Fibromuscular dysplasia of cardiac conduction system arteries in traumatic and nonnatural sudden death victims aged 0 to 40 years: a histological analysis of 100 cases

2014 ◽  
Vol 23 (1) ◽  
pp. 12-16 ◽  
Author(s):  
Fred Zack ◽  
Grit Kutter ◽  
Verena Blaas ◽  
Ann-Katrin Rodewald ◽  
Andreas Büttner
Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1323
Author(s):  
Giulia Ottaviani ◽  
Graziella Alfonsi ◽  
Simone G. Ramos ◽  
L. Maximilian Buja

A retrospective study was conducted on pathologically diagnosed arrhythmogenic cardiomyopathy (ACM) from consecutive cases over the past 34 years (n = 1109). The anatomo-pathological analyses were performed on 23 hearts diagnosed as ACM (2.07%) from a series of 1109 suspected cases, while histopathological data of cardiac conduction system (CCS) were available for 15 out of 23 cases. The CCS was removed in two blocks, containing the following structures: Sino-atrial node (SAN), atrio-ventricular junction (AVJ) including the atrio-ventricular node (AVN), the His bundle (HB), the bifurcation (BIF), the left bundle branch (LBB) and the right bundle branch (RBB). The ACM cases consisted of 20 (86.96%) sudden unexpected cardiac death (SUCD) and 3 (13.04%) native explanted hearts; 16 (69.56%) were males and 7 (30.44%) were females, ranging in age from 5 to 65 (mean age ± SD, 36.13 ± 16.06) years. The following anomalies of the CCS, displayed as percentages of the 15 ACM SUCD cases in which the CCS has been fully analyzed, have been detected: Hypoplasia of SAN (80%) and/or AVJ (86.67%) due to fatty-fibrous involvement, AVJ dispersion and/or septation (46.67%), central fibrous body (CFB) hypoplasia (33.33%), fibromuscular dysplasia of SAN (20%) and/or AVN (26.67%) arteries, hemorrhage and infarct-like lesions of CCS (13.33%), islands of conduction tissue in CFB (13.33%), Mahaim fibers (13.33%), LBB block by fibrosis (13.33%), AVN tongue (13.33%), HB duplicity (6.67%%), CFB cartilaginous meta-hyperplasia (6.67%), and right sided HB (6.67%). Arrhythmias are the hallmark of ACM, not only from the fatty-fibrous disruption of the ventricular myocardium that accounts for reentrant ventricular tachycardia, but also from the fatty-fibrous involvement of CCS itself. Future research should focus on application of these knowledge on CCS anomalies to be added to diagnostic criteria or at least to be useful to detect the patients with higher sudden death risks.


2003 ◽  
Vol 12 (2) ◽  
pp. 77-81 ◽  
Author(s):  
Giulia Ottaviani ◽  
Luigi Matturri ◽  
Lino Rossi ◽  
Dan Jones

1988 ◽  
Vol 19 (1) ◽  
pp. 116-119 ◽  
Author(s):  
David A. Schwartz ◽  
Sydney D. Finkelstein ◽  
George D. Lumb

2015 ◽  
Vol 5 (9) ◽  
pp. 709-716
Author(s):  
LM Ilias ◽  
MR Pai ◽  
S Shukla ◽  
A Ponniah ◽  
K Gautam ◽  
...  

Background: Atherosclerotic coronary artery disease is the modern pandemic and is said to be responsible for a major chunk of sudden cardiac deaths. However, in few cases; especially among the young, conduction system abnormalities were found to be the cause of death with absence of significant coronary lesions. These days, examination of the cardiac conduction system during autopsy has been abandoned as it is said to be a time consuming process. The present study was undertaken to highlight the importance of examining the cardiac conduction system in all sudden death cases.Materials and Methods: This study was a prospective one, conducted from June 2007 to June 2009 in the department of Pathology, Kasturba Medical College, Mangalore. Forty six hearts of persons who died suddenly were studied.Results: Our study showed that even though atherosclerotic coronary lesions were the most common cause of sudden death, cardiac conduction defects also contributed to a significant number of cases; especially in the young. In our study of 46 cases; 6 cases showed only conduction system abnormalities. These were young adults who had died suddenly. The predominant lesion identified in them was fibromuscular dysplasia of the nodal arteries.Conclusion: A detailed examination of the coronaries and the cardiac conduction system should be made mandatory in all sudden death cases, especially in the young as they will help to throw some light into the cause of death.Journal of Pathology of Nepal (2015) Vol. 5, 709-716


2013 ◽  
Vol 7 (1) ◽  
pp. 47-49 ◽  
Author(s):  
Donatella Mecchia ◽  
Anna Maria Lavezzi ◽  
Luigi Matturri

A 4-month-old female infant considered to be in good health died suddenly and unexpectedly. Post- mortem examination was requested, with clinical diagnosis of sudden infant death syndrome. At autopsy the infant was described in good health. Histo- logical examination of the heart found a cardiac fibroma compressing the atrio-ventricular node and the examination of the cardiac conduction system showed an accessory fiber of Mahaim (nodo-ventricular) and cartilaginous metaplasia of the cardiac fibrous body. Probably the concomitant presence of cardiac conduction system abnormalities and a septal fibroma, compressing the atrio-ventricular node, could have an important role in causing the sudden death.


1962 ◽  
Vol 44 (5) ◽  
pp. 687-698 ◽  
Author(s):  
Nalda Thung ◽  
J. Francis Dammann ◽  
Rodrigo Diaz-Perez ◽  
Webb M. Thompson ◽  
Miguel Sanmarco ◽  
...  

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