Right Ventricle Involvement in Hypertrophic Cardiomyopathy and Role of Cardiac Magnetic Resonance in Hypertrophic Cardiomyopathy: Review Article
DOI:
https://doi.org/10.1532/hsf.3977Keywords:
Cardiac Magnetic Resonance, Hypertrophic Cardiomyopathy, Right Ventricular Dysfunction, Right Ventricular HypertrophyAbstract
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac disease, and its main characteristic is symmetrical or asymmetrical hypertrophy of the left ventricle and/or right ventricle. Most previous studies mainly include the left ventricle for definition of HCM, thus neglecting the right ventricle. But recently, many studies have reported the right ventricular involvement in HCM. Histopathological results showed that similar pathogenic changes in both the right and left ventricles, which suggests common myopathic processes and sarcomere genetic mutations.
Cardiovascular magnetic resonance (CMR) is a gold standard imaging modality to assess heart anatomy and function and provides highly accurate and reproducible measurements. CMR is very useful in characterizing the various phenotypes of right and left ventricles in HCM. CMR also can be useful in detecting early and dominant phenotypic expression of HCM. Due to the complex geometry of the right ventricle and its retrosternal position, echocardiography may not provide accurate measurements. CMR also provides more accurate and repeatable right ventricular measurements. Thus, right ventricle evaluation along with left ventricle should routinely be done for better assessment of HCM patients.
References
Alcalai R, Seidman JG, Seidman CE. 2008. Genetic basis of hypertrophic cardiomyopathy: from bench to the clinics. J Cardiovasc Electrophysiol. 19(1):104-10.
Ashrafian H, Watkins H. 2007. Reviews of translational medicine and genomics in cardiovascular disease: new disease taxonomy and therapeutic implications cardiomyopathies: therapeutics based on molecular phenotype. J Am Coll Cardiol. 49(12):1251-64.
Authors/Task Force m, Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, et al. 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):2733-79.
Bhushan S, Huang X, Li Y, et al. 2020. Evaluation of right ventricle pulmonary artery coupling on right ventricular function in post operative Tetralogy of Fallot patients underwent for pulmonary valve replacement. Journal of Cardiothoracic Surgery.
Dhillon A, Desai MY. 2011. Cardiac magnetic resonance in hypertrophic cardiomyopathy. JACC Cardiovasc Imaging. 4(10):1123-37.
Elliott P, McKenna WJ. 2004. Hypertrophic cardiomyopathy. Lancet. 363(9424):1881-91.
Foale R PN, McKenna W, et al. 1986. Echocardiographic measurement of the normal adult right ventricle. Br Heart J. 56(1):33-44.
Frank S, Braunwald E. 1968. Idiopathic hypertrophic subaortic stenosis. Clinical analysis of 126 patients with emphasis on the natural history. Circulation. 37(5):759-88.
Galea N, Carbone I, Cannata D, Cannavale G, Conti B, Galea R, et al. 2013. Right ventricular cardiovascular magnetic resonance imaging: normal anatomy and spectrum of pathological findings. Insights Imaging. 4(2):213-23.
Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, et al. 2011. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 58(25):2703-38.
Greupner J, Zimmermann E, Grohmann A, Dubel HP, Althoff TF, Borges AC, et al. 2012. Head-to-head comparison of left ventricular function assessment with 64-row computed tomography, biplane left cineventriculography, and both 2- and 3-dimensional transthoracic echocardiography: comparison with magnetic resonance imaging as the reference standard. J Am Coll Cardiol. 59(21):1897-907.
Guo X, Fan C, Tian L, Liu Y, Wang H, Zhao S, et al. 2017. The clinical features, outcomes and genetic characteristics of hypertrophic cardiomyopathy patients with severe right ventricular hypertrophy. PLoS One. 12(3):e0174118.
Guo YK, Gao HL, Zhang XC, Wang QL, Yang ZG, Ma ES. 2010. Accuracy and reproducibility of assessing right ventricular function with 64-section multi-detector row CT: comparison with magnetic resonance imaging. Int J Cardiol. 139(3):254-62.
Harris KM, Spirito P, Maron MS, Zenovich AG, Formisano F, Lesser JR, et al. 2006. Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation. 114(3):216-25.
Kawashiri MA, Hayashi K, Konno T, Fujino N, Ino H, Yamagishi M. 2014. Current perspectives in genetic cardiovascular disorders: from basic to clinical aspects. Heart Vessels. 29(2):129-41.
Kuehne T, Yilmaz S, Steendijk P, Moore P, Groenink M, Saaed M, et al. 2004. Magnetic resonance imaging analysis of right ventricular pressure-volume loops: in vivo validation and clinical application in patients with pulmonary hypertension. Circulation. 110(14):2010-6.
Kukulski T, Hubbert L, Arnold M, Wranne B, Hatle L, Sutherland GR. 2000. Normal regional right ventricular function and its change with age: a Doppler myocardial imaging study. J Am Soc Echocardiogr. 13(3):194-204.
Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA, et al. 2006. Recommendations for chamber quantification. Eur J Echocardiogr. 7(2):79-108.
Marian AJ, Roberts R. 2001. The molecular genetic basis for hypertrophic cardiomyopathy. J Mol Cell Cardiol. 33(4):655-70.
Maron BJ. 1993. Hypertrophic cardiomyopathy. Curr Probl Cardiol. 1993;18(11):639-704.
Maron BJ. 2002. Hypertrophic cardiomyopathy: a systematic review. JAMA. 2002;287(10):1308-20.
Maron BJ. 2004. Hypertrophic cardiomyopathy: an important global disease. Am J Med. 2004;116(1):63-5.
Maron MS, Hauser TH, Dubrow E, Horst TA, Kissinger KV, Udelson JE, et al. 2007. Right ventricular involvement in hypertrophic cardiomyopathy. Am J Cardiol. 100(8):1293-8.
McKenna WJ, Kleinebenne A, Nihoyannopoulos P, Foale R. 1988. Echocardiographic measurement of right ventricular wall thickness in hypertrophic cardiomyopathy: relation to clinical and prognostic features. J Am Coll Cardiol. 11(2):351-8.
Mittal SR. 2010. Isolated right ventricular hypertrophic obstructive cardiomyopathy. J Assoc Physicians India. 58:249-50.
Nagata Y, Konno T, Fujino N, Hodatsu A, Nomura A, Hayashi K, et al. 2015. Right ventricular hypertrophy is associated with cardiovascular events in hypertrophic cardiomyopathy: evidence from study with magnetic resonance imaging. Can J Cardiol. 31(6):702-8.
Prinz C, van Buuren F, Faber L, Bitter T, Bogunovic N, Burchert W, et al. 2012. Myocardial fibrosis is associated with biventricular dysfunction in patients with hypertrophic cardiomyopathy. Echocardiography. 29(4):438-44.
Redington AN. 2002. Right ventricular function. Cardiol Clin. 20(3):341-9, v.
Rowin EJ, Maron MS. 2016. The Role of Cardiac MRI in the Diagnosis and Risk Stratification of Hypertrophic Cardiomyopathy. Arrhythm Electrophysiol Rev. 5(3):197-202.
Sandeep B, Huang X, Xu F, et al. 2019. Etiology of right ventricular restrictive physiology early after repair of tetralogy of Fallot in pediatric patients[J]. Journal of Cardiothoracic Surgery. 14(1).
Shimizu M, Kawai H, Yokota Y, Yokoyama M. 1993. Echocardiographic assessment of right ventricular obstruction in hypertrophic cardiomyopathy. Circ J. 67(10):855-60.
Weissler-Snir A, Adler A, Williams L, Gruner C, Rakowski H. 2017. Prevention of sudden death in hypertrophic cardiomyopathy: bridging the gaps in knowledge. Eur Heart J. 38(22):1728-37.
Zemanek D, Tomasov P, Prichystalova P, Linhartova K, Veselka J. 2010. Evaluation of the right ventricular function in hypertrophic obstructive cardiomyopathy: a strain and tissue Doppler study. Physiol Res. 59(5):697-702.
