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Ventricular tachycardia storm--an atypical presentation of sarcoidosis exacerbation.
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MedLine Citation:
PMID:  19898658     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Management of ventricular tachycardia (VT) storm in a patient with an implantable cardioverter-defibrillator (ICD) is a challenging medical emergency. We describe a patient with cardiac sarcoidosis (CS) and an ICD who is admitted with VT storm. Management of VT was difficult due to resistance to multiple antiarrhythmic drugs. He responded to immunosuppressive therapy supporting active CS as the cause of his VT. This case suggests that CS may underlie some cases of refractory VT and that immunosuppressive therapy may be effective in controlling this arrhythmia.
Authors:
Subba Reddy Vanga; James Vacek; Loren Berenbom; Dhanunjaya R Lakkireddy
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Publication Detail:
Type:  Journal Article     Date:  2009-11-01
Journal Detail:
Title:  Indian pacing and electrophysiology journal     Volume:  9     ISSN:  0972-6292     ISO Abbreviation:  Indian Pacing Electrophysiol J     Publication Date:  2009  
Date Detail:
Created Date:  2009-11-09     Completed Date:  2010-05-20     Revised Date:  2013-05-23    
Medline Journal Info:
Nlm Unique ID:  101157207     Medline TA:  Indian Pacing Electrophysiol J     Country:  India    
Other Details:
Languages:  eng     Pagination:  351-4     Citation Subset:  -    
Affiliation:
Mid-America Cardiology, Kansas University Hospital, Kansas City KS 66220, USA.
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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Journal Information
Journal ID (nlm-ta): Indian Pacing Electrophysiol J
Journal ID (publisher-id): Indian Pacing Electrophysiol J
ISSN: 0972-6292
Publisher: Indian Heart Rhythm Society
Article Information
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Copyright: © 2009 Vanga et al.
open-access:
collection publication date: Season: Nov–Dec Year: 2009
Electronic publication date: Day: 01 Month: 11 Year: 2009
Volume: 9 Issue: 6
First Page: 351 Last Page: 354
ID: 2766583
PubMed Id: 19898658
Publisher Id: ipej090351-00

Ventricular Tachycardia Storm - An Atypical Presentation of Sarcoidosis Exacerbation
Subba Reddy Vanga, MBBS
James Vacek, MD
Loren Berenbom, MD
Dhanunjaya R Lakkireddy, MD
Mid-America Cardiology, Kansas University Hospital, Kansas City KS
Correspondence: Address for correspondence: Dr. Dhanunjaya Lakkireddy, MD, FACC, Director, Center for Excellence in Atrial Fibrillation & EP Research, Bloch Heart Rhythm Center, Mid-America Cardiology @ Kansas University Hospital, 3901 Rainbow Blvd, Kansas City KS 66220. E-mail: dlakkireddy@mac.md

Introduction

Sarcoidosis is a disease of unknown etiology and protean manifestations. Cardiac involvement is not uncommon. Rhythm disturbances are an important manifestation and can lead to sudden cardiac death. We report a patient with CS who presented with ventricular tachycardia storm resistant to traditional antiarrhythmics. He responded well to corticosteroids and methotrexate.


Case Report

A 46-year-old male with hypertension, type 2 diabetes mellitus and obstructive sleep apnea was diagnosed histologically with pulmonary sarcoidosis 5 years previously. He was treated with oral prednisone tapered over 6 months. Three years later, he developed Mobitz 1 second-degree atrioventricular block. Six months after that he developed congestive heart failure (CHF) with left ventricular ejection fraction (LVEF) of 40%. A coronary angiogram did not reveal any significant stenosis. Cardiac MRI showed concentric left ventricular hypertrophy with hyperenhancement. Endomyocardial biopsy confirmed extensive myocardial sarcoidosis. (Figure 1). He had progressive decline of his LVEF to 20% and developed left bundle branch block (QRS 165 msec). He was treated with oral prednisone 60mg daily. In light of functional class III CHF despite optimal medical therapy, a cardiac re-synchronization ICD (Medtronic Concerto CI54DWK) was implanted. Over the next two months, he received six shocks for sustained VT and was placed on sotalol 120 mg twice daily with continued oral steroid therapy. His CHF symptoms improved and arrhythmia was under control.

Five months after CRT-D implantation, he was admitted with multiple episodes of VT despite being on oral prednisone 30 mg daily and sotalol. He was treated successfully with antitachycardia pacing 56 times, and he was cardioverted 12 times over the course of 24 hours. VT was right bundle inferior axis with negative precordial concordance suggesting an apical origin. All episodes of VT were of similar morphology with a cycle length of 300 mSec. Lidocaine, mexiletine and amiodarone reduced the frequency of sustained VT episodes, but he continued to have episodes of symptomatic non-sustained VT. LVEF was 15-20%. A whole body gallium scan demonstrated uptake in the heart consistent with exacerbation of cardiac sarcoidosis (Figure 2). High dose intravenous steroid therapy (methylprednisolone 60 mg every 6 hours) was initiated in place of chronic oral steroid therapy and VT resolved within 12 hours. Oral methotrexate was added as a steroid sparing strategy. Sotalol was continued and other antiarrhythmics were stopped. Three days later, he was discharged on methotrexate (15mg/week PO) and a tapering dose of oral prednisone. At 3 and 6 months follow-up visits, his CHF was compensated with an LVEF of 25-30% and he was free of ventricular tachycardia on device interrogation. Repeat gallium scan at 6 months showed no significant uptake in the heart.


Discussion

Management of arrhythmias in CS is difficult and effective control of VT often is not achievable by a single method of therapy. Acute inflammation with new granuloma formation that can disrupt myocardial fibers together with the patchy scarring associated with old healed granulomas can provide a substrate for new VT reentrant circuits. Antiarrhythmic drugs can facilitate occurrence of VT, as well as aggravate conduction abnormalities. Hypercalcemia, inherent to sarcoidosis; hypokalemia from concurrent corticosteroid use, as well as the interaction between acute phase reactants and antiarrhythmics can further complicate drug therapy [1].

Optimal therapy of ventricular arrhythmia associated with CS is not well established. Various regimens including corticosteroids, antiarrhythmic medications, ICDs and catheter ablations have been attempted. Steroids improved overall mortality in CS [2]. Steroids are believed to be capable of attenuating the inflammatory response and slowing subsequent fibrosis. Although corticosteroids help in suppressing arrhythmia secondary to sarcoid exacerbation, they may be of little use in the management of VT from scarred myocardium. This can explain the recurrence of VT despite treatment with corticosteroids in previously reported cases [3-4]. Active sarcoid exacerbation may cause VT in patients who are already on treatment with moderate to high doses of corticosteroids as seen in this patient. Immunosuppressive therapies are recommended as a steroid sparing strategy in the management of sarcoidosis and can be beneficial in controlling arrhythmia.

A stepwise approach in the management this malignant arrhythmia was studied [5]. ICD implantation, immunosuppression with corticosteroids, then up to two antiarrhythmic drugs were used (in that order) before patients were taken to the EP lab for mapping and ablation. 9 of the 21 patients with sustained VT underwent ablation. 44 VTs were induced and 31 were ablated. VT burden was reduced more than 98% in the first 3 months post ablation. The mechanism of VT in virtually all patients was re-entry [5,6].

Cardiac Resynchronization Therapy (CRT), which is an adjunct treatment to pharmacological therapy in patients with advanced heart failure, may have potential proarrhythmic effects [7]. Although rare, CRT has been shown to cause VT [8] and electrical storm [9]. Most CRT associated ventricular proarrhythmia was seen in ischemic cardiomyopathy patients soon after biventricular pacing lead implantation, and improved with cessation of biventricular pacing [8,9]. This patient tolerated CRT well for months. The presence of extensive myocardial sarcoidosis and lack of recurrent VT during the follow-up period despite continued CRT makes CRT related proarrhythmia an unlikely mechanism of VT in our patient. Electrophysiology study with ablation can be undertaken in CS, but VT circuits can change as inflammation waxes and wanes. VT may be ablated only to reoccur as the substrate shifts. Ablation should be considered as an option in those patients with recurrent VT that are unresponsive to antiarrhythmic and immunosuppressive drugs.


Conclusion

Cardiac sarcoidosis related arrhythmia can be very difficult to manage. Because of increased awareness of the risk of sudden cardiac death and early ICD implantation, CS patients can survive to present with VT storm, which is not uncommonly resistant to standard antiarrhythmic drugs. While corticosteroids are mainstay of therapy to control the acute inflammation and hence related arrhythmia, immunosuppressive therapy such as methotrexate should be considered early if steroid resistance develops and before proceeding to catheter ablation.


References
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Belhassen B,et al. Failure of corticosteroid therapy to prevent induction of ventricular tachycardia in sarcoidosisChestYear: 1989959182924626
Hiramastu S,et al. Steroid treatment deteriorated ventricular tachycardia in a patient with right ventricle-dominant cardiac sarcoidosisInt J CardiolYear: 2009132e8518037174
Jefic D,et al. Role of radiofrequency catheter ablation of ventricular tachycardia in cardiac sarcoidosis: report from a multicenter registryHeart RhythmYear: 2009618919187909
Koplan BA,et al. Refractory ventricular tachycardia secondary to cardiac sarcoid: electrophysiologic characteristics, mapping, and ablationHeart RhythmYear: 2006392416876741
Medina-Ravell VA,et al. Effect of epicardial or biventricular pacing to prolong QT interval and increase transmural dispersion of repolarization: does resynchronization therapy pose a risk for patients predisposed to long QT or torsade de pointes?CirculationYear: 200310774012578878
Mykytsey A,et al. Ventricular tachycardia induced by biventricular pacing in patient with severe ischemic cardiomyopathyJ Cardiovasc ElectrophysiolYear: 20051665515946367
Kantharia K,et al. Electrical storm of monomorphic ventricular tachycardia after a cardiac-resynchronization-therapy-defibrillator upgradeEuropaceYear: 2006862516772367

Article Categories:
  • Case Report

Keywords: Cardiac Sarcoidosis, VT Storm.

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