Asymmetrical Septal Hypertrophy diagnosed by MRI: a case report

Authors

  • Mauricio Muleiro Álvarez Universidad Anáhuac México Norte, Centro de Investigación en Ciencias de la Salud (CICSA), Facultad de Ciencias de la Salud, Estado de México, México.
  • Felipe Esparza Salazar Universidad Anáhuac México Norte, Centro de Investigación en Ciencias de la Salud (CICSA), Facultad de Ciencias de la Salud https://orcid.org/0000-0003-1884-5389
  • Ángel David Alvarado Torres Center of Excellence for Aging and Brain Repair, Morsani College of Medicine, University of South Florida, Tampa, FL, USA. https://orcid.org/0000-0001-7514-8083
  • María Fernanda Osorio Martínez Universidad Autónoma Metropolitana, Unidad Xochimilco, División de Ciencias Biológicas y de la Salud, Ciudad de México, México.

DOI:

https://doi.org/10.36105/psrua.2024v4n7.05

Keywords:

asymmetric septal hypertrophy, sarcomere, sudden death, syncope

Abstract

Asymmetrical septal hypertrophy (ASH) is defined as an increase in ventricular wall thickness greater than 15 mm that is not associated with any other pathology. It is a condition that, in most cases, is caused by a mutation in one of the genes associated with the proteins that form the sarcomere. In this article, we present a case of ASH in a 43-year-old adult. After manifesting tachypnea, dyspnea, and cutaneous pallor, followed by a syncopal episode, the individual seeks medical attention. During the medical evaluation, an electrocardiogram (ECG) is performed, revealing bradycardia at 48 beats per minute and an inverted T wave in leads DI, AVL, V3, V4, V5, and V6. The diagnosis is confirmed through cardiac magnetic resonance imaging, which shows hypertrophic cardiomyopathy with non-obstructive ASH of 27.22 mm. Consequently, it is decided to initiate pharmacological treatment with propranolol, and the patient is still awaiting a surgical timeframe for the placement of an implantable cardioverter-defibrillator (ICD).

Downloads

Download data is not yet available.

PLUMX metrics

References

Schaufelberger M. Cardiomyopathy and pregnancy. Heart. 2019;105(20):1543-1551. https://pubmed.ncbi.nlm.nih.gov/31308064/ DOI: https://doi.org/10.1136/heartjnl-2018-313476

Gowda SN, Ali HJ, Hussain I. Overview of Restrictive Cardiomyopathies. Methodist Debakey Cardiovasc J. 2022;18(2):4-16. https://pubmed.ncbi.nlm.nih.gov/35414858/ DOI: https://doi.org/10.14797/mdcvj.1078

Sebastian SA, Panthangi V, Singh K, Rayaroth S, Gupta A, Shantharam D, Rasool BQ, Padda I, Co EL, Johal G. Hypertrophic Cardiomyopathy: Current Treatment and Future Options. Curr Probl Cardiol. 2023;48(4):101552. https://doi.org/10.1016/j.cpcardiol.2022.101552 DOI: https://doi.org/10.1016/j.cpcardiol.2022.101552

Biddinger KJ, Jurgens SJ, Maamari D, Gaziano L, Choi SH, Morrill VN, Halford JL, Khera AV, Lubitz SA, Ellinor PT, Aragam KG. Rare and Common Genetic Variation Underlying the Risk of Hypertrophic Cardiomyopathy in a National Biobank. JAMA Cardiol. 2022;7(7):715-722. https://doi.org/10.1001/jamacardio.2022.1061 DOI: https://doi.org/10.1001/jamacardio.2022.1061

Ottaviani A, Mansour D, Molinari LV, Galanti K, Mantini C, Khanji MY, Chahal AA, Zimarino M, Renda G, Sciarra L, Pelliccia F, Gallina S, Ricci F. Revisiting Diagnosis and Treatment of Hypertrophic Cardiomyopathy: Current Practice and Novel Perspectives. J Clin Med. 2023;12(17):5710. https://doi.org/10.3390/jcm12175710 DOI: https://doi.org/10.3390/jcm12175710

Litt MJ, Ali A, Reza N. Familial Hypertrophic Cardiomyopathy: Diagnosis and Management. Vasc Health Risk Manag. 2023;19:211-221. https://doi.org/10.2147/VHRM.S365001 DOI: https://doi.org/10.2147/VHRM.S365001

Kochi AN, Vettor G, Dessanai MA, Pizzamiglio F, Tondo C. Sudden cardiac death in athletes: From the basics to the practical work-up. Medicina. 2021;57(2):168. https://doi.org/10.3390/medicina57020168 DOI: https://doi.org/10.3390/medicina57020168

Márquez MF, Ruíz-Siller TJ, Méndez-Ramos R, Karabut E, Aranda-Fraustro A, Jiménez-Becerra S. Miocardiopatía hipertrófica (MCH). Una revisión histórica y anatomopatológica. Gac Med Mex. 2016;152(5):697-702. Available from: https://www.medigraphic.com/cgi-bin/new/resumen.cgi?IDARTICULO=68916

Veselka J, Anavekar NS, Charron P. Hypertrophic obstructive cardiomyopathy. Lancet. 2017;389(10075):1253-1267. https://doi.org/10.1016/S0140-6736(16)31321-6 DOI: https://doi.org/10.1016/S0140-6736(16)31321-6

Bayonas-Ruiz A, Muñoz-Franco FM, Sabater-Molina M, Oliva-Sandoval MJ, Gimeno JR, Bonacasa B. Current therapies for hypertrophic cardiomyopathy: a systematic review and meta-analysis of the literature. ESC Heart Fail. 2023;10(1):8-23. https://doi.org/10.1002/ehf2.14142 DOI: https://doi.org/10.1002/ehf2.14142

Ozdemir S, Tan YZ, Gazi E. Is the Increased Septal Perfusion the Signal of Asymmetrical Septal Hypertrophy? World J Nucl Med. 2016;15(3):184-9. https://doi.org/10.4103/1450-1147.174706 DOI: https://doi.org/10.4103/1450-1147.174706

Kuznetsov VA, Yaroslavskaya EI, Zyrianov IP, Kolunin GV, Krinochkin DV, Bessonova MI, Bessonov IS. Asymmetric septal hypertrophy in patients with coronary artery disease. Eur J Echocardiogr. 2010;11(8):698-702. https://doi.org/10.1093/ejechocard/jeq046 DOI: https://doi.org/10.1093/ejechocard/jeq046

Tuseth N, Cramariuc D, Rieck AE, Wachtell K, Gerdts E. Asymmetric septal hypertrophy - a marker of hypertension in aortic stenosis (a SEAS substudy). Blood Press. 2010;19(3):140-4. https://doi.org/10.3109/08037051.2010.481816 DOI: https://doi.org/10.3109/08037051.2010.481816

Melas M, Beltsios ET, Adamou A, Koumarelas K, McBride KL. Molecular Diagnosis of Hypertrophic Cardiomyopathy (HCM): In the Heart of Cardiac Disease. J Clin Med. 2022;12(1):225. https://doi.org/10.3390/jcm12010225 DOI: https://doi.org/10.3390/jcm12010225

Weissler-Snir A, Crean A, Rakowski H. The role of imaging in the diagnosis and management of hypertrophic cardiomyopathy. Expert Rev Cardiovasc Ther. 2016;14(1):51-74. https://doi.org/10.1586/14779072.2016.1113130 DOI: https://doi.org/10.1586/14779072.2016.1113130

Tschöpe C, Cooper LT, Torre-Amione G, Van Linthout S. Management of Myocarditis-Related Cardiomyopathy in Adults. Circ Res. 2019;124(11):1568-1583. https://doi.org/10.1161/CIRCRESAHA.118.313578 DOI: https://doi.org/10.1161/CIRCRESAHA.118.313578

Gragnano F, Pelliccia F, Guarnaccia N, Niccoli G, De Rosa S, Piccolo R, Moscarella E, Fabris E, Montone RA, Cesaro A, Porto I, Indolfi C, Sinagra G, Perrone Filardi P, Andò G, Calabrò P; Working Group of Interventional Cardiology of the Italian Society of Cardiology. Alcohol Septal Ablation in Patients with Hypertrophic Obstructive Cardiomyopathy: A Contemporary Perspective. J Clin Med. 2023;12(8):2810. https://doi.org/10.3390/jcm12082810 DOI: https://doi.org/10.3390/jcm12082810

Grillo MP, Erve JCL, Dick R, Driscoll JP, Haste N, Markova S, Brun P, Carlson TJ, Evanchik M. In vitro and in vivo pharmacokinetic characterization of mavacamten, a first-in-class small molecule allosteric modulator of beta cardiac myosin. Xenobiotica. 2019;49(6):718–733. https://doi.org/10.1080/00498254.2018.1495856 DOI: https://doi.org/10.1080/00498254.2018.1495856

Dong T, Alencherry B, Ospina S, Desai MY. Review of Mavacamten for Obstructive Hypertrophic Cardiomyopathy and Future Directions. Drug design, development and therapy. 2023;17, 1097–1106. https://doi.org/10.2147/DDDT.S368590 DOI: https://doi.org/10.2147/DDDT.S368590

Keam SJ. Mavacamten: First Approval. Drugs, 2022;82(10), 1127–1135. https://doi.org/10.1007/s40265-022-01739-7 DOI: https://doi.org/10.1007/s40265-022-01739-7

Heitner SB, Jacoby D, Lester, SJ, Owens A, Wang A, Zhang D, Lambing J, Lee J, Semigran M, Sehnert AJ. Mavacamten Treatment for Obstructive Hypertrophic Cardiomyopathy: A Clinical Trial. Annals of internal medicine, 2019;170(11), 741–748. https://doi.org/10.7326/M18-3016 DOI: https://doi.org/10.7326/M18-3016

Olivotto I, Oreziak A, Barriales-Villa R, Abraham TP, Masri A, Garcia-Pavia P, Saberi S, Lakdawala NK, Wheeler MT, Owens A, Kubanek M, Wojakowski W, Jensen MK, Gimeno-Blanes J, Afshar K, Myers J, Hegde S M, Solomon SD, Sehnert AJ, Zhang D, Li W, Bhattacharya M, Edelberg JM,Burstein-Waldman C, Lester SJ, Wang A, Ho CY, Jacoby D, EXPLORER-HCM study investigators. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet, 2020;396(10253):759–769. https://doi.org/10.1016/S0140-6736(20)31792-X DOI: https://doi.org/10.1016/S0140-6736(20)31792-X

Desai MY, Owens A, Geske JB, Wolski K, Naidu SS, Smedira NG, Cremer PC, Schaff H, McErlean E, Sewell C, Li W, Sterling L, Lampl K, Edelberg JM, Sehnert AJ, Nissen SE. Myosin inhibition in patients with obstructive hypertrophic cardiomyopathy referred for septal reduction therapy. J Am Coll Cardiol. 2022;80(2):95–108. https://doi.org/10.1016/j.jacc.2022.04.048 DOI: https://doi.org/10.1016/j.jacc.2022.04.048

Downloads

Published

2024-06-06

How to Cite

Muleiro Álvarez, M. ., Esparza Salazar, F. ., Alvarado Torres, Ángel D. ., & Osorio Martínez, M. F. . (2024). Asymmetrical Septal Hypertrophy diagnosed by MRI: a case report. Proceedings of Scientific Research Universidad Anáhuac. Multidisciplinary Journal of Healthcare, 4(7), 43–49. https://doi.org/10.36105/psrua.2024v4n7.05