Abstract
Intramyocardial cartilage has never been reported in the human heart before. In the literature, the only reported localizations of cartilage in the heart were in the central fibrous body and the valves. We report a case of an unusual presence of cartilage tissue within the myocardial wall of the left ventricle in a 10-year-old boy who died unexpectedly. This case presents an interesting, unusual and apparently asymptomatic sudden cardiac death related to a cartilaginous myocardial tumor. Conducting system disturbance secondary to the myocardial tumor is the probable cause of death. This case is relevant not only for its singularity and originality, but also for the diverse and controversial hypotheses related to the onset of cartilaginous tissue in the myocardial wall. Early detection of this tumor by modern thoracic imaging may have prevented a fatal unexpected outcome.
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Durán AC, López D, Guerrero A, Mendoza A, Arqué JM, Sans-Coma V. Formation of cartilaginous foci in the central fibrous body of the heart in Syrian hamsters (Mesocricetus auratus). J Anat. 2004;205:219–27.
Ferris JAJ, Aherne WA. Cartilage in relation to the conducting tissue of the heart in sudden death. Lancet. 1971;297:64–6.
Seemayer TA, Thelmo WL, Morin J. Cartilaginous transformation of the aortic valve. Am J Clin Pathol. 1973;60:616–20.
Ho SY, Anderson RH. Conduction tissue and SIDS. Ann N Y Acad Sci. 1988;533:176–90.
James TN, Marshall TK. Persistent fetal dispersion of the atrioventricular node and his bundle within the central fibrous body. Circulation. 2001;50:127–47.
Arbustini E, Jones M, Ferrans VJ. Formation of cartilage in bioprosthetic cardiac valves implanted in sheep: A morphologic study. Am J Cardiol. 1983;52:632–6.
Groom DA, Starke W. Cartilaginous metaplasia in calcific aortic valve disease. Am J Clin Pathol. 1990;93:809–12.
Matturri L, Ottaviani G, Ramos SG, Rossi L. Sudden infant death syndrome (SIDS): A study of cardiac conduction system. Cardiovasc Pathol. 2000;9:137–45.
Ottaviani G, Matturri L. Histopathology of the cardiac conduction system in sudden intrauterine unexplained death. Cardiovasc Pathol. 2008;17:146–55.
Scapinelli R, Little K. Observations on the mechanically induced differentiation of cartilage from fibrous connective tissue. J Pathol. 1970;101:85–91.
Cummings PM, Trelka DP, Springer KM. Atlas of forensic histopathology. New York: Cambrige University Press; 2010.
Oueida FM, Lui RC, Al-Refae MA, Al-Omran HM. Left ventricular hemangioma. Asian Cardiovasc Thorac Ann. 2014;22:77–9.
Kitchin PL, Benson P. Left ventricular myxoma causing sudden death. Am J Forensic Med Pathol. 2015;36:58–60.
Archontakis S, Koumallos N, Demosthenous M, Latsios G, Sideris S. Epicardial right ventricular lipoma presenting with sustained ventricular tachycardia. J Cardiac Surg. 2018;33:438–9.
Edelman JJB, Teoh J, Okiwelu NLE, Hung J, Passage J. Resection of left ventricular rhabdomyosarcoma. J Am Coll Cardiol. 2013;62:2013.
Mendes GS, Abecasis J, Gouveia R, Castro M, Carvalho S, Ramos S, et al. A cardiac mass beyond symptomatic palpitations. Cardiovasc Pathol. 2019;39:1–4.
Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996;77:107.
Pompa AG, LaRossa P, Beerman LB, Sogawa Y, Rosen J, Arora G. Utility of ECGs in the pediatric emergency department for patients presenting with a seizure. Am J Emerg Med. 2020;38:1362–6.
Strano S, Toni D, Ammirati F, Sanna T, Tomaino M, Brignole M, et al. Neuro-arrhythmology: A challenging field of action and research: A review from the Task Force of Neuro-arrhythmology of Italian Association of Arrhythmias and Cardiac Pacing. J Cardiovasc Med. 2019;20:731–44.
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Makni, C., Manoubi, S.A., Bekir, O. et al. Unexpected fatal intramyocardial cartilaginous tumor: pathophysiology, mechanism of death and review of the literature. Forensic Sci Med Pathol 17, 308–311 (2021). https://doi.org/10.1007/s12024-020-00351-z
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DOI: https://doi.org/10.1007/s12024-020-00351-z