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The mechanisms, diagnosis and management of MR in MVP and HCM

DISCOVERIES (ISSN 2359-7232), 2016, April-June issue

CITATION: 

Popa MO, Irimia AM, Papagheorghe MN, Vasile EM, Tircol SA, Negulescu RA et al. The mechanisms, diagnosis and management of mitral regurgitation in mitral valve prolapse and hypertrophic cardiomyopathy. Discoveries 2016, Apr-Jun; 4(2): e61. DOI: 10.15190/d.2016.8

Submitted: June 19, 2016; Revised: June 30, 2016; Accepted: June 30, 2016; Published: June 30, 2016;

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The mechanisms, diagnosis and management of mitral regurgitation in mitral valve prolapse and hypertrophic cardiomyopathy

Mihaela Octavia Popa (1,*), Ana Maria Irimia (1), Mihai Nicolae Papagheorghe(1), Elena Miruna Vasile (1), Simona Andreea Tircol (1), Raluca Andreea Negulescu (1), Catalina Toader (1), Robert Adam (1), Lucian Dorobantu (2), Cristina Caldararu (3), Maria Alexandrescu (4), Sebastian Onciul (5)

(1) Carol Davila University of Medicine and Pharmacy, Bucharest, Romania; 

(2) Department of Cardiovascular Surgery, Monza Hospital, Bucharest, Romania; 

(3) Department of Cardiology, Monza Hospital, Bucharest, Romania; 

(4) Department of Radiology and Imaging Sciences, Monza Hospital, Bucharest, Romania; 

(5) Department of Cardiology, Floreasca Clinical Emergency Hospital, Bucharest, Romania;

*Correspondence to: Mihaela Octavia Popa, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania; Phone: +4 0727754559; Email: mihaela.octavia@yahoo.com 

Abstract

     Valvular disease is a frequent cardiac pathology leading to heart failure and, ultimately, death. Mitral regurgitation, defined as the inability of the two mitral leaflets to coapt, is a common valvular disease and a self sustained pathology. A better understanding of the mitral valve histological layers provides a better understanding of the leaflet and chordae changes in mitral valve prolapse. 

Mitral valve prolapse may occur in myxomatous degenerative abnormalities, connective tissue disorders or in sporadic isolated cases. It is the most common mitral abnormality of non-ischemic cause leading to severe surgery-requiring mitral regurgitation. In addition to standard echocardiographic investigations, newly implemented three-dimensional techniques are being used and they permit a better visualisation, from the so-called ‘surgical view’, and an improved evaluation of the mitral valve. 

     Hypertrophic cardiomyopathy is the most frequent inherited myocardial disease caused by mutations in various genes encoding proteins of the cardiac sarcomere, leading to a marked left ventricular hypertrophy unexplained by other comorbidities. The pathological echocardiographic hallmarks of hypertrophic cardiomyopathy are left ventricular hypertrophy, left ventricular outflow tract obstruction and systolic anterior motion of the mitral valve. The systolic anterior motion of the mitral valve contributes to the development of mitral regurgitation and further narrows the left ventricular outflow tract, leading to more severe symptomatology. Cardiac magnetic resonance imaging accurately measures the left ventricular mass, the degree of diastolic function and it may also be used to distinguish phenotypic variants. The clinical outcome of patients with these pathologies is mostly determined by the selected option of treatment. 

     The purpose of surgical correction regarding mitral valve involvement is to restore valvular competence. Surgery has proven to be the only useful treatment in preventing heart failure, improving symptomatology and reducing mortality. Our approach wishes to enhance the understanding of the mitral valve’s involvement in hypertrophic cardiomyopathy and mitral valve prolapse from genetic, haemodynamic and clinical perspectives, as well as to present novelties in the grand field of treatment.

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107. Teo EP, Teoh JG, Hung J. Mitral valve and papillary muscle abnormalities in hypertrophic obstructive cardiomyopathy. Current Opinion in Cardiology. 2015 Sep;30(5):475–82. doi:10.1097/HCO.0000000000000200.

108. Moon JC, Reed E, Sheppard MN, Elkington AG, Ho SY, Burke M et al. The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2004 Jun 16;43(12):2260-4.

109. Elliott P, McKenna W. Hypertrophic cardiomyopathy. Lancet (London, England). 2004 Jun 9; 363(9424):1881–91.

110. Hughes SE. The pathology of hypertrophic cardiomyopathy. Histopathology. 2004 May 14; 44(5):412–27.

111. Walker CM, Reddy GP, Mohammed T-LH, Chung JH. Systolic anterior motion of the mitral valve. J Thorac Imaging. 2012 Jul;27(4):W87. doi: 10.1097/RTI.0b013e31825412dd.

112. Maron BJ, Epstein SE. Hypertrophic cardiomyopathy. Recent observations regarding the specificity of three hallmarks of the disease: asymmetric septal hypertrophy, septal disorganization and systolic anterior motion of the anterior mitral leaflet.Am J Cardiol. 1980 Jan; 45(1):141-54.

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