Arrhythmias in Left Ventricular Noncompaction

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Key points

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    Left ventricular noncompaction (LVNC) is associated with heart failure, arrhythmias, thromboembolic events, and sudden death.

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    Arrhythmias are common and may have prognostic significance in LVNC; the risk of sudden death seems to be associated with left ventricular (LV) size, systolic function, and presence of arrhythmias.

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    Arrhythmias are not restricted to noncompacted myocardium and include atrial fibrillation (AF) (adults), atrioventricular (AV) accessory pathways/Wolff-Parkinson-White (WPW)

Genetics

LVNC is associated with both sporadic and familial cases, with an estimated familial recurrence rate of 23% to 33%.16, 19, 20, 21, 22 Inheritance most commonly follows an autosomal dominant or X-linked pattern, although autosomal recessive and mitochondrial inheritance have also been observed.23, 24 As is seen with many inherited cardiac diseases, variable penetrance and phenotypic variability result in lack of clear genotype-phenotype correlation, even among family members carrying the same

Electrocardiographic findings

Electrocardiographic (ECG) abnormalities are a hallmark of many cardiomyopathies. Early findings in recent studies suggest that LVNC may be similar in this regard. An evaluation by Steffel and colleagues39 found that only 13% of adults with diagnosed LVNC had normal results of ECGs at presentation.39 The most common findings were intraventricular conduction delay such as left bundle branch block, voltage signs of hypertrophy, and repolarization abnormalities (Table 3). Over 70% of the

Ventricular arrhythmias

Early descriptions of LVNC point to 3 major clinical manifestations of the disease, including heart failure, embolic events, and arrhythmias.5, 8 Although a variety of arrhythmias have subsequently been identified in association with LVNC, ventricular tachyarrhythmias have been considered the hallmark. Recent larger series have found VT in as many as 47% of affected individuals (Table 4), with sudden cardiac death (SCD) accounting for a significant portion of the mortality.5, 8, 17, 40, 41

Supraventricular arrhythmias

Recent reports suggest that supraventricular arrhythmias are also implicated in the natural history of LVNC. In adults, this seems to be primarily manifested by AF. Studies by Weiford and colleagues18 and Ritter and colleagues8 describe a prevalence of AF in their cohorts of as high as 29% (see Table 4). Other evaluations, however, found a slightly lower prevalence of AF in LVNC, ranging from 5% to 26%.20, 21, 50 The mechanistic role of LVNC in the development of AF remains unclear; it may be

Sudden death

In current reports, rates of sudden death have varied significantly, ranging from 0% to 18%, possibly because of differences in selection of patient population (isolated vs nonisolated LVNC) and length of follow-up.5, 6, 8, 16, 17, 19, 20, 21, 52 In the largest pediatric study of isolated LVNC to date, the risk of sudden death was reported to be 6%.16 In the scheme of all-cause mortality, sudden death may account for up to 50% of deaths, suggesting the possible importance of primary or

Risk assessment

The ability to identify predictors of outcome is important in the effective and optimal management of all patients with cardiomyopathy. Unfortunately, determining risk factors for mortality or sudden death in LVNC has been complicated by several factors including: (1) phenotypic heterogeneity of disease, (2) varying diagnostic criteria, and (3) lack of randomized controlled studies. Thus, definitive risk factors remain lacking. Nonetheless, as mentioned above, increased mortality does seem to

Therapeutic approaches

Proposed therapeutic strategies for arrhythmias in LVNC are summarized in Table 5.

Summary

LVNC is a newly recognized form of cardiomyopathy that has been associated with heart failure, arrhythmias, thromboembolic events, and sudden death. Both ventricular and supraventricular arrhythmias are now well described as prominent clinical components of LVNC. Arrhythmias are not restricted to noncompacted myocardium and can include AF (adults), AV accessory pathways/WPW and SVT (children), and VT. Throughout the spectrum of age, these arrhythmias have been associated with prognosis and

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References (74)

  • B. Chang et al.

    Noncompaction study collaborators. Identification of a novel TPM1 mutation in a family with left ventricular noncompaction and sudden death

    Mol Genet Metab

    (2011)
  • P. Teekakirikul et al.

    Inherited cardiomyopathies: molecular genetics and clinical genetic testing in the postgenomic era

    J Mol Diagn

    (2013)
  • L. Shan et al.

    SCN5A variants in Japanese patients with left ventricular noncompaction and arrhythmia

    Mol Genet Metab

    (2008)
  • J. Steffel et al.

    Electrocardiographic characteristics at initial diagnosis in patients with isolated left ventricular noncompaction

    Am J Cardiol

    (2009)
  • N.L. Bhatia et al.

    Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview

    J Card Fail

    (2011)
  • N. Derval et al.

    Apparent idiopathic ventricular tachycardia associated with isolated ventricular noncompaction

    Heart Rhythm

    (2009)
  • S. Barra et al.

    Incessant slow bundle branch reentrant ventricular tachycardia in a young patient with left ventricular noncompaction

    Rev Port Cardiol

    (2013)
  • C. Stöllberger et al.

    Atrial fibrillation in left ventricular noncompaction with and without neuromuscular disorders is associated with a poor prognosis

    Int J Cardiol

    (2009)
  • D. Ivan et al.

    Isolated ventricular non-compaction in adults with idiopathic cardiomyopathy: cardiac magnetic resonance and pathologic characterization of the anomaly

    J Heart Lung Transplant

    (2005)
  • R. Kobza et al.

    Implantable cardioverter-defibrillator and cardiac resynchronization therapy in patients with left ventricular noncompaction

    Heart Rhythm

    (2010)
  • R. Amadieu et al.

    Implantable cardioverter defibrillator in a young child with left ventricular noncompaction

    Arch Cardiovasc Dis

    (2011)
  • K. Okubo et al.

    Cardiac resynchronization and cardioverter defibrillation therapy in a patient with isolated noncompaction of the ventricular myocardium

    Int J Cardiol

    (2009)
  • S. Kubota et al.

    Cardiac resynchronization therapy in a patient with isolated noncompaction of the left ventricle and a narrow QRS complex

    Heart Rhythm

    (2006)
  • R.M. Freedom et al.

    Congenital absence of the pulmonary valve associated with imperforate membrane type of tricuspid atresia, right ventricular tensor apparatus and intact ventricular septum: a curious developmental complex

    Eur J Cardiol

    (1979)
  • F. Ichida et al.

    Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome

    Circulation

    (2001)
  • T.K. Chin et al.

    Isolated noncompaction of left ventricular myocardium. A study of eight cases

    Circulation

    (1990)
  • R.H. Pignatelli et al.

    Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy

    Circulation

    (2003)
  • R. Jenni et al.

    Isolated noncompaction of the myocardium

    N Engl J Med

    (1999)
  • S. Hook et al.

    Isolated noncompaction of the ventricular myocardium

    Pediatr Cardiol

    (1996)
  • E. Oechslin et al.

    Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity?

    Eur Heart J

    (2011)
  • A.W. Nugent et al.

    National Australian Childhood Cardiomyopathy Study. Clinical features and outcomes of childhood hypertrophic cardiomyopathy: results from a national population-based study

    Circulation

    (2005)
  • J. Steffel et al.

    Rhythm disorders in isolated left ventricular noncompaction

    Ann Med

    (2012)
  • S.T. Brescia et al.

    Mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center

    Circulation

    (2013)
  • B.C. Weiford et al.

    Noncompaction of the ventricular myocardium

    Circulation

    (2004)
  • C. Lofiego et al.

    Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction

    Heart

    (2007)
  • R.T. Murphy et al.

    Natural history and familial characteristics of isolated left ventricular non-compaction

    Eur Heart J

    (2005)
  • M.C. Digilio et al.

    Genetic heterogeneity of isolated noncompaction of the left ventricular myocardium

    Am J Med Genet

    (1999)

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