Journal ArticleDOI
Indications and Outcome of Implantable Cardioverter-Defibrillators for Primary and Secondary Prophylaxis in Patients with Noncompaction Cardiomyopathy
Kadir Caliskan,Tamas Szili-Torok,Dominic A.M.J. Theuns,Attila Kardos,Marcel L. Geleijnse,Aggie H.M.M. Balk,Ron T. van Domburg,Luc Jordaens,Maarten L. Simoons +8 more
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TLDR
This study investigates ICD indications and outcomes in NCCM patients with initial presentation of heart failure, emboli, or arrhythmias, including sudden cardiac death with implantable cardioverter‐defibrillators.Abstract:
Prophylactic ICDs for Noncompaction Cardiomyopathy. Background: Noncompaction cardiomyopathy (NCCM) is a rare, primary cardiomyopathy, with initial presentation of heart failure, emboli, or arrhythmias, including sudden cardiac death. Implantable cardioverter-defibrillators (ICDs) are frequently used for primary and secondary prevention in different cardiomyopathy patients, but data about ICD in NCCM are scarce. The aim of this study was, therefore, to investigate ICD indications and outcomes in NCCM patients.
Methods and Results: We collected prospective data from our NCCM cohort (n = 77 pts, mean age: 40 ± 14 years). ICD was implanted in 44 (57%) patients with NCCM according to the current ICD guidelines for nonischemic cardiomyopathies: in 12 for secondary prevention (7 × ventricular fibrillation, 5 × sustained ventricular tachycardia [VT]) and in 32 patients for primary prevention (heart failure/severe LV dysfunction). During a mean follow-up of 33 ± 24 months, 8 patients presented with appropriate ICD shocks due to sustained VT after median 6.1 [1–16] months. This included 4 of 32 (13%) patients in the primary prevention group and 4 of 12 (33%) in the secondary prevention group (P = 0.04). 9 patients presented with inappropriate ICD therapy: 6 (19%) in the primary and 3 (25%) in the secondary prevention group, at a median follow-up of 4 (2–23) months.
Conclusions: In our cohort of NCCM patients, an ICD was frequently implanted for primary or secondary prevention of sudden cardiac death. At follow-up, frequent appropriate ICD therapy was observed in both groups, supporting the application of current ICD guidelines for primary and secondary prevention of sudden cardiac death in NCCM. (J Cardiovasc Electrophysiol, Vol. 22, pp. 898-904, August 2011)read more
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2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy
Jeffrey A. Towbin,William J. McKenna,Dominic Abrams,Michael J. Ackerman,Hugh Calkins,Francisco Darrieux,James P. Daubert,Christian de Chillou,Eugene C. DePasquale,Milind Y. Desai,N.A. Mark Estes,Wei Hua,Julia H. Indik,Jodie Ingles,Cynthia A. James,Roy M. John,Daniel P. Judge,Roberto Keegan,Andrew D. Krahn,Mark S. Link,Frank I. Marcus,Christopher J. McLeod,Luisa Mestroni,Silvia G. Priori,Jeffrey E. Saffitz,Shubhayan Sanatani,Wataru Shimizu,J. Peter van Tintelen,J. Peter van Tintelen,Arthur A.M. Wilde,Arthur A.M. Wilde,Wojciech Zareba +31 more
TL;DR: This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms.
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Left ventricular non-compaction cardiomyopathy.
TL;DR: Treating of arrhythmia and implantation of an automatic implantable cardioverter-defibrillator for prevention of sudden death are mainstays of therapy when deemed necessary and appropriate in patients with systolic dysfunction.
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Left ventricular noncompaction cardiomyopathy: cardiac, neuromuscular, and genetic factors
TL;DR: The current findings on the aetiology and pathophysiology of LVHT are discussed, and an overview of the diagnosis, available treatment, and prognosis of this cardiomyopathy is provided.
Journal ArticleDOI
2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy: Executive summary.
Jeffrey A. Towbin,William J. McKenna,Dominic Abrams,Michael J. Ackerman,Hugh Calkins,Francisco Darrieux,James P. Daubert,Christian de Chillou,Eugene C. DePasquale,Milind Y. Desai,N.A. Mark Estes,Wei Hua,Julia H. Indik,Jodie Ingles,Cynthia A. James,Roy M. John,Daniel P. Judge,Roberto Keegan,Andrew D. Krahn,Mark S. Link,Frank I. Marcus,Christopher J. McLeod,Luisa Mestroni,Silvia G. Priori,Jeffrey E. Saffitz,Shubhayan Sanatani,Wataru Shimizu,J. Peter van Tintelen,J. Peter van Tintelen,Arthur A.M. Wilde,Arthur A.M. Wilde,Wojciech Zareba +31 more
TL;DR: This expert consensus statement provides the clinician with guidance on evaluation and management of arrhythmogenic cardiomyopathy and includes clinically relevant information on genetics and disease mechanisms.
Journal ArticleDOI
Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism.
TL;DR: In this review, left ventricular noncompaction cardiomyopathy, which is often caused by mutations in sarcomere and cytoskeletal proteins and is also associated with metabolic abnormalities, is discussed andCardiomyopathies resulting from mitochondrial dysfunction, metabolism abnormalities, storage diseases, and inborn errors of metabolism are described.
References
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Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis
TL;DR: Its mortality and morbidity are high, including heart failure, thrombo-embolic events and ventricular arrhythmias, and classification as a specific cardiomyopathy seems to be more appropriate.