Myocardial scar predicts monomorphic ventricular tachycardia but not polymorphic ventricular tachycardia or ventricular fibrillation in nonischemic dilated cardiomyopathy

Heart Rhythm ◽  
2015 ◽  
Vol 12 (10) ◽  
pp. 2106-2114 ◽  
Author(s):  
Sebastiaan R.D. Piers ◽  
Kimberly Everaerts ◽  
Rob J. van der Geest ◽  
Mark R. Hazebroek ◽  
Hans-Marc Siebelink ◽  
...  

Author(s):  
Granitz Christina ◽  
Jirak Peter ◽  
Strohmer Bernhard ◽  
Pölzl Gerhard

Abstract Background  Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a severe genetic arrhythmogenic disorder characterized by adrenergically induced ventricular tachycardia manifesting as stress-induced syncope and sudden cardiac death. While CPVT is not associated with dilated cardiomyopathy (DCM) in most cases, the combination of both disease entities poses a major diagnostic and therapeutic challenge. Case summary  We present the case of a young woman with CPVT. The clinical course since childhood was characterized by repetitive episodes of exercise-induced ventricular arrhythmias and a brady-tachy syndrome due to rapid paroxysmal atrial fibrillation and sinus bradycardia. Medical treatment included propranolol and flecainide until echocardiography showed a dilated left ventricle with severely depressed ejection fraction when the patient was 32 years old. Cardiac magnetic resonance imaging revealed non-specific late gadolinium enhancement. Myocardial inflammation, however, was excluded by subsequent endomyocardial biopsy. Genetic analysis confirmed a mutation in the cardiac ryanodine receptor but no pathogenetic variant associated with DCM. Guideline-directed medical therapy for HFrEF was limited due to symptomatic hypotension. Over the next months, the patient developed progressive heart failure symptoms that were finally managed by heart transplantation. Discussion  Management in patients with CPVT and DCM is challenging, as Class I antiarrhythmic drugs are not recommended in structural heart disease and prophylactic internal cardioverter-defibrillator implantation without adjuvant antiarrhythmic therapy can be detrimental. Regular echocardiographic screening for DCM is recommendable in patients with CPVT. A multidisciplinary team of heart failure specialists, electrophysiologists, geneticists, and imaging specialists is needed to collaborate in the delivery of clinical care.



Heart Rhythm ◽  
2013 ◽  
Vol 10 (12) ◽  
pp. 1913-1916 ◽  
Author(s):  
Justin Hayase ◽  
Roderick Tung ◽  
Sanjiv M. Narayan ◽  
David E. Krummen


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Borislav Dinov ◽  
Arash Arya ◽  
Valentina Schirripa ◽  
Livio Bertagnolli ◽  
Lukas Fiedler ◽  
...  

Introduction: Recent publications reported on higher recurrence rates and lack of survival benefit after catheter ablation (CA) of ventricular tachycardia (VT) in nonischemic dilated cardiomyopathy (NIDCM). Methods: We aimed to investigate the VT recurrence and cardiac mortality in patients with NIDCM ablated for VT. The studied cohort was divided in 2 groups depending on procedure success: complete success (group 1), and failure or incomplete success (group 2). Success definition was based on the VT inducibility after CA. The patients were prospectively followed for cardiac mortality and VT recurrence. Results: 104 patients with NIDCM (87 males, mean age 59.65 ± 14.69 years, mean ejection fraction 33.42 ± 11.42 %) underwent VT ablation. Ventricular stimulation after CA was not attempted in 13 (12.5%) patients. Out of the rest 91, complete success was achieved in 62 (68.1%) patients (group1), and incomplete success or failure in 29 (31.9%) patients (group 2). During 2-years follow-up, VT recurrence was observed in 56.5% in group 1 vs. 82.8% in group 2. Incomplete success was associated with higher VT recurrence (HR 1.91; 95% CI 1.13-3.22; p=0.015). The 2-years mortality was 14.5% in group 1 vs 34.5% in group 2. The probability for death was 3-times higher in group 2 (adjusted HR 3.18; 95% CI 1.18-8.56; p=0.022). The primary and secondary endpoints were comparable between patients with idiopathic, post-myocarditis and secondary NIDCM. Conclusion: Procedure success, defined as complete VT noninducibility after CA of VT, was associated with reduced VT recurrence and improved survival in patients with nonischemic dilated cardiomyopathy.



2013 ◽  
pp. 577-589
Author(s):  
Frédéric Sacher ◽  
Mélèze Hocini ◽  
Sébastien Knecht ◽  
Nicolas Derval ◽  
Pierre Jaïs ◽  
...  


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