Primary Cardiac Fibroma and Cardiac Conduction System Alterations in a Case of Sudden Death of a 4-month-old Infant

Mecchia, Donatella; Lavezzi, Anna Maria; Matturri, Luigi

doi:10.2174/1874192401307010047

Cited by 5 publications

(4 citation statements)

References 14 publications

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“…Cases reported in the literature shed light on the relationship between LVOT and HLHS. An infantile cardiac fibroma causing compression of the AV node and sudden cardiac death was documented in the literature; however, it was unrelated to HLHS 20…”

Section: Discussionmentioning

confidence: 99%

Cardiac fibroma presenting as hypoplastic left heart syndrome in a foetus: causal or coincidental?

Sundari,

Gurusamy

2024

BMJ Case Rep

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Primary cardiac tumours are very rare. Cardiac tumours in the perinatal period are even more uncommon with a prevalence of 0.0017% to 0.28% in autopsy series. The majority of benign cardiac tumours are cardiac rhabdomyomas, followed by cardiac fibromas. Another rare congenital heart disease is hypoplastic left heart syndrome (HLHS). Here we present a 21-week-old foetus diagnosed antenatally with HLHS on foetal echocardiogram. An autopsy done on the foetus following medical termination of pregnancy revealed a cardiac fibroma in the ventricular septum. It is very uncommon to have a combination of two congenital heart diseases. An extensive literature review revealed only three cases that had rhabdomyoma and associated HLHS. This case presented with cardiac fibroma which in early gestation would have resulted in left ventricular outflow obstruction leading to the development of HLHS. Small cardiac tumours which are difficult to detect by echocardiogram in early gestation can lead to the development of HLHS. A thorough and hierarchical autopsy examination of such cases can help in a better understanding of the relationship between HLHS and cardiac tumours.

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Section: Discussionmentioning

confidence: 99%

Cardiac fibroma presenting as hypoplastic left heart syndrome in a foetus: causal or coincidental?

Sundari,

Gurusamy

2024

BMJ Case Rep

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“…Microscopically, the amount of collagen increases with age, whereas the degree of cellularity decreases with age [ 1 , 8 ]. Fibromas occurring in the interventricular septum can lead to compression of the atrioventricular node, cartilaginous metaplasia of the central fibrous body [ 5 ], and infiltration of the His bundle [ 8 , 15 ]. In this case report, a large fibroma of the right ventricle caused mechanical overload and increased stress on the central fibrous body presenting as focal osseous metaplasia.…”

Section: Discussionmentioning

confidence: 99%

“…Cardiac fibroma is a benign tumor of the heart that occurs primarily in infants and children and may be congenital [ 1 ]. Presenting symptoms include heart failure, pericardial effusion, syncope, cyanosis, chest pain [ 1 , 2 ], ventricular tachycardia [ 3 , 4 ], arrhythmias, sudden death [ 1 , 5 ], and heart murmurs [ 2 ]. This article reports the autopsy findings of a 1.5-year-old girl who presented to the hospital with ventricular tachycardia and arrhythmia after she suddenly collapsed at home.…”

Section: Introductionmentioning

confidence: 99%

Ventricular fibrillation and arrhythmia associated with cardiac fibromas involving both ventricles in a 1.5-year-old girl

et al. 2023

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This article reports the autopsy findings of a 1.5-year-old girl with no history of previous hospital admission who suddenly collapsed at home. After 45 minutes of resuscitation efforts, the cardiac activity was restored. During hospitalization, she had ventricular arrhythmia and extremely elevated cardiac troponin levels. Internal examination and immunohistochemistry revealed cardiac fibromas of the right and left ventricles and extensive hypoxic myocardial damage. The right ventricular fibroma demonstrated interdigitating and entrapped myocardium visible at the edges and within the central portion of the tumor. The left ventricular fibroma originated in the subepicardial region and propagated towards the endocardium.

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“…Further clinical manifestations include syncope, chest pain, and heart failure symptoms, but rarely it can present with ventricular arrhythmia and sudden death [ 3 , 4 , 19 ] . They are also associated with Gorlin syndrome, equally known as nevoid basal cell carcinoma, an autosomal dominant genetic condition due to mutations in the PCTH1 gene, which predisposes individuals to a wide range of developmental problems and a high likelihood for developing malignancies [ 20 ] .…”

Section: Tumour Typesmentioning

confidence: 99%

The Current Management of Cardiac Tumours: a Comprehensive Literature Review

Joshi¹,

Kumar²,

Noshirwani³

et al. 2020

Braz J Cardiovasc Surg

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Objective To understand the current evidence and guidelines behind the appropriate management of cardiac tumours. Methods A comprehensive electronic literature search has been performed in major databases - PubMed, Embase, Scopus, Ovid, and Google Scholar. All articles that discussed all different forms of cardiac tumours, their clinical presentation, diagnosis, and management methods have been critically appraised in this narrative review. Results All relevant studies have been summarized in appropriate sections within our review. Cardiac tumours are rare but can be catastrophic and life-threatening if not identified and managed on timely manner. Utilization of all the available imaging methods can be of equivocal importance, relevant to each cardiac tumour. Surgical excision is the ultimate treatment method, however histopathological results can guide the adjunct treatment. Conclusion Early detection of cardiac tumours has significant effect on planning the method of intervention. Technological advancements and increased availability of imaging modalities have enabled earlier and more accurate detection of these tumours. Novel medical therapies, recommendations for screening, and operative techniques have all contributed to overall improving knowledge of these tumours and ultimately patient outcomes.

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Primary Cardiac Fibroma and Cardiac Conduction System Alterations in a Case of Sudden Death of a 4-month-old Infant

Cited by 5 publications

References 14 publications

Cardiac fibroma presenting as hypoplastic left heart syndrome in a foetus: causal or coincidental?

Cardiac fibroma presenting as hypoplastic left heart syndrome in a foetus: causal or coincidental?

Ventricular fibrillation and arrhythmia associated with cardiac fibromas involving both ventricles in a 1.5-year-old girl

The Current Management of Cardiac Tumours: a Comprehensive Literature Review

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