md-medicaldata


Go to content

ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA - A CASE REPORT
ARITMOGENA DISPLAZIJA DESNE KOMORE - PRIKAZ SLUČAJA

Authors

 

Dušan Vapa1, Milan Popović2, Sonja Petrović, Slobodanka Vapa, Miljen Maletin1, Radosav Radosavkić1, Igor Veselinović1

1Katedra za sudsku medicinu, Medicinski fakultet, Univerzitet u Novom Sadu
2Katedra za histologiju i embriologiju, Medicinski Fakultet, Univerzitet u Novom Sadu

 


Rad primljen: 23.11.2017. /Rad prihvaćen: 13.12.2017.

 


Correspondence to


Doc. dr Dušan Vapa,
Katedra za sudsku medicinu, Medicinski fakultet, Univerzitet u Novom Sadu
Hajduk Veljkova 3, 21 000 Novi Sad
e-mail: dusan.vapa@mf.uns.ac.rs

 

 

Sažetak

 

Uvod: Aritmogena displazija desne komore predstavlja retko oboljenje miokarda u kojem srčano mišićno tkivo biva zamenjeno masnim i vezivnim tkivom. Oboljenje se klinički manifestuje pojavom životno ugrožavajućih ventrikularnih aritmija. Predstavlja vodeći uzrok iznenadne smrti kod mladih sportista.
Prikaz slučaja: U prvom prikazanom slučaju, radi se o osobi muškog pola, starosti 31 godinu, jače razvijene mišićne građe, pronađenoj pored reke, u stanju mrtvačke ukočenosti. Na telu nema vidljivih znakova povređivanja. Obdukcijom je utvrđeno da deo zida desne srčane komore gubi svoje mišićno tkivo i zamenjuje se masnim tkivom. Patohistološkim pregledom uzoraka srčanog mišića uočavaju se kardiomiociti, međusobno razdvojeni ostrvima masnog tkiva, što odgovara promenama kod aritmogene displazije desne komore. U drugom prikazanom slučaju, radi se o mlađoj ženskoj osobi, starosti 14 godina, koju su spasioci izvukli iz bazena. Obdukcijom tela utvrđen je gotovo identičan nalaz na srcu kao kod prethodnog slučaja. Međutim, utvrđeni su i znaci koji ukazuju da je ova smrt nastala usled mehaničke asfiksije nastale u sklopu utopljenja. Makroskopski i patohistološki nalazi na srcu, odgovaraju aritmogenoj displaziji desne komore.
Zaključak: Aritmogena displazija desne komore je oboljenje koje odnosi, pre svega, osobe mlađeg uzrasta. Upravo zbog toga, neophodno je imati u vidu mogućnosti dijagnostike ovog oboljenja i sprovoditi ih među decom školskog uzrasta, pogotovo onom koja se aktivno bave sportom.

 

 

 

Ključne reči:

aritmogena displazija desne komore, kardiomiopatija, iznenadna srčana smrt, obdukcija

 

 

Abstract

 

Introduction: Arrhythmogenic right ventricular dysplasia (ARVD) is a rare form of cardiomyopathy in which the heart muscle of the right ventricle is replaced by fat and fibrous tissue. These patients often have arrhythmias which can increase the risk of sudden cardiac arrest or death. It is a leading cause of sudden death among young athletes.
Case report: During external examination, it was noted that the deceased was a 31 year old male, found on the banks of the river. He was well nourished, with a greater developed muscular build, in a state of rigor mortis. No signs of mechanical trauma were found. Internal examination revealed that a part of right ventricle muscle was replaced with fat tissue. Microscopic findings were in concordance with macroscopic findings of ARVD, revealing fat changes of heart muscle tissue.The second case involved a young girl at the age of 14. She was pulled out from a swimming pool by the lifeguards. During autopsy, internal examination revealed almost identical findings on the heart muscle as in the first case. But in this case, signs of drowning were also revealed, so it was determined that the mechanism of death was mechanical asphyxia due to drowning. Microscopic findings of the heart muscle were in concordance with macroscopic findings of ARVD.
Conclusion: ARVD is a disease that primarily affects younger people. It is necessary to have in mind the possibilities for diagnosis of this disease and implement them among children of school age, especially those who are actively engaged in sports.

 

 

Key words:

Arrhythmogenic right ventricular dysplasia, ARVD, cardiomyopathy, autopsy

 

 

References

 

  1. Norman MW, McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy/ dysplasia: perspectives on diseases. Z Kardiol. 1999; 88:550-4.
  2. Marcus F, Fontaine G, Guirdaudon G, Frank R, Laurenceau JL, Malergue C, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982; 65:384-98.
  3. Kies P, Bootsma M, Bax J, Schalij MJ, van der Wall EE. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: screening, diagnosis and treatment. J Hrt Rhm. 2006; 3:225–234.
  4. Dalal D, Nasir K, Bomma C, Prakasa K, Tandri H, Piccini J, et al. Arrhythmogenic right ventricular dysplasia. A United States Experience. Circulation.2005; 112:3823–3832.
  5. Pelliccia A, Corrado D, Bjørnstad HH, Panhuyzen-Goedkoop N, Urhausen A, Carre F, et al. Recommendations for participation in competitive sport and leisure-time physical activity in individuals with cardiomyopathies, myocarditis and pericarditis. Eur J Cardiovasc Prev Rehabil. 2006; 13(6):876-85.
  6. Chan EL, Shannon KM, Klitzner TS. A pediatric case report on arrhythmogenic right ventricular dysplasia. Congenit Heart Dis.2008; 3:132–137.
  7. Richardson P, McKenna, Bristow M, Maisch B, Mautner B, O’Connel J, et al. Report of the 1995 WHO/ISFC Task Force on the definition and classification of cardiomyopathies. Circulation. 1996; 93:841-2.
  8. Sen-Chowdhry S, Syrris P, McKenna WJ. Genetics of right ventricular cardiomyopathy. J Cardiovasc Electrophysiol. 2005; 16:927-35.
  9. Butcovan D, Amalinei C, Grigoriu C. Arrhythmogenic right ventricular cardiomyopathy – cause of sudden death in young people. Rom J Leg Med. 2011; 19:189-194.
  10. Patil AM, Anchinmane VT, Kavishwar VS. Sudden death due to arrhythmogenic right ventricular dysplasia: A medico-legal case report. J Indian Acad Forensic Med. 2012; 34(2):185-187.
  11. Vadysinghe AN, Jayasooriya RP, Gunatilake GK, Sivasubramanium M. Unexpected sudden death in pregnancy – arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report. Forensic Sciences Research. 2017; 2(3);161–163.
  12. Milovanović M. Sudska medicina. Beograd: Medicinska knjiga Beograd-Zagreb; 1960.
  13. Tasić M. Sudska medicina. Novi Sad: Zmaj; 2006.
  14. Popović V, Atanasijević T. Sudska medicina. Beograd: Libri medicorum; 2009.
  15. Popović D, Brkić P, Nešić D, Stojiljković S, Šćepanović Lj, Ostojić M. Elektrofiziološke karakteristike sportskog srca. Med Pregl. 2007; 60(3-4):156-9.
  16. Gimeno JR, Lacunza J, García-Alberola A, Cerdán MC, Oliva MJ, García-Molina E, et al. Penetrance and risk profile in inherited cardiac diseases studied in a dedicated screening clinic. Am J Cardiol. 2009; 104(3):406-10.
  17. Vermes E, Strohm O, Otmani A, Childs H, Duff H, Friedrich MG. Impact of the revision of arrhythmogenic right ventricular cardiomyopathy/dysplasia task force criteria on its prevalence by CMR criteria. JACC Cardiovasc Imaging. 2011; 4(3):282-7.

UDK: 616.12-007



PDF Vapa D. et al. • MD-Medical Data 2017;9(4): 249-252

 

 

 

Naslovna | Revija | Galerija | Dešavanja | Instrukcije | Redakcija | Izdavač | Prijatelji sajta | Saradnja | Kontakt | Site Map


Back to content | Back to main menu