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Published in: The International Journal of Cardiovascular Imaging 4/2016

01-04-2016 | Original Paper

Relationship of basal-septal fibrosis with LV outflow tract obstruction in hypertrophic cardiomyopathy: insights from cardiac magnetic resonance analysis

Authors: Takashi Nakamura, Yoshitaka Iwanaga, Masakazu Yasuda, Takayuki Kawamura, Yuki Miyaji, Hanako Morooka, Shunichi Miyazaki

Published in: The International Journal of Cardiovascular Imaging | Issue 4/2016

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Abstract

Myocardial fibrosis is frequently observed and may be associated with the prognosis in patients with hypertrophic cardiomyopathy (HCM); however, the clinical pathophysiological features, particularly in terms of fibrosis, of hypertrophic obstructive cardiomyopathy (HOCM) remain unclear. This study aimed to determine a role of local fibrosis in HOCM using cardiac magnetic resonance (CMR). 108 consecutive HCM patients underwent CMR. HOCM was defined as a left ventricular outflow tract (LVOT) pressure gradient ≥30 mmHg at rest. Myocardial mass and fibrosis mass by late gadolinium-enhancement CMR (LGE-CMR) were calculated and the distribution/pattern was analyzed using the AHA 17-segment model. LV ejection fraction (LVEF) was significantly higher in patients with HOCM (n = 19) than in those with nonobstructive HCM (n = 89) (P < 0.05). Both total myocardial and fibrosis masses in LV were similar in the two groups (P = 0.385 and P = 0.859, respectively). However, fibrosis in the basal septum was significantly less frequent in the HOCM group than in the nonobstructive HCM group (P < 0.01). The LVOT pressure gradient was significantly higher in the basal-septal non-fibrosis group than in the fibrosis group (23.6 ± 37.3 vs. 4.8 ± 11.4 mmHg, P < 0.01). Multivariate analysis revealed that basal-septal fibrosis was an independent negative predictor of LVOT obstruction in addition to the local wall thickness and LVEF as positive predictors in HCM patients. In conclusion, a significant association was observed between LVOT obstruction and basal septal fibrosis by LGE-CMR in HCM patients. In addition to negative impact of basal-septal fibrosis, basal-septal hypertrophy and preserved global LV contractility may be associated with the pathophysiological features of LVOT obstruction.
Literature
1.
go back to reference Nagueh SF, Mahmarian JJ (2006) Noninvasive cardiac imaging in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 48:2410–2422CrossRefPubMed Nagueh SF, Mahmarian JJ (2006) Noninvasive cardiac imaging in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 48:2410–2422CrossRefPubMed
2.
go back to reference Olivotto I, Cecchi F, Poggesi C, Yacoub MH (2012) Patterns of disease progression in hypertrophic cardiomyopathy: an individualized approach to clinical staging. Circ Heart Fail 5:535–546CrossRefPubMed Olivotto I, Cecchi F, Poggesi C, Yacoub MH (2012) Patterns of disease progression in hypertrophic cardiomyopathy: an individualized approach to clinical staging. Circ Heart Fail 5:535–546CrossRefPubMed
3.
go back to reference Varnava AM, Elliott PM, Mahon N, Davies MJ, McKenna WJ (2001) Relation between myocyte disarray and outcome in hypertrophic cardiomyopathy. Am J Cardiol 88:275–279CrossRefPubMed Varnava AM, Elliott PM, Mahon N, Davies MJ, McKenna WJ (2001) Relation between myocyte disarray and outcome in hypertrophic cardiomyopathy. Am J Cardiol 88:275–279CrossRefPubMed
4.
go back to reference Chan RH, Maron BJ, Olivotto I, Pencina MJ, Assenza GE, Haas T, Lesser JR, Gruner C, Crean AM, Rakowski H, Udelson JE, Rowin E, Lombardi M, Cecchi F, Tomberli B, Spirito P, Formisano F, Biagini E, Rapezzi C, De Cecco CN, Autore C, Cook EF, Hong SN, Gibson CM, Manning WJ, Appelbaum E, Maron MS (2014) Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation 130:484–495CrossRefPubMed Chan RH, Maron BJ, Olivotto I, Pencina MJ, Assenza GE, Haas T, Lesser JR, Gruner C, Crean AM, Rakowski H, Udelson JE, Rowin E, Lombardi M, Cecchi F, Tomberli B, Spirito P, Formisano F, Biagini E, Rapezzi C, De Cecco CN, Autore C, Cook EF, Hong SN, Gibson CM, Manning WJ, Appelbaum E, Maron MS (2014) Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation 130:484–495CrossRefPubMed
5.
go back to reference Kwon DH, Smedira NG, Desai MY (2009) Cardiac magnetic resonance detection of myocardial scarring in hypertrophic cardiomyopathy. J Am Coll Cardiol 54:242–249CrossRefPubMed Kwon DH, Smedira NG, Desai MY (2009) Cardiac magnetic resonance detection of myocardial scarring in hypertrophic cardiomyopathy. J Am Coll Cardiol 54:242–249CrossRefPubMed
6.
go back to reference Maron BJ, Wolfson JK, Epstein SE, Roberts WC (1986) Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy. J Am Coll Cardiol 8:545–557CrossRefPubMed Maron BJ, Wolfson JK, Epstein SE, Roberts WC (1986) Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy. J Am Coll Cardiol 8:545–557CrossRefPubMed
7.
go back to reference Varnava AM, Elliott PM, Sharma S, McKenna WJ, Davies MJ (2000) Hypertrophic cardiomyopathy: the interrelation of disarray, fibrosis, and small vessel disease. Heart 84:476–482CrossRefPubMedPubMedCentral Varnava AM, Elliott PM, Sharma S, McKenna WJ, Davies MJ (2000) Hypertrophic cardiomyopathy: the interrelation of disarray, fibrosis, and small vessel disease. Heart 84:476–482CrossRefPubMedPubMedCentral
8.
go back to reference Ismail TF, Prasad SK, Pennell DJ (2012) Prognostic importance of late gadolinium enhancement cardiovascular magnetic resonance in cardiomyopathy. Heart 98:438–442CrossRefPubMed Ismail TF, Prasad SK, Pennell DJ (2012) Prognostic importance of late gadolinium enhancement cardiovascular magnetic resonance in cardiomyopathy. Heart 98:438–442CrossRefPubMed
9.
go back to reference O’Hanlon R, Grasso A, Roughton M, Moon JC, Clark S, Wage R, Webb J, Kulkarni M, Dawson D, Sulaibeekh L, Chandrasekaran B, Bucciarelli-Ducci C, Pasquale F, Cowie MR, McKenna WJ, Sheppard MN, Elliott PM, Pennell DJ, Prasad SK (2010) Prognostic significance of myocardial fibrosis in hypertrophic cardiomyopathy. J Am Coll Cardiol 56:867–874CrossRefPubMed O’Hanlon R, Grasso A, Roughton M, Moon JC, Clark S, Wage R, Webb J, Kulkarni M, Dawson D, Sulaibeekh L, Chandrasekaran B, Bucciarelli-Ducci C, Pasquale F, Cowie MR, McKenna WJ, Sheppard MN, Elliott PM, Pennell DJ, Prasad SK (2010) Prognostic significance of myocardial fibrosis in hypertrophic cardiomyopathy. J Am Coll Cardiol 56:867–874CrossRefPubMed
10.
go back to reference Maron MS, Appelbaum E, Harrigan CJ, Buros J, Gibson CM, Hanna C, Lesser JR, Udelson JE, Manning WJ, Maron BJ (2008) Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy. Circ Heart Fail 1:184–191CrossRefPubMed Maron MS, Appelbaum E, Harrigan CJ, Buros J, Gibson CM, Hanna C, Lesser JR, Udelson JE, Manning WJ, Maron BJ (2008) Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy. Circ Heart Fail 1:184–191CrossRefPubMed
11.
go back to reference Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P et al (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 35:2733–2779CrossRefPubMed Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P et al (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 35:2733–2779CrossRefPubMed
12.
go back to reference Kramer CM, Barkhausen J, Flamm SD, Kim RJ, Nagel E (2008) Society for cardiovascular magnetic resonance board of trustees task force on standardized protocols. Standardized cardiovascular magnetic resonance imaging (CMR) protocols, society for cardiovascular magnetic resonance: board of trustees task force on standardized protocols. J Cardiovasc Magn Reson 10:35CrossRefPubMedPubMedCentral Kramer CM, Barkhausen J, Flamm SD, Kim RJ, Nagel E (2008) Society for cardiovascular magnetic resonance board of trustees task force on standardized protocols. Standardized cardiovascular magnetic resonance imaging (CMR) protocols, society for cardiovascular magnetic resonance: board of trustees task force on standardized protocols. J Cardiovasc Magn Reson 10:35CrossRefPubMedPubMedCentral
13.
go back to reference Flett AS, Hasleton J, Cook C, Hausenloy D, Quarta G, Ariti C, Muthurangu V, Moon JC (2011) Evaluation of techniques for the quantification of myocardial scar of differing etiology using cardiac magnetic resonance. JACC Cardiovasc Imaging 4:150–156CrossRefPubMed Flett AS, Hasleton J, Cook C, Hausenloy D, Quarta G, Ariti C, Muthurangu V, Moon JC (2011) Evaluation of techniques for the quantification of myocardial scar of differing etiology using cardiac magnetic resonance. JACC Cardiovasc Imaging 4:150–156CrossRefPubMed
14.
go back to reference Vermes E, Childs H, Carbone I, Barckow P, Friedrich MG (2013) Auto-threshold quantification of late gadolinium enhancement in patients with acute heart disease. J Magn Reson Imaging 37:382–390CrossRefPubMed Vermes E, Childs H, Carbone I, Barckow P, Friedrich MG (2013) Auto-threshold quantification of late gadolinium enhancement in patients with acute heart disease. J Magn Reson Imaging 37:382–390CrossRefPubMed
15.
go back to reference Peshock RM, Rokey R, Malloy GM, McNamee P, Buja LM, Parkey RW, Willerson JT (1989) Assessment of myocardial systolic wall thickening using nuclear magnetic resonance. Imaging J Am Co11 Cardiol 14:653–659CrossRef Peshock RM, Rokey R, Malloy GM, McNamee P, Buja LM, Parkey RW, Willerson JT (1989) Assessment of myocardial systolic wall thickening using nuclear magnetic resonance. Imaging J Am Co11 Cardiol 14:653–659CrossRef
16.
go back to reference Bondarenko O, Beek AM, Hofman MB, Kühl HP, Twisk JW, van Dockum WG, Visser CA, van Rossum AC (2005) Standardizing the definition of hyperenhancement in the quantitative assessment of infarct size and myocardial viability using delayed contrast-enhanced CMR. J Cardiovasc Magn Reson 7:481–485CrossRefPubMed Bondarenko O, Beek AM, Hofman MB, Kühl HP, Twisk JW, van Dockum WG, Visser CA, van Rossum AC (2005) Standardizing the definition of hyperenhancement in the quantitative assessment of infarct size and myocardial viability using delayed contrast-enhanced CMR. J Cardiovasc Magn Reson 7:481–485CrossRefPubMed
17.
go back to reference Spiewak M, Malek LA, Misko J, Chojnowska L, Milosz B, Klopotowski M, Petryka J, Dabrowski M, Kepka C, Ruzyllo W (2010) Comparison of different quantification methods of late gadolinium enhancement in patients with hypertrophic cardiomyopathy. Eur J Radiol 74:e149–e153CrossRefPubMed Spiewak M, Malek LA, Misko J, Chojnowska L, Milosz B, Klopotowski M, Petryka J, Dabrowski M, Kepka C, Ruzyllo W (2010) Comparison of different quantification methods of late gadolinium enhancement in patients with hypertrophic cardiomyopathy. Eur J Radiol 74:e149–e153CrossRefPubMed
18.
go back to reference Rudolph A, Abdel-Aty H, Bohl S, Boyé P, Zagrosek A, Dietz R, Schulz-Menger J (2009) Noninvasive detection of fibrosis applying contrast-enhanced cardiac magnetic resonance in different forms of left ventricular hypertrophy relation to remodeling. J Am Coll Cardiol 53:284–291CrossRefPubMed Rudolph A, Abdel-Aty H, Bohl S, Boyé P, Zagrosek A, Dietz R, Schulz-Menger J (2009) Noninvasive detection of fibrosis applying contrast-enhanced cardiac magnetic resonance in different forms of left ventricular hypertrophy relation to remodeling. J Am Coll Cardiol 53:284–291CrossRefPubMed
19.
go back to reference Shirani J, Pick R, Roberts WC, Maron BJ (2000) Morphology and significance of the left ventricular collagen network in young patients with hypertrophic cardiomyopathy and sudden cardiac death. J Am Coll Cardiol 35:36–44CrossRefPubMed Shirani J, Pick R, Roberts WC, Maron BJ (2000) Morphology and significance of the left ventricular collagen network in young patients with hypertrophic cardiomyopathy and sudden cardiac death. J Am Coll Cardiol 35:36–44CrossRefPubMed
20.
go back to reference Lopes LR, Rahman MS, Elliott PM (2013) A systematic review and meta-analysis of genotype-phenotype associations in patients with hypertrophic cardiomyopathy caused by sarcomeric protein mutations. Heart 99:1800–1811CrossRefPubMed Lopes LR, Rahman MS, Elliott PM (2013) A systematic review and meta-analysis of genotype-phenotype associations in patients with hypertrophic cardiomyopathy caused by sarcomeric protein mutations. Heart 99:1800–1811CrossRefPubMed
21.
go back to reference Otsuka H, Arimura T, Abe T, Kawai H, Aizawa Y, Kubo T, Kitaoka H, Nakamura H, Nakamura K, Okamoto H, Ichida F, Ayusawa M, Nunoda S, Isobe M, Matsuzaki M, Doi YL, Fukuda K, Sasaoka T, Izumi T, Ashizawa N, Kimura A (2012) Prevalence and distribution of sarcomeric gene mutations in Japanese patients with familial hypertrophic cardiomyopathy. Circ J 76:453–461CrossRefPubMed Otsuka H, Arimura T, Abe T, Kawai H, Aizawa Y, Kubo T, Kitaoka H, Nakamura H, Nakamura K, Okamoto H, Ichida F, Ayusawa M, Nunoda S, Isobe M, Matsuzaki M, Doi YL, Fukuda K, Sasaoka T, Izumi T, Ashizawa N, Kimura A (2012) Prevalence and distribution of sarcomeric gene mutations in Japanese patients with familial hypertrophic cardiomyopathy. Circ J 76:453–461CrossRefPubMed
22.
go back to reference Kubo T, Kitaoka H, Okawa M, Matsumura Y, Hitomi N, Yamasaki N, Furuno T, Takata J, Nishinaga M, Kimura A, Doi YL (2005) Lifelong left ventricular remodeling of hypertrophic cardiomyopathy caused by a founder frameshift deletion mutation in the cardiac Myosin-binding protein C gene among Japanese. J Am Coll Cardiol. 46:1737–1743CrossRefPubMed Kubo T, Kitaoka H, Okawa M, Matsumura Y, Hitomi N, Yamasaki N, Furuno T, Takata J, Nishinaga M, Kimura A, Doi YL (2005) Lifelong left ventricular remodeling of hypertrophic cardiomyopathy caused by a founder frameshift deletion mutation in the cardiac Myosin-binding protein C gene among Japanese. J Am Coll Cardiol. 46:1737–1743CrossRefPubMed
23.
go back to reference Biagini E, Lorenzini M, Olivotto I, Rocchi G, Lovato L, Lai F, Rosmini S, Pazzi C, Pasquale F, Reggiani ML, Fattori R, Rapezzi C (2012) Effects of myocardial fibrosis assessed by MRI on dynamic left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy: a retrospective database analysis. BMJ Open 2:e001267CrossRefPubMedPubMedCentral Biagini E, Lorenzini M, Olivotto I, Rocchi G, Lovato L, Lai F, Rosmini S, Pazzi C, Pasquale F, Reggiani ML, Fattori R, Rapezzi C (2012) Effects of myocardial fibrosis assessed by MRI on dynamic left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy: a retrospective database analysis. BMJ Open 2:e001267CrossRefPubMedPubMedCentral
24.
go back to reference Ellims AH, Iles LM, Ling LH, Chong B, Macciocca I, Slavin GS, Hare JL, Kaye DM, Marasco SF, McLean CA, James PA, du Sart D, Taylor AJ (2014) A comprehensive evaluation of myocardial fibrosis in hypertrophic cardiomyopathy with cardiac magnetic resonance imaging: linking genotype with fibrotic phenotype. Eur Heart J Cardiovasc Imaging 15:1108–1116CrossRefPubMed Ellims AH, Iles LM, Ling LH, Chong B, Macciocca I, Slavin GS, Hare JL, Kaye DM, Marasco SF, McLean CA, James PA, du Sart D, Taylor AJ (2014) A comprehensive evaluation of myocardial fibrosis in hypertrophic cardiomyopathy with cardiac magnetic resonance imaging: linking genotype with fibrotic phenotype. Eur Heart J Cardiovasc Imaging 15:1108–1116CrossRefPubMed
25.
go back to reference Jensen MK, Prinz C, Horstkotte D, van Buuren F, Bitter T, Faber L, Bundgaard H (2013) Alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy: low incidence of sudden cardiac death and reduced risk profile. Heart 99:1012–1017CrossRefPubMed Jensen MK, Prinz C, Horstkotte D, van Buuren F, Bitter T, Faber L, Bundgaard H (2013) Alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy: low incidence of sudden cardiac death and reduced risk profile. Heart 99:1012–1017CrossRefPubMed
26.
go back to reference Fananapazir L, McAreavey D (1998) Therapeutic options in patients with obstructive hypertrophic cardiomyopathy and severe drug-refractory symptoms. J Am Coll Cardiol 31:259–264CrossRefPubMed Fananapazir L, McAreavey D (1998) Therapeutic options in patients with obstructive hypertrophic cardiomyopathy and severe drug-refractory symptoms. J Am Coll Cardiol 31:259–264CrossRefPubMed
27.
go back to reference van Dockum WG, Beek AM, van Rossum AC (2005) Early onset and progression of left ventricular remodeling after alcohol septal ablation in hypertrophic obstructive cardiomyopathy. Circulation 111:2503–2508CrossRefPubMed van Dockum WG, Beek AM, van Rossum AC (2005) Early onset and progression of left ventricular remodeling after alcohol septal ablation in hypertrophic obstructive cardiomyopathy. Circulation 111:2503–2508CrossRefPubMed
Metadata
Title
Relationship of basal-septal fibrosis with LV outflow tract obstruction in hypertrophic cardiomyopathy: insights from cardiac magnetic resonance analysis
Authors
Takashi Nakamura
Yoshitaka Iwanaga
Masakazu Yasuda
Takayuki Kawamura
Yuki Miyaji
Hanako Morooka
Shunichi Miyazaki
Publication date
01-04-2016
Publisher
Springer Netherlands
Published in
The International Journal of Cardiovascular Imaging / Issue 4/2016
Print ISSN: 1569-5794
Electronic ISSN: 1875-8312
DOI
https://doi.org/10.1007/s10554-015-0806-8

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