Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant

Divyesh Dadhania; Jineesh Valakada; Anoop Ayyappan; Ansan Joseph

doi:doi:10.11648/j.sjcm.20211004.14

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Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant

Divyesh Dadhania, Jineesh Valakada, Anoop Ayyappan, Ansan Joseph

Published in Science Journal of Clinical Medicine (Volume 10, Issue 4)

Received: 24 May 2021 Accepted: 13 July 2021 Published: 10 November 2021

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Abstract

Hypertrophic cardiomyopathy (HCM) is characterized by cardiac muscle hypertrophy, unexplained by loading conditions with a nondilated left ventricle and normal or increased ejection fraction Midventricular obstruction of the left ventricle is an increasingly recognized phenotypic variant of hypertrophic cardiomyopathy due to the widespread availability of Cardiac Magnetic Resonance Imaging (CMR). Mid ventricular obstruction with apical aneurysm is a high risk variant of HCM which can lead to higher risk of arrhythmia and SCD. This variant is clinically and prognostically significant with MRI playing a major role in detection. CMR has nearly 100 % sensitivity in diagnosis and also helps in identifying other high risk features. CMR is helpful in detecting Late gadolinium enhancement (LGE) and apical aneurysm for which echocardiography is less sensitive. LGE represent area of replacement fibrosis which can act as a source for life threatening arrhythmia. Although this variant is not considered high risk feature for sudden cardiac death (SCD) in HCM cohort according to standard guideline, it is increasingly being recognized as a high risk feature by recent studies. Treatment is controversial however patients with multiple risk factors of SCD can be treated with ICD implantation to prevent catastrophic events. Herein we present a case of HCM with midventricular obstruction where MRI showed apical aneurysm with scarring and midventricular gradient. Incidence, pathophysiology, diagnosis role of CMR and treatment of this variant of HCM is discussed.

Published in	Science Journal of Clinical Medicine (Volume 10, Issue 4)
DOI	10.11648/j.sjcm.20211004.14
Page(s)	102-105
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Heart, Myocardium, Hypertrophy, Hypertrophic Cardiomyopathy, MRI, Fibrosis, Ischemia, Scar

References

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[2]	Manning, W. and Pennell, D., Cardiovascular Magnetic Resonance. 3^rd edition: A Companion To Braunwald's Heart Disease.
[3]	Rowin, E., Maron, B., Haas, T., Garberich, R., Wang, W., Link, M. and Maron, M., 2017. Hypertrophic Cardiomyopathy With Left Ventricular Apical Aneurysm. Journal of the American College of Cardiology, 69 (7), pp. 761-773.
[4]	K. Ananthasubramaniam, “Hypertrophic cardiomyopathy,” in Current Diagnosis & Treatment: Cardiology, M. H. Crawford, Ed., pp. 301–313, McGraw-Hill Education, New York, NY, USA, 4th edition, 2014.
[5]	Elsheshtawy, M., Mahmoud, A., Abdelghany, M., Suen, I., Sadiq, A. and Shani, J., 2018. Left ventricular aneurysms in hypertrophic cardiomyopathy with midventricular obstruction: A systematic review of literature. Pacing and Clinical Electrophysiology, 41 (7), pp. 854-865.
[6]	Gibelli, G., Biasi, S. and Buonamici, V., 2013. Severe midventricular hypertrophic obstructive cardiomyopathy and apical aneurysm. Journal of Cardiovascular Echography, 23 (3), p. 81.
[7]	Maron, M., Finley, J., Bos, J., Hauser, T., Manning, W., Haas, T., Lesser, J., Udelson, J., Ackerman, M. and Maron, B., 2008. Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy. Circulation, 118 (15), pp. 1541-1549.
[8]	Sivanandam, A. and Ananthasubramaniam, K., 2016. Midventricular Hypertrophic Cardiomyopathy with Apical Aneurysm: Potential for Underdiagnosis and Value of Multimodality Imaging. Case Reports in Cardiology, 2016, pp. 1-5.
[9]	Cui, L., Tse, G., Zhao, Z., Bazoukis, G., Letsas, K., Korantzopoulos, P., Roever, L., Li, G. and Liu, T., 2019. Mid-ventricular obstructive hypertrophic cardiomyopathy with apical aneurysm: An important subtype of arrhythmogenic cardiomyopathy. Annals of Noninvasive Electrocardiology, 24 (5).
[10]	Po, J., Kim, B., Aslam, F., Arabadjian, M., Winson, G., Cantales, D., Kushner, J., Kornberg, R. and Sherrid, M., 2015. Doppler Systolic Signal Void in Hypertrophic Cardiomyopathy: Apical Aneurysm and Severe Obstruction without Elevated Intraventricular Velocities. Journal of the American Society of Echocardiography, 28 (12), pp. 1462-1473.
[11]	Tezuka, A., Higo, K., Nakamukae, Y., Nishihara, S., Kamikawa, M., Shimofuku, C., Kawazoe, K. and Ohishi, M., 2019. Bisoprolol Successfully Improved the Intraventricular Pressure Gradient in a Patient with Midventricular Obstructive Hypertrophic Cardiomyopathy with an Apex Aneurysm due to Apical Myocardial Damage. Internal Medicine, 58 (4), pp. 535-539.
[12]	Efthimiadis, G., Pliakos, C., Pagourelias, E., Parcharidou, D., Spanos, G., Paraskevaidis, S., Styliadis, I. and Parcharidis, G., 2009. Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm formation in a single family: case report. Cardiovascular Ultrasound, 7 (1).
[13]	Minami Y, Kajimoto K, Terajima Y, Yashiro B, Okayama D, Haruki S, Nakajima T, Kawashiro N, Kawana M, Hagiwara N. Clinical implications of midventricular obstruction in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2011 Jun 7; 57 (23): 2346-55. doi: 10.1016/j.jacc.2011.02.033. PMID: 21636036.
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APA Style

Divyesh Dadhania, Jineesh Valakada, Anoop Ayyappan, Ansan Joseph. (2021). Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant. Science Journal of Clinical Medicine, 10(4), 102-105. https://doi.org/10.11648/j.sjcm.20211004.14

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ACS Style

Divyesh Dadhania; Jineesh Valakada; Anoop Ayyappan; Ansan Joseph. Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant. Sci. J. Clin. Med. 2021, 10(4), 102-105. doi: 10.11648/j.sjcm.20211004.14

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AMA Style

Divyesh Dadhania, Jineesh Valakada, Anoop Ayyappan, Ansan Joseph. Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant. Sci J Clin Med. 2021;10(4):102-105. doi: 10.11648/j.sjcm.20211004.14

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@article{10.11648/j.sjcm.20211004.14,
  author = {Divyesh Dadhania and Jineesh Valakada and Anoop Ayyappan and Ansan Joseph},
  title = {Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant},
  journal = {Science Journal of Clinical Medicine},
  volume = {10},
  number = {4},
  pages = {102-105},
  doi = {10.11648/j.sjcm.20211004.14},
  url = {https://doi.org/10.11648/j.sjcm.20211004.14},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.sjcm.20211004.14},
  abstract = {Hypertrophic cardiomyopathy (HCM) is characterized by cardiac muscle hypertrophy, unexplained by loading conditions with a nondilated left ventricle and normal or increased ejection fraction Midventricular obstruction of the left ventricle is an increasingly recognized phenotypic variant of hypertrophic cardiomyopathy due to the widespread availability of Cardiac Magnetic Resonance Imaging (CMR). Mid ventricular obstruction with apical aneurysm is a high risk variant of HCM which can lead to higher risk of arrhythmia and SCD. This variant is clinically and prognostically significant with MRI playing a major role in detection. CMR has nearly 100 % sensitivity in diagnosis and also helps in identifying other high risk features. CMR is helpful in detecting Late gadolinium enhancement (LGE) and apical aneurysm for which echocardiography is less sensitive. LGE represent area of replacement fibrosis which can act as a source for life threatening arrhythmia. Although this variant is not considered high risk feature for sudden cardiac death (SCD) in HCM cohort according to standard guideline, it is increasingly being recognized as a high risk feature by recent studies. Treatment is controversial however patients with multiple risk factors of SCD can be treated with ICD implantation to prevent catastrophic events. Herein we present a case of HCM with midventricular obstruction where MRI showed apical aneurysm with scarring and midventricular gradient. Incidence, pathophysiology, diagnosis role of CMR and treatment of this variant of HCM is discussed.},
 year = {2021}
}

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TY  - JOUR
T1  - Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant
AU  - Divyesh Dadhania
AU  - Jineesh Valakada
AU  - Anoop Ayyappan
AU  - Ansan Joseph
Y1  - 2021/11/10
PY  - 2021
N1  - https://doi.org/10.11648/j.sjcm.20211004.14
DO  - 10.11648/j.sjcm.20211004.14
T2  - Science Journal of Clinical Medicine
JF  - Science Journal of Clinical Medicine
JO  - Science Journal of Clinical Medicine
SP  - 102
EP  - 105
PB  - Science Publishing Group
SN  - 2327-2732
UR  - https://doi.org/10.11648/j.sjcm.20211004.14
AB  - Hypertrophic cardiomyopathy (HCM) is characterized by cardiac muscle hypertrophy, unexplained by loading conditions with a nondilated left ventricle and normal or increased ejection fraction Midventricular obstruction of the left ventricle is an increasingly recognized phenotypic variant of hypertrophic cardiomyopathy due to the widespread availability of Cardiac Magnetic Resonance Imaging (CMR). Mid ventricular obstruction with apical aneurysm is a high risk variant of HCM which can lead to higher risk of arrhythmia and SCD. This variant is clinically and prognostically significant with MRI playing a major role in detection. CMR has nearly 100 % sensitivity in diagnosis and also helps in identifying other high risk features. CMR is helpful in detecting Late gadolinium enhancement (LGE) and apical aneurysm for which echocardiography is less sensitive. LGE represent area of replacement fibrosis which can act as a source for life threatening arrhythmia. Although this variant is not considered high risk feature for sudden cardiac death (SCD) in HCM cohort according to standard guideline, it is increasingly being recognized as a high risk feature by recent studies. Treatment is controversial however patients with multiple risk factors of SCD can be treated with ICD implantation to prevent catastrophic events. Herein we present a case of HCM with midventricular obstruction where MRI showed apical aneurysm with scarring and midventricular gradient. Incidence, pathophysiology, diagnosis role of CMR and treatment of this variant of HCM is discussed.
VL  - 10
IS  - 4
ER  -

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Author Information

Divyesh Dadhania

Imaging Sciences and Interventional Radiology, Sree Chithra Thirunal Institute of Medical Sciences and Technology (SCTIMST), Thiruvanathapuram, India
Jineesh Valakada

Imaging Sciences and Interventional Radiology, Sree Chithra Thirunal Institute of Medical Sciences and Technology (SCTIMST), Thiruvanathapuram, India
Anoop Ayyappan

Imaging Sciences and Interventional Radiology, Sree Chithra Thirunal Institute of Medical Sciences and Technology (SCTIMST), Thiruvanathapuram, India
Ansan Joseph

Imaging Sciences and Interventional Radiology, Sree Chithra Thirunal Institute of Medical Sciences and Technology (SCTIMST), Thiruvanathapuram, India

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APA Style

Divyesh Dadhania, Jineesh Valakada, Anoop Ayyappan, Ansan Joseph. (2021). Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant. Science Journal of Clinical Medicine, 10(4), 102-105. https://doi.org/10.11648/j.sjcm.20211004.14

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ACS Style

Divyesh Dadhania; Jineesh Valakada; Anoop Ayyappan; Ansan Joseph. Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant. Sci. J. Clin. Med. 2021, 10(4), 102-105. doi: 10.11648/j.sjcm.20211004.14

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AMA Style

Divyesh Dadhania, Jineesh Valakada, Anoop Ayyappan, Ansan Joseph. Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant. Sci J Clin Med. 2021;10(4):102-105. doi: 10.11648/j.sjcm.20211004.14

Copy | Download

@article{10.11648/j.sjcm.20211004.14,
  author = {Divyesh Dadhania and Jineesh Valakada and Anoop Ayyappan and Ansan Joseph},
  title = {Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant},
  journal = {Science Journal of Clinical Medicine},
  volume = {10},
  number = {4},
  pages = {102-105},
  doi = {10.11648/j.sjcm.20211004.14},
  url = {https://doi.org/10.11648/j.sjcm.20211004.14},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.sjcm.20211004.14},
  abstract = {Hypertrophic cardiomyopathy (HCM) is characterized by cardiac muscle hypertrophy, unexplained by loading conditions with a nondilated left ventricle and normal or increased ejection fraction Midventricular obstruction of the left ventricle is an increasingly recognized phenotypic variant of hypertrophic cardiomyopathy due to the widespread availability of Cardiac Magnetic Resonance Imaging (CMR). Mid ventricular obstruction with apical aneurysm is a high risk variant of HCM which can lead to higher risk of arrhythmia and SCD. This variant is clinically and prognostically significant with MRI playing a major role in detection. CMR has nearly 100 % sensitivity in diagnosis and also helps in identifying other high risk features. CMR is helpful in detecting Late gadolinium enhancement (LGE) and apical aneurysm for which echocardiography is less sensitive. LGE represent area of replacement fibrosis which can act as a source for life threatening arrhythmia. Although this variant is not considered high risk feature for sudden cardiac death (SCD) in HCM cohort according to standard guideline, it is increasingly being recognized as a high risk feature by recent studies. Treatment is controversial however patients with multiple risk factors of SCD can be treated with ICD implantation to prevent catastrophic events. Herein we present a case of HCM with midventricular obstruction where MRI showed apical aneurysm with scarring and midventricular gradient. Incidence, pathophysiology, diagnosis role of CMR and treatment of this variant of HCM is discussed.},
 year = {2021}
}

Copy | Download

TY  - JOUR
T1  - Role of MRI in Hypertrophic Cardiomyopathy with Midventricular Obstruction and Apical Aneurysm: Uncommon High-risk Variant
AU  - Divyesh Dadhania
AU  - Jineesh Valakada
AU  - Anoop Ayyappan
AU  - Ansan Joseph
Y1  - 2021/11/10
PY  - 2021
N1  - https://doi.org/10.11648/j.sjcm.20211004.14
DO  - 10.11648/j.sjcm.20211004.14
T2  - Science Journal of Clinical Medicine
JF  - Science Journal of Clinical Medicine
JO  - Science Journal of Clinical Medicine
SP  - 102
EP  - 105
PB  - Science Publishing Group
SN  - 2327-2732
UR  - https://doi.org/10.11648/j.sjcm.20211004.14
AB  - Hypertrophic cardiomyopathy (HCM) is characterized by cardiac muscle hypertrophy, unexplained by loading conditions with a nondilated left ventricle and normal or increased ejection fraction Midventricular obstruction of the left ventricle is an increasingly recognized phenotypic variant of hypertrophic cardiomyopathy due to the widespread availability of Cardiac Magnetic Resonance Imaging (CMR). Mid ventricular obstruction with apical aneurysm is a high risk variant of HCM which can lead to higher risk of arrhythmia and SCD. This variant is clinically and prognostically significant with MRI playing a major role in detection. CMR has nearly 100 % sensitivity in diagnosis and also helps in identifying other high risk features. CMR is helpful in detecting Late gadolinium enhancement (LGE) and apical aneurysm for which echocardiography is less sensitive. LGE represent area of replacement fibrosis which can act as a source for life threatening arrhythmia. Although this variant is not considered high risk feature for sudden cardiac death (SCD) in HCM cohort according to standard guideline, it is increasingly being recognized as a high risk feature by recent studies. Treatment is controversial however patients with multiple risk factors of SCD can be treated with ICD implantation to prevent catastrophic events. Herein we present a case of HCM with midventricular obstruction where MRI showed apical aneurysm with scarring and midventricular gradient. Incidence, pathophysiology, diagnosis role of CMR and treatment of this variant of HCM is discussed.
VL  - 10
IS  - 4
ER  -

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