Biventriküler Kalp Yetmezliği ile Karışımıza Çıkan Aritmojenik Sağ Ventrikül Kardiyomiyopatisi (ASVK)
Abstract
Aritmojenik sağ ventrikül kardiyomiyopatisi (ASVK), farkındalığı giderek artan bir klinik tablodur. Seyrek görülmesine rağmen gençlerde beklenmedik ölümlerin sık karşılaşılan nedenlerindendir. Sağ ventrikülün baskın tutulumu nedeniyle aritmojenik sağ ventrikül kardiyomiyopatisi olarak adlandırılmış olmasına rağmen, son yıllarda ciddi bir sol ventrikül tutulumunun eşlik ettiği tablolar da tanımlanmıştır. Bu yazıda, daha öncesinde herhangi bir klinik belirti vermeyen, iki kardeşinde de ani ölüm öyküsü olan, ani konjestif kalp yetmezliği nedeniyle kaybedilen on beş yaşında bir erkek hastayı sunduk. Erken tanı sayesinde istenmeyen sonuçların önüne geçebilme şansımız olduğunu, bir kez daha hatırlatmak istedik
References
- Fontaine G, Fontaliran F, Hébert JL, Chemla D, Zenati O, Lecarpentier Y, et al. Arrhythmogenic right ventricu- lar dysplasia. Annu Rev Med. 1999;50:17–35.
- Dalal D, Molin LH, Piccini J, Tichnell C, James C, Bomma C, et al. Clinical features of arrhythmogenic right ventricular dysplasia/cardiomyopathy associ- ated with mutations in plakophilin-2. Circulation. 2006;113(13):1641–9.
- Thiene G, Corrado D, Nava A, Rossi L, Poletti A, Boffa GM, et al. Right ventricular cardiomyopathy: is there evidence of an inflammatory etiology? Eur Heart J. 1991;12(supp D):22–5.
- Corrado D, Wichter T, Link M, Hauer R, Marchlinski F, Anastasakis A, et al. Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia an inter- national task force consensus statement. Circulation. 2015;132(5): 441–53.
- McKenna WJ, Thiene G, Nava A, Fontaliran F, Blom- strom-Lundqvist C, Fontaine G, et al. Diagnosis of ar- rhythmogenic right ventricular dysplasia/cardiomyopa- thy. Br Heart J 1994;71(3):215–8.
- Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modiŞcation of the Task Force Criteria. Eur Heart J. 2010;31(7):806–14.
- Corrado D, Basso C, Thiene G, McKenna WJ, Davies MJ, Fontaliran F, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular car- diomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997;30(6):1512–20.
- De Pasquale CG, Heddle WF. Left sided arrhythmogenic ventricular dysplasia in siblings. Heart. 2001;86:128–30.
- Sen-Chowdhry S, Syrris P, Ward D, Asimaki A, Sevda- lis E, McKenna WJ, et al. Clinical and genetic charac- terization of families with arrhythmogenic right ven- tricular dysplasia/cardiomyopathy provides novel in- sights into patterns of disease expression. Circulation. 2007;115(13):1710–20.
- Corrado D, Leoni L, Link MS, Della Bella P, Gaita F, Curnis A, et al. Implantable cardioverter-deŞbrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dys- plasia. Circulation. 2003;108(25):3084–91.
- Aoute P, Fontaliran F, Fontaine G, Frank R, Benassar A, Lascault G, et al. Holter et mort subite: intérêt dans un cas de dysplasie ventriculaire droite arythmogène. Arch Mal Coeur Vaiss. 1993; 86:363–7.
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Presenting with Biventricular Heart Failure / Biventriküler Kalp Yetmezliği ile Karışımıza Çıkan Aritmojenik Sağ Ventrikül Kardiyomiyopatisi (ASVK)
Abstract
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder which is attracting increased awareness in clinical practice. Despite being an uncommon disease, it is a frequent cause of unexpected death in young persons. Although the term ARVC used for this cardiomyopathy suggests that it involves the muscle of the right ventricle, in recent years there were cases reported in which the left ventricle was severely affected. The current study reports an ARVC-diagnosed fifteen-year-old male patient who had no clinical features previously and died from sudden congestive heart failure; he had two siblings with a history of sudden unexpected death. We would like to bring to mind once again the important role of early diagnosis for ARVC to avoid undesirable consequences of the disease.
Keywords
arrhythmogenic right ventricular cardiomyopathy (ARVC) biventricular heart failure adolescent
References
- Fontaine G, Fontaliran F, Hébert JL, Chemla D, Zenati O, Lecarpentier Y, et al. Arrhythmogenic right ventricu- lar dysplasia. Annu Rev Med. 1999;50:17–35.
- Dalal D, Molin LH, Piccini J, Tichnell C, James C, Bomma C, et al. Clinical features of arrhythmogenic right ventricular dysplasia/cardiomyopathy associ- ated with mutations in plakophilin-2. Circulation. 2006;113(13):1641–9.
- Thiene G, Corrado D, Nava A, Rossi L, Poletti A, Boffa GM, et al. Right ventricular cardiomyopathy: is there evidence of an inflammatory etiology? Eur Heart J. 1991;12(supp D):22–5.
- Corrado D, Wichter T, Link M, Hauer R, Marchlinski F, Anastasakis A, et al. Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia an inter- national task force consensus statement. Circulation. 2015;132(5): 441–53.
- McKenna WJ, Thiene G, Nava A, Fontaliran F, Blom- strom-Lundqvist C, Fontaine G, et al. Diagnosis of ar- rhythmogenic right ventricular dysplasia/cardiomyopa- thy. Br Heart J 1994;71(3):215–8.
- Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modiŞcation of the Task Force Criteria. Eur Heart J. 2010;31(7):806–14.
- Corrado D, Basso C, Thiene G, McKenna WJ, Davies MJ, Fontaliran F, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular car- diomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997;30(6):1512–20.
- De Pasquale CG, Heddle WF. Left sided arrhythmogenic ventricular dysplasia in siblings. Heart. 2001;86:128–30.
- Sen-Chowdhry S, Syrris P, Ward D, Asimaki A, Sevda- lis E, McKenna WJ, et al. Clinical and genetic charac- terization of families with arrhythmogenic right ven- tricular dysplasia/cardiomyopathy provides novel in- sights into patterns of disease expression. Circulation. 2007;115(13):1710–20.
- Corrado D, Leoni L, Link MS, Della Bella P, Gaita F, Curnis A, et al. Implantable cardioverter-deŞbrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dys- plasia. Circulation. 2003;108(25):3084–91.
- Aoute P, Fontaliran F, Fontaine G, Frank R, Benassar A, Lascault G, et al. Holter et mort subite: intérêt dans un cas de dysplasie ventriculaire droite arythmogène. Arch Mal Coeur Vaiss. 1993; 86:363–7.
Details
| Subjects | Health Care Administration |
|---|---|
| Journal Section | CASE REPORT |
| Authors | |
| Publication Date | April 30, 2016 |
| Acceptance Date | November 24, 2015 |
| Published in Issue | Year 2016 Volume: 21 Issue: 2 |
This Journal licensed under a CC BY-NC (Creative Commons Attribution-NonCommercial 4.0) International License.