and Dysfunction Are Associated With Increased All-Cause Mortality in Hypertrophic Cardiomyopathy

      Abstract

      Objective

      To assess whether right ventricular enlargement (RVE) and right ventricular dysfunction (RVD) adversely affect prognosis in hypertrophic cardiomyopathy (HCM).

      Patients and Methods

      Data were retrieved from Mayo Clinic’s prospectively collected HCM registry between January 1, 2000, and September 30, 2012. Right ventricle (RV) size and function were semiquantitatively categorized via echocardiography as normal (RV-Norm) versus abnormal (RV-Abn) (RVE or RVD). All-cause mortality was the primary endpoint.

      Results

      Of 1878 HCM patients studied (mean age 53±15 years; 41.6% female), only 71 (3.8%) had RV-Abn (24 RVE, 28 RVD, 19 combined RVE and RVD). Compared with HCM patients with RV-Norm, RV-Abn patients were older (57±14 vs 53±15 years, P=.02), more symptomatic (New York Heart Association functional class III-IV in 62.0% vs 48.6%, P=.03), had more atrial fibrillation (53.5% vs 17.3%, P<.001), and more prior implantable cardioverter-defibrillator implantation (23.9% vs 11.3%, P=.02). Median follow-up was 9.4 years with 311 deaths. Patients who were RV-Abn had higher all-cause mortality compared with RV-Norm (log-rank P<.001); 24.1% (95% CI, 15.5% to 35.3%) vs 6.1% (95% CI, 5.1% to 7.3%) at 5 years. In multivariable Cox modeling, RV-Abn (hazard ratio, 1.89; 95% CI, 1.18 to 3.03; P=.008) was associated independently with all-cause mortality after adjusting for age, female sex, New York Heart Association functional class, atrial fibrillation, hypertension, coronary artery disease, implantable cardioverter-defibrillator implantation, beta blocker use, prior septal reduction therapy, resting LV outflow tract gradient, maximal LV wall thickness, and moderate or greater tricuspid regurgitation.

      Conclusion

      Although perturbations in RV size and function were observed in fewer than 5% of patients with HCM, they were associated with nearly two-fold higher all-cause mortality at long-term follow-up.

      Abbreviations and Acronyms:

      HCM (hypertrophic cardiomyopathy), HR (hazard ratio), ICD (implantable cardioverter-defibrillator), LA (left atrium), LV (left ventricle), PH (pulmonary hypertension), RA (right atrium), RV (right ventricle), RV-Abn (abnormal right ventricle), RVD (right ventricular dysfunction), RVE (right ventricular enlargement), RV-Norm (normal right ventricle), RVSP (right ventricular systolic pressure), SCD (sudden cardiac death)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Mayo Clinic Proceedings
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Maron B.J.
        • Ommen S.R.
        • Semsarian C.
        • Spirito P.
        • Olivotto I.
        • Maron M.S.
        Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine.
        J Am Coll Cardiol. 2014; 64: 83-99
        • Maron B.J.
        • Gardin J.M.
        • Flack J.M.
        • Gidding S.S.
        • Kurosaki T.T.
        • Bild D.E.
        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. 1995; 92: 785-789
        • Maron B.J.
        Hypertrophic cardiomyopathy.
        Lancet. 1997; 350: 127-133
        • Weissler-Snir A.
        • Allan K.
        • Cunningham K.
        • et al.
        Hypertrophic cardiomyopathy-related sudden cardiac death in young people in Ontario.
        Circulation. 2019; 140: 1706-1716
        • Maron B.J.
        • Olivotto I.
        • Spirito P.
        • et al.
        Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population.
        Circulation. 2000; 102: 858-864
        • Maron B.J.
        • Rowin E.J.
        • Casey S.A.
        • et al.
        Risk stratification and outcome of patients with hypertrophic cardiomyopathy ≥60 years of age.
        Circulation. 2013; 127: 585-593
        • McKenna W.J.
        • Kleinebenne A.
        • Nihoyannopoulos P.
        • Foale R.
        Echocardiographic measurement of right ventricular wall thickness in hypertrophic cardiomyopathy: relation to clinical and prognostic features.
        J Am Coll Cardiol. 1988; 11: 351-358
        • Finocchiaro G.
        • Knowles J.W.
        • Pavlovic A.
        • et al.
        Prevalence and clinical correlates of right ventricular dysfunction in patients with hypertrophic cardiomyopathy.
        Am J Cardiol. 2014; 113: 361-367
        • Shah J.P.
        • Yang Y.
        • Chen S.
        • et al.
        Prevalence and prognostic significance of right ventricular dysfunction in patients with hypertrophic cardiomyopathy.
        Am J Cardiol. 2018; 122: 1932-1938
        • Mörner S.
        • Lindqvist P.
        • Waldenström A.
        • Kazzam E.
        Right ventricular dysfunction in hypertrophic cardiomyopathy as evidenced by the myocardial performance index.
        Int J Cardiol. 2008; 124: 57-63
        • Nagata Y.
        • Konno T.
        • Fujino N.
        • et al.
        Right ventricular hypertrophy is associated with cardiovascular events in hypertrophic cardiomyopathy: evidence from study with magnetic resonance imaging.
        Can J Cardiol. 2015; 31: 702-708
        • Kammerlander A.A.
        • Marzluf B.A.
        • Graf A.
        • et al.
        Right ventricular dysfunction, but not tricuspid regurgitation, is associated with outcome late after left heart valve procedure.
        J Am Coll Cardiol. 2014; 64: 2633-2642
        • Padang R.
        • Chandrashekar N.
        • Indrabhinduwat M.
        • et al.
        Aetiology and outcomes of severe right ventricular dysfunction.
        Eur Heart J. 2020; 41: 1273-1282
        • Vonk Noordegraaf A.
        • Galie N.
        The role of the right ventricle in pulmonary arterial hypertension.
        Eur Respir Rev. 2011; 20: 243-253
        • Geske J.B.
        • Ong K.C.
        • Siontis K.C.
        • et al.
        Women with hypertrophic cardiomyopathy have worse survival.
        Eur Heart J. 2017; 38: 3434-3440
        • Finocchiaro G.
        • Haddad F.
        • Knowles J.W.
        • et al.
        Cardiopulmonary responses and prognosis in hypertrophic cardiomyopathy: a potential role for comprehensive noninvasive hemodynamic assessment.
        J Am Coll Cardiol HF. 2015; 3: 408-418
        • Rudski L.G.
        • Lai W.W.
        • Afilalo J.
        • et al.
        Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography.
        J Am Soc Echocardiogr. 2010; 23 (quiz 786): 685-713
        • Mohammed S.F.
        • Hussain I.
        • AbouEzzeddine O.F.
        • et al.
        Right ventricular function in heart failure with preserved ejection fraction: a community-based study.
        Circulation. 2014; 130: 2310-2320
        • Lang R.M.
        • Badano L.P.
        • Mor-Avi V.
        • et al.
        Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging.
        Eur Heart J Cardiovasc Imaging. 2015; 16: 233-270
        • Obokata M.
        • Kane G.C.
        • Sorimachi H.
        • et al.
        Noninvasive evaluation of pulmonary artery pressure during exercise: the importance of right atrial hypertension.
        Eur Respir J. 2020; 55: 1901617
        • Ong K.C.
        • Geske J.B.
        • Hebl V.B.
        • et al.
        Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy.
        Eur Heart J Cardiovasc Imaging. 2016; 17: 604-610
        • Hiemstra Y.L.
        • Debonnaire P.
        • Bootsma M.
        • et al.
        Prevalence and prognostic implications of right ventricular dysfunction in patients with hypertrophic cardiomyopathy.
        Am J Cardiol. 2019; 124: 604-612
        • Kane G.C.
        • Maradit-Kremers H.
        • Slusser J.P.
        • Scott C.G.
        • Frantz R.P.
        • McGoon M.D.
        Integration of clinical and hemodynamic parameters in the prediction of long-term survival in patients with pulmonary arterial hypertension.
        Chest. 2011; 139: 1285-1293
        • Dwivedi S.K.
        • Bansal S.
        • Puri A.
        • et al.
        Diastolic and systolic right ventricular dysfunction precedes left ventricular dysfunction in patients paced from right ventricular apex.
        Indian Pacing Electrophysiol J. 2006; 6: 142-152
        • Redfield M.M.
        • Jacobsen S.J.
        • Borlaug B.A.
        • Rodeheffer R.J.
        • Kass D.A.
        Age- and gender related ventricular-vascular stiffening — a community-based study.
        Circulation. 2005; 112: 2254-2262
        • Geske J.B.
        • Konecny T.
        • Ommen S.R.
        • et al.
        Surgical myectomy improves pulmonary hypertension in obstructive hypertrophic cardiomyopathy.
        Eur Heart J. 2014; 35: 2032-2039
        • Konecny T.
        • Somers V.K.
        Sleep-disordered breathing in hypertrophic cardiomyopathy: challenges and opportunities.
        Chest. 2014; 146: 228-234
        • Rowin E.J.
        • Sridharan A.
        Thinking outside the heart to treat atrial fibrillation in hypertrophic cardiomyopathy.
        J Am Heart Assoc. 2020; 9e016260