title: Electrical storm in systemic sclerosis: Inside the electroanatomic substrate.
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.
authors.affiliation: 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.
authors.forename: Michela
authors.initials: M
authors.lastname: Casella
authors.affiliation: 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.
authors.forename: Corrado
authors.initials: C
authors.lastname: Carbucicchio
authors.affiliation: 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.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Russo
authors.affiliation: 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.
authors.forename: Francesca
authors.initials: F
authors.lastname: Pizzamiglio
authors.affiliation: 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.
authors.forename: Paolo
authors.initials: P
authors.lastname: Golia
authors.affiliation: 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.
authors.forename: Sergio
authors.initials: S
authors.lastname: Conti
authors.affiliation: 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.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Costa
authors.affiliation: 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.
authors.forename: Antonio
authors.initials: A
authors.lastname: Dello Russo
authors.affiliation: 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.
authors.forename: Claudio
authors.initials: C
authors.lastname: Tondo
keywords: Electrical storm; Radiofrequency catheter ablation; Systemic sclerosis; Ventricular tachycardia