Case Report
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World J Cardiol. Oct 26, 2014; 6(10): 1127-1130
Published online Oct 26, 2014. doi: 10.4330/wjc.v6.i10.1127
Electrical storm in systemic sclerosis: Inside the electroanatomic substrate
Michela Casella, Corrado Carbucicchio, Eleonora Russo, Francesca Pizzamiglio, Paolo Golia, Sergio Conti, Fabrizio Costa, Antonio Dello Russo, Claudio Tondo
Michela Casella, Corrado Carbucicchio, Eleonora Russo, Francesca Pizzamiglio, Sergio Conti, Antonio Dello Russo, Claudio Tondo, Cardiac Arrhythmia Research Centre, Centro Cardiologico Monzino IRCCS, 20138 Milan, Italy
Paolo Golia, Department of Cardiovascular Disease, Morgagni-Pierantoni Hospital, 27121 Forlì, Italy
Fabrizio Costa, Biosense-Webster, 00040 Pomezia, Italy
Author contributions: Casella M, Carbucicchio C and Dello Russo A contributed to conception and design, acquisition of data, analysis and interpretation of data, drafting the article, final approval of the version to be published; Russo E, Golia P and Costa F contributed to conception and design, acquisition of data, analysis and interpretation of data; Pizzamiglio F and Conti S contributed to drafting the article; Tondo C final approval of the version to be published.
Correspondence to: Michela Casella, MD, PhD, Cardiac Arrhythmia Research Centre, Centro Cardiologico Monzino, IRCCS, Via Parea 4, 20138 Milan, Italy. michela.casella@ccfm.it
Telephone: +39-02-58002340 Fax: +39-02-58002398
Received: March 25, 2014
Revised: April 23, 2014
Accepted: July 25, 2014
Published online: October 26, 2014
Processing time: 224 Days and 13.6 Hours
Abstract

We report the case of a 63-year-old woman affected by a severe form of systemic scleroderma with pulmonary involvement (interstitial fibrosis diagnosed by biopsy and moderate pulmonary hypertension) and cardiac involvement (paroxysmal atrial fibrillation, right atrial flutter treated by catheter ablation, ventricular tachyarrhythmias, previous dual chamber implantable cardioverter defibrillator implant). Because of recurrent electrical storms refractory to iv antiarrhythmic drugs the patient was referred to our institution to undergo catheter ablation. During electrophysiological procedure a 3D shell of cardiac anatomy was created with intracardiac echocardiography pointing out a significant right ventricular dilatation with a complex aneurysmal lesion characterized by thin walls and irregular multiple trabeculae. A substrate-guided strategy of catheter ablation was accomplished leading to a complete electrical isolation of the aneurism and to the abolishment of all abnormal electrical activities. The use of advanced strategies of imaging together with electroanatomical mapping added important information to the complex arrhythmogenic substrate and improved efficacy and safety.

Keywords: Ventricular tachycardia; Electrical storm; Radiofrequency catheter ablation; Systemic sclerosis

Core tip: We report the case of a 63-year-old woman affected by a severe form of systemic scleroderma with cardiac involvement. Because of recurrent electrical storms the patient underwent catheter ablation. Intracardiac echocardiography pointed out a significant right ventricular dilatation with a complex aneurysmal lesion characterized by thin walls and irregular multiple trabeculae. A substrate-guided strategy of catheter ablation was accomplished leading to a complete electrical isolation of the aneurism. The use of advanced strategies of imaging together with electroanatomical mapping added important information to the complex arrhythmogenic substrate and improved efficacy and safety.