Is left ventricular noncompaction in children truly an isolated lesion?

Shane F. Tsai, Eric S. Ebenroth, Roger A. Hurwitz, Timothy M. Cordes, Marcus S. Schamberger, Anjan S. Batra

Research output: Contribution to journalArticle

34 Citations (Scopus)

Abstract

Left ventricular noncompaction (LVNC) is a form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis. It has been associated with significant morbidity and mortality. The aim of this study was to characterize associated cardiac findings in children with LVNC and to identify risk factors associated with increased mortality. From our echocardiography database, we identified 46 patients diagnosed with LVNC between December 1999 and February 2005. The mean age at presentation was 3.6 ± 5.6 years, and the mean duration of follow-up was 1.9 ± 2.1 years. Left ventricular ejection fraction was decreased in 24 patients (52%; mean 39.5% ± 13.1%). Thirty-six patients (78%) had associated cardiac lesions, including atrial septal defect (n = 16 [35%]), ventricular septal defect (n = 17 [37%]), patent ductus arteriosus (n = 14 [30%]), and Ebstein's anomaly (n = 5 [11%]). Electrocardiogram abnormalities were found in 80% of patients; most commonly they included left (n = 15 [43%]) and right ventricular hypertrophy (n = 19 [54%]). Documented arrhythmias included ectopic atrial rhythm (n = 2), junctional rhythm (n = 2), supraventricular tachycardia (n = 2), and ventricular tachycardia (n = 1). Overall mortality was 20%, and there was no association with ejection fraction, morphologic defect, or arrhythmia. Mean age at diagnosis in survivors (4.5 ± 6.1 years) was higher than nonsurvivors (0.4 ± 0.7 years) (p < 0.0001). LVNC is a rarely isolated form of cardiomyopathy, and it is associated with significant additional cardiac abnormalities. Although it does not have an invariably fatal course, early presentation in infancy does carry an increased risk of mortality.

Original languageEnglish (US)
Pages (from-to)597-602
Number of pages6
JournalPediatric Cardiology
Volume30
Issue number5
DOIs
StatePublished - Jul 1 2009

Fingerprint

Mortality
Cardiomyopathies
Cardiac Arrhythmias
Ebstein Anomaly
Right Ventricular Hypertrophy
Supraventricular Tachycardia
Patent Ductus Arteriosus
Atrial Heart Septal Defects
Ventricular Heart Septal Defects
Ventricular Tachycardia
Morphogenesis
Stroke Volume
Survivors
Echocardiography
Electrocardiography
Databases
Morbidity

Keywords

  • Cardiomyopathy
  • Noncompaction

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Cite this

Tsai, S. F., Ebenroth, E. S., Hurwitz, R. A., Cordes, T. M., Schamberger, M. S., & Batra, A. S. (2009). Is left ventricular noncompaction in children truly an isolated lesion? Pediatric Cardiology, 30(5), 597-602. https://doi.org/10.1007/s00246-008-9382-1

Is left ventricular noncompaction in children truly an isolated lesion? / Tsai, Shane F.; Ebenroth, Eric S.; Hurwitz, Roger A.; Cordes, Timothy M.; Schamberger, Marcus S.; Batra, Anjan S.

In: Pediatric Cardiology, Vol. 30, No. 5, 01.07.2009, p. 597-602.

Research output: Contribution to journalArticle

Tsai, SF, Ebenroth, ES, Hurwitz, RA, Cordes, TM, Schamberger, MS & Batra, AS 2009, 'Is left ventricular noncompaction in children truly an isolated lesion?', Pediatric Cardiology, vol. 30, no. 5, pp. 597-602. https://doi.org/10.1007/s00246-008-9382-1
Tsai SF, Ebenroth ES, Hurwitz RA, Cordes TM, Schamberger MS, Batra AS. Is left ventricular noncompaction in children truly an isolated lesion? Pediatric Cardiology. 2009 Jul 1;30(5):597-602. https://doi.org/10.1007/s00246-008-9382-1
Tsai, Shane F. ; Ebenroth, Eric S. ; Hurwitz, Roger A. ; Cordes, Timothy M. ; Schamberger, Marcus S. ; Batra, Anjan S. / Is left ventricular noncompaction in children truly an isolated lesion?. In: Pediatric Cardiology. 2009 ; Vol. 30, No. 5. pp. 597-602.
@article{45af9d213be74e35b2ceafe30208351a,
title = "Is left ventricular noncompaction in children truly an isolated lesion?",
abstract = "Left ventricular noncompaction (LVNC) is a form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis. It has been associated with significant morbidity and mortality. The aim of this study was to characterize associated cardiac findings in children with LVNC and to identify risk factors associated with increased mortality. From our echocardiography database, we identified 46 patients diagnosed with LVNC between December 1999 and February 2005. The mean age at presentation was 3.6 ± 5.6 years, and the mean duration of follow-up was 1.9 ± 2.1 years. Left ventricular ejection fraction was decreased in 24 patients (52{\%}; mean 39.5{\%} ± 13.1{\%}). Thirty-six patients (78{\%}) had associated cardiac lesions, including atrial septal defect (n = 16 [35{\%}]), ventricular septal defect (n = 17 [37{\%}]), patent ductus arteriosus (n = 14 [30{\%}]), and Ebstein's anomaly (n = 5 [11{\%}]). Electrocardiogram abnormalities were found in 80{\%} of patients; most commonly they included left (n = 15 [43{\%}]) and right ventricular hypertrophy (n = 19 [54{\%}]). Documented arrhythmias included ectopic atrial rhythm (n = 2), junctional rhythm (n = 2), supraventricular tachycardia (n = 2), and ventricular tachycardia (n = 1). Overall mortality was 20{\%}, and there was no association with ejection fraction, morphologic defect, or arrhythmia. Mean age at diagnosis in survivors (4.5 ± 6.1 years) was higher than nonsurvivors (0.4 ± 0.7 years) (p < 0.0001). LVNC is a rarely isolated form of cardiomyopathy, and it is associated with significant additional cardiac abnormalities. Although it does not have an invariably fatal course, early presentation in infancy does carry an increased risk of mortality.",
keywords = "Cardiomyopathy, Noncompaction",
author = "Tsai, {Shane F.} and Ebenroth, {Eric S.} and Hurwitz, {Roger A.} and Cordes, {Timothy M.} and Schamberger, {Marcus S.} and Batra, {Anjan S.}",
year = "2009",
month = "7",
day = "1",
doi = "10.1007/s00246-008-9382-1",
language = "English (US)",
volume = "30",
pages = "597--602",
journal = "Pediatric Cardiology",
issn = "0172-0643",
publisher = "Springer New York",
number = "5",

}

TY - JOUR

T1 - Is left ventricular noncompaction in children truly an isolated lesion?

AU - Tsai, Shane F.

AU - Ebenroth, Eric S.

AU - Hurwitz, Roger A.

AU - Cordes, Timothy M.

AU - Schamberger, Marcus S.

AU - Batra, Anjan S.

PY - 2009/7/1

Y1 - 2009/7/1

N2 - Left ventricular noncompaction (LVNC) is a form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis. It has been associated with significant morbidity and mortality. The aim of this study was to characterize associated cardiac findings in children with LVNC and to identify risk factors associated with increased mortality. From our echocardiography database, we identified 46 patients diagnosed with LVNC between December 1999 and February 2005. The mean age at presentation was 3.6 ± 5.6 years, and the mean duration of follow-up was 1.9 ± 2.1 years. Left ventricular ejection fraction was decreased in 24 patients (52%; mean 39.5% ± 13.1%). Thirty-six patients (78%) had associated cardiac lesions, including atrial septal defect (n = 16 [35%]), ventricular septal defect (n = 17 [37%]), patent ductus arteriosus (n = 14 [30%]), and Ebstein's anomaly (n = 5 [11%]). Electrocardiogram abnormalities were found in 80% of patients; most commonly they included left (n = 15 [43%]) and right ventricular hypertrophy (n = 19 [54%]). Documented arrhythmias included ectopic atrial rhythm (n = 2), junctional rhythm (n = 2), supraventricular tachycardia (n = 2), and ventricular tachycardia (n = 1). Overall mortality was 20%, and there was no association with ejection fraction, morphologic defect, or arrhythmia. Mean age at diagnosis in survivors (4.5 ± 6.1 years) was higher than nonsurvivors (0.4 ± 0.7 years) (p < 0.0001). LVNC is a rarely isolated form of cardiomyopathy, and it is associated with significant additional cardiac abnormalities. Although it does not have an invariably fatal course, early presentation in infancy does carry an increased risk of mortality.

AB - Left ventricular noncompaction (LVNC) is a form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis. It has been associated with significant morbidity and mortality. The aim of this study was to characterize associated cardiac findings in children with LVNC and to identify risk factors associated with increased mortality. From our echocardiography database, we identified 46 patients diagnosed with LVNC between December 1999 and February 2005. The mean age at presentation was 3.6 ± 5.6 years, and the mean duration of follow-up was 1.9 ± 2.1 years. Left ventricular ejection fraction was decreased in 24 patients (52%; mean 39.5% ± 13.1%). Thirty-six patients (78%) had associated cardiac lesions, including atrial septal defect (n = 16 [35%]), ventricular septal defect (n = 17 [37%]), patent ductus arteriosus (n = 14 [30%]), and Ebstein's anomaly (n = 5 [11%]). Electrocardiogram abnormalities were found in 80% of patients; most commonly they included left (n = 15 [43%]) and right ventricular hypertrophy (n = 19 [54%]). Documented arrhythmias included ectopic atrial rhythm (n = 2), junctional rhythm (n = 2), supraventricular tachycardia (n = 2), and ventricular tachycardia (n = 1). Overall mortality was 20%, and there was no association with ejection fraction, morphologic defect, or arrhythmia. Mean age at diagnosis in survivors (4.5 ± 6.1 years) was higher than nonsurvivors (0.4 ± 0.7 years) (p < 0.0001). LVNC is a rarely isolated form of cardiomyopathy, and it is associated with significant additional cardiac abnormalities. Although it does not have an invariably fatal course, early presentation in infancy does carry an increased risk of mortality.

KW - Cardiomyopathy

KW - Noncompaction

UR - http://www.scopus.com/inward/record.url?scp=67449104718&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=67449104718&partnerID=8YFLogxK

U2 - 10.1007/s00246-008-9382-1

DO - 10.1007/s00246-008-9382-1

M3 - Article

C2 - 19184169

AN - SCOPUS:67449104718

VL - 30

SP - 597

EP - 602

JO - Pediatric Cardiology

JF - Pediatric Cardiology

SN - 0172-0643

IS - 5

ER -