Journal ArticleDOI
Left ventricular noncompaction cardiomyopathy: cardiac, neuromuscular, and genetic factors
TLDR
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.Abstract:
Left ventricular hypertrabeculation (LVHT) or noncompaction is a myocardial abnormality of unknown aetiology, frequently associated with monogenic disorders, particularly neuromuscular disorders, or with chromosomal defects. LVHT is diagnosed usually by echocardiography by the presence of a bilayered myocardium consisting of a thick, spongy, noncompacted endocardial layer and a thin, compacted, epicardial layer. The pathogenesis of LVHT is unsolved, and the diagnostic criteria, prognosis, and optimal treatment of patients with LVHT are under debate. LVHT is categorized as distinct primary genetic cardiomyopathy by the AHA and as unclassified cardiomyopathy by the ESC. LVHT is usually asymptomatic, but can be complicated by heart failure, thromboembolism, or ventricular arrhythmias, including sudden cardiac death. Mortality of patients with LVHT ranges from 5% to 47%. Anticoagulation is indicated if atrial fibrillation, severe heart failure, previous embolism, or intracardiac thrombus formation are present. In patients with LVHT with late gadolinium enhancement, an implantable cardioverter-defibrillator might be considered if systolic dysfunction, a family history of sudden cardiac death, nonsustained ventricular tachycardia, or previous syncope is additionally present. In this Review, we discuss the current findings on the aetiology and pathophysiology of LVHT, and provide an overview of the diagnosis, available treatment, and prognosis of this cardiomyopathy.read more
Citations
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2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio-Thoracic Surgery (EACTS).
Gerhard Hindricks,Tatjana S. Potpara,Nikolaos Dagres,Elena Arbelo,Jeroen J. Bax,Carina Blomström-Lundqvist,Giuseppe Boriani,Manuel Castellá,Gheorghe Andrei Dan,Polychronis Dilaveris,Laurent Fauchier,Gerasimos Filippatos,Jonathan M. Kalman,Jonathan M. Kalman,Mark La Meir,Deirdre A. Lane,Jean-Pierre Lebeau,Maddalena Lettino,G. Y. H. Lip,Fausto J. Pinto,G. Neil Thomas,Marco Valgimigli,Isabelle C. Van Gelder,Isabelle C. Van Gelder,Bart P. Van Putte,Caroline L Watkins +25 more
TL;DR: The content of these European Society of Cardiology (ESC) Guidelines has been published for personal and educational use only and no commercial use is authorized.
Journal ArticleDOI
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.
Journal ArticleDOI
Prevalence of congenital heart disease
TL;DR: Survival of patients with congenital heart disease, treated or untreated, is expected to produce large numbers of adults with congenitals disease, and it is likely that many more adult cardiologists will need to be trained to manage moderate and complex congenital lesions.
Journal ArticleDOI
Classification, Epidemiology, and Global Burden of Cardiomyopathies
TL;DR: Cardiomyopathies are easily diagnosed but treated only with palliative pharmacological or invasive therapy, and curative therapy has to target the fundamental mechanisms involved in the onset and progression of these conditions.
Journal ArticleDOI
Oligogenic inheritance of a human heart disease involving a genetic modifier
Casey A. Gifford,Sanjeev S. Ranade,Ryan Samarakoon,Hazel T. Salunga,T. Yvanka de Soysa,Yu Huang,Ping Zhou,Arye Elfenbein,Stacia K. Wyman,Yen Kim Bui,Kimberly R. Cordes Metzler,Philip C. Ursell,Kathryn N. Ivey,Deepak Srivastava +13 more
TL;DR: It is shown that a human cardiac anomaly can be caused by a combination of rare, inherited heterozygous mutations, and compound heterozygosity for all three variants recapitulated the human disease phenotype.
References
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Journal ArticleDOI
Isolated noncompaction of left ventricular myocardium. A study of eight cases.
TL;DR: It is concluded that isolated noncompaction of left ventricular myocardium is a rare if not unique disorder with characteristic morphological features that can be identified by two-dimensional echocardiography.
Journal ArticleDOI
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.