Case Report
BibTex RIS Cite

APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU

Year 2018, Volume: 19 Issue: 1, 34 - 37, 15.01.2018
https://doi.org/10.18229/kocatepetip.411211

Abstract

Aritmojenik sağ ventrikül displazisi (ARVD) na-dir görülen bir kardiyomiyopatidir. ARVD ço-ğunlukla genç yaşlarda tanı alır ve kendisini ventriküler aritmiler, çarpıntı, baş dönmesi, kalp yetmezliği ve hatta ani kardiyak ölüm ile gösterebilir. Görüntüleme yöntemleri ile sağ ventrikül (SV) dilatasyonu ve apikal anevrizma tipik bulgusudur. Fakat ARVD olgularında int-raventriküler trombüs çok nadir görülmektedir. 19 yaşında erkek hasta, hastanemize çarpıntı ve bayılma şikâyetleri ile başvurdu. Elektrokar-diyografisinde ön yüz derivasyonlarda T nega-tifliği bulunmakta idi. Ekokardiyografide sağ ventrikül dilate ve SV apeksinde anevrizmatik oluşum içinde trombüs görüldü (Fig-1). Kardi-yak manyetik rezonans incelemede sağ vent-rikül genişlemesini, yağ infiltrasyonunu, fibro-tik dokuları, SV duvar hareket bozukluğunu ve trombüslü apikal anevrizma doğrulandı. Anti-koagulan tedaviyle üç ay sonra trombüsün rezole olduğu gözlendi ve ICD implante edildi. ARVD tanısında elektrokardiyografik, aritmik, histolojik ve ailesel özelliklerin yanında görün-tüleme yöntemleri de büyük önem taşımak-tadır. Sağ ventrikül dilatasyonu ve apikal anev-rizması tanı sürecinde önemli kriterler olmakla birlikte bu gibi bulgular saptandığında trom-büs varlığı da dikkatlice değerlendirilmelid

References

  • Bacior B., Kubinyi A., Grodecki.Arrhythmogenicright ventricular dysplasia—ARVD Case study. Kardiol Pol2002;57(10):337-9.
  • McKenna W.J., Thiene G., Nava A.et al .Diagnosis ofarrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 1994;71(3):215-8. F.I.Marcus, A. Nava, G. Thiene (Editor.)Zareba, W.,Piotrowicz,K., Turrini, P. Electrocardiographic manifestations. in: Arrhythmogenic RV Cardiomyopathy/Dysplasia. Springer, Berlin; 2007:121–128.
  • Durrer D, Van Dam THR, Freud GE, Janse MJ, Meijler FL,Arzbaecher RC. Total excitation of the isolated human heart. Circulation ;1970;41(6):899-912.
  • Fontaine G,Guiraudon G, Frank R (Editors). Intramyocardial conduction defects patients prone to chronic ventricular tachycardia. The postexci-tation syndrome in sinus rhythm. In: Sondoe E,Julian DG, BellJW, Management of ventriculart achy cardia-role of mexilitine. Amsterdam-Oxford: Experta Medica;1978:39-
  • Marcus FI, McKenna WJ, Sherrill D et al. Diagnosis ofarrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010;121 (13):1533–41.
  • Marcus FI, Fontaine GH, Guiraudon G. et al. Rigth ventricular dysplasia: a report of 24 adult cases. Circulation.1982; 65 (2): 384-98.
  • Corrado D, Basso C, Thine G. et al. spectrum of clinicopathologic manifestation of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am CollCardiol. 1997;30 (6):1512–20.
  • G, Nava A, Corrado D, Rossi L, Pennelli N. Right ventricular cardiomyopathy and sudden death in young people.N Engl J Med. 1988;318 (3):129 –133.
  • .Awad MM, Dalal D, Cho E. et al. DSG2 mutations contributeto arrhythmogenic right ventricular dysplasia/cardiomyopathy. Am J Hum Genet. 2006;79 (1):136 –42.
  • Pilichou K, Nava A, Basso C. et al. Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy. Circulation. 2006;113 (9):1171–79.
  • Beffagna G, Occhi G, Nava A. et al. Regulatory mutations intransforming growth factor-3 gene cause arrhythmogenic right ventricular cardiomyopathy type 1. Cardiovasc Res. 2005;65 (2): 366–73.
  • Jain R, Dalal D, DalyA et al. Electrocardiographic featuresof arrhythmogenic right ventricular dysplasia, Circulation

ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA WITH APICAL THROMBUS ATYPICAL PRESENTATION OF CARDIOMYOPATIES

Year 2018, Volume: 19 Issue: 1, 34 - 37, 15.01.2018
https://doi.org/10.18229/kocatepetip.411211

Abstract

Arrhythmogenic right ventricular dysplasi-a (ARVD) is a rare form of cardiomyopathy. It commonly presents in young adults with ventricular tachycardia or sudden death. Right ventricular (RV) dilatation and apical aneurysm are the typical findings in ima-ging methods. However intraventricular thrombus is rarely seen in ARVD cases. A 19 year old male was admitted to hospital with palpitation and syncope. T wave inversion was detected on anterior surface electro-cardiogram. Transthoracic echocardio-graphy revealed dilated RV and apical ane-urysm in which thrombus located (Fig-1). Cardiac magnetic rezonans imaging comfir-med RV enlargement, fatty infiltration, fibro-sis, wall motion abnormalities and apical ane-ursym with thrombus. Anticoagulation thera-phy commenced to the patient. After three months later trombus resoluted and ICD was implanted. Imaging methods have a great importance in the diagnosis of ARVD besides electrocardiographic, arrhythmic, histological and familial characteristics. While right ventri-cular dilatation and apical aneurysm are im-portant criteria for the diagnosis process, the presence of thrombus should be evaluated carefully.

References

  • Bacior B., Kubinyi A., Grodecki.Arrhythmogenicright ventricular dysplasia—ARVD Case study. Kardiol Pol2002;57(10):337-9.
  • McKenna W.J., Thiene G., Nava A.et al .Diagnosis ofarrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 1994;71(3):215-8. F.I.Marcus, A. Nava, G. Thiene (Editor.)Zareba, W.,Piotrowicz,K., Turrini, P. Electrocardiographic manifestations. in: Arrhythmogenic RV Cardiomyopathy/Dysplasia. Springer, Berlin; 2007:121–128.
  • Durrer D, Van Dam THR, Freud GE, Janse MJ, Meijler FL,Arzbaecher RC. Total excitation of the isolated human heart. Circulation ;1970;41(6):899-912.
  • Fontaine G,Guiraudon G, Frank R (Editors). Intramyocardial conduction defects patients prone to chronic ventricular tachycardia. The postexci-tation syndrome in sinus rhythm. In: Sondoe E,Julian DG, BellJW, Management of ventriculart achy cardia-role of mexilitine. Amsterdam-Oxford: Experta Medica;1978:39-
  • Marcus FI, McKenna WJ, Sherrill D et al. Diagnosis ofarrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010;121 (13):1533–41.
  • Marcus FI, Fontaine GH, Guiraudon G. et al. Rigth ventricular dysplasia: a report of 24 adult cases. Circulation.1982; 65 (2): 384-98.
  • Corrado D, Basso C, Thine G. et al. spectrum of clinicopathologic manifestation of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am CollCardiol. 1997;30 (6):1512–20.
  • G, Nava A, Corrado D, Rossi L, Pennelli N. Right ventricular cardiomyopathy and sudden death in young people.N Engl J Med. 1988;318 (3):129 –133.
  • .Awad MM, Dalal D, Cho E. et al. DSG2 mutations contributeto arrhythmogenic right ventricular dysplasia/cardiomyopathy. Am J Hum Genet. 2006;79 (1):136 –42.
  • Pilichou K, Nava A, Basso C. et al. Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy. Circulation. 2006;113 (9):1171–79.
  • Beffagna G, Occhi G, Nava A. et al. Regulatory mutations intransforming growth factor-3 gene cause arrhythmogenic right ventricular cardiomyopathy type 1. Cardiovasc Res. 2005;65 (2): 366–73.
  • Jain R, Dalal D, DalyA et al. Electrocardiographic featuresof arrhythmogenic right ventricular dysplasia, Circulation
There are 12 citations in total.

Details

Primary Language Turkish
Subjects Health Care Administration
Journal Section Case Report
Authors

Özgen Şafak

S. Volkan Emren This is me

Publication Date January 15, 2018
Acceptance Date March 8, 2016
Published in Issue Year 2018 Volume: 19 Issue: 1

Cite

APA Şafak, Ö., & Emren, S. V. (2018). APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU. Kocatepe Tıp Dergisi, 19(1), 34-37. https://doi.org/10.18229/kocatepetip.411211
AMA Şafak Ö, Emren SV. APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU. KTD. January 2018;19(1):34-37. doi:10.18229/kocatepetip.411211
Chicago Şafak, Özgen, and S. Volkan Emren. “APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU”. Kocatepe Tıp Dergisi 19, no. 1 (January 2018): 34-37. https://doi.org/10.18229/kocatepetip.411211.
EndNote Şafak Ö, Emren SV (January 1, 2018) APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU. Kocatepe Tıp Dergisi 19 1 34–37.
IEEE Ö. Şafak and S. V. Emren, “APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU”, KTD, vol. 19, no. 1, pp. 34–37, 2018, doi: 10.18229/kocatepetip.411211.
ISNAD Şafak, Özgen - Emren, S. Volkan. “APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU”. Kocatepe Tıp Dergisi 19/1 (January 2018), 34-37. https://doi.org/10.18229/kocatepetip.411211.
JAMA Şafak Ö, Emren SV. APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU. KTD. 2018;19:34–37.
MLA Şafak, Özgen and S. Volkan Emren. “APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU”. Kocatepe Tıp Dergisi, vol. 19, no. 1, 2018, pp. 34-37, doi:10.18229/kocatepetip.411211.
Vancouver Şafak Ö, Emren SV. APİKAL TROMBÜS İLE SEYREDEN ARİTMOJENİK SAĞ VENTRİKÜL DİSPLAZİSİ KARDİYOMİYOPATİLERİN ATİPİK BULGUSU. KTD. 2018;19(1):34-7.

88x31.png
Bu Dergi Creative Commons Atıf-GayriTicari-AynıLisanslaPaylaş 4.0 Uluslararası Lisansı ile lisanslanmıştır.