Left ventricular noncompaction cardiomyopathy

Cardiac, neuromuscular, and genetic factors

Josef Finsterer, Claudia Stöllberger, Jeffrey Towbin

Research output: Contribution to journalReview article

46 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)224-237
Number of pages14
JournalNature Reviews Cardiology
Volume14
Issue number4
DOIs
StatePublished - Apr 1 2017

Fingerprint

Cardiomyopathies
Sudden Cardiac Death
Heart Failure
Implantable Defibrillators
Thromboembolism
Gadolinium
Syncope
Ventricular Tachycardia
Embolism
Atrial Fibrillation
Echocardiography
Cardiac Arrhythmias
Myocardium
Thrombosis
Mortality
Therapeutics

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Left ventricular noncompaction cardiomyopathy : Cardiac, neuromuscular, and genetic factors. / Finsterer, Josef; Stöllberger, Claudia; Towbin, Jeffrey.

In: Nature Reviews Cardiology, Vol. 14, No. 4, 01.04.2017, p. 224-237.

Research output: Contribution to journalReview article

@article{89a8638d722f4c4881fe271eef65f851,
title = "Left ventricular noncompaction cardiomyopathy: Cardiac, neuromuscular, and genetic factors",
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.",
author = "Josef Finsterer and Claudia St{\"o}llberger and Jeffrey Towbin",
year = "2017",
month = "4",
day = "1",
doi = "10.1038/nrcardio.2016.207",
language = "English (US)",
volume = "14",
pages = "224--237",
journal = "Nature Reviews Cardiology",
issn = "1759-5002",
publisher = "Nature Publishing Group",
number = "4",

}

TY - JOUR

T1 - Left ventricular noncompaction cardiomyopathy

T2 - Cardiac, neuromuscular, and genetic factors

AU - Finsterer, Josef

AU - Stöllberger, Claudia

AU - Towbin, Jeffrey

PY - 2017/4/1

Y1 - 2017/4/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85009360714&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85009360714&partnerID=8YFLogxK

U2 - 10.1038/nrcardio.2016.207

DO - 10.1038/nrcardio.2016.207

M3 - Review article

VL - 14

SP - 224

EP - 237

JO - Nature Reviews Cardiology

JF - Nature Reviews Cardiology

SN - 1759-5002

IS - 4

ER -