Isolated noncompaction cardiomyopathy presenting with paroxysmal supraventricular tachycardia: Case report and literature review

Ijaz A. Khan, William P. Biddle, Syed A. Najeed, Syed Abdul-Aziz, Nirav J. Mehta, Vikrant Salaria, Annette L. Murcek, David M. Harris

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

Isolated noncompaction cardiomyopathy is an exceedingly rare congenital cardiomyopathy. A case of isolated noncompaction cardiomyopathy is reported and the literature on the subject collected through a comprehensive literature search is reviewed. Fewer than 100 cases of this condition have been reported. Isolated noncompaction cardiomyopathy is caused by a defect in cardiac morphogenesis resulting in an arrest of compaction of loose interwoven meshwork of myocardial fibers during intrauterine life, which results in severe systolic dysfunction as well as undue hypertrophy of the involved walls of the ventricles. Although the most frequent sites involved are left ventricular apex and inferior wall, involvement of other left ventricular walls and right ventricle has also been reported. Etiology of the isolated noncompaction of myocardium is not clear. Familial cases have been reported and the mode of inheritance is heterogeneous. In X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene. The age of onset of symptoms ranges from infancy to the geriatric age. Patients with isolated noncompaction cardiomyopathy have a high incidence of heart failure, arrhythmias, and thromboembolism. The most common presentation is congestive heart failure. Arrhythmias include atrial arrhythmias, ventricular tachycardia, and sudden cardiac death. The patient reported in this article presented with paroxysmal supraventricular tachycardia. Echocardiography is the procedure of choice to establish diagnosis. Due to the lack of associated cardiac anomalies, antenatal detection is difficult. The treatment is that for congestive heart failure, arrhythmias, and thromboembolism. The end-stage congestive heart failure is managed with heart transplantation and potential life-threatening ventricular tachyarrhythmias with an implantable cardioverter defibrillator. Prognosis is poor and the common causes of death are intractable heart failure and sudden cardiac death.

Original languageEnglish (US)
Pages (from-to)243-250
Number of pages8
JournalAngiology
Volume54
Issue number2
DOIs
StatePublished - Jan 1 2003

Fingerprint

Paroxysmal Tachycardia
Supraventricular Tachycardia
Cardiomyopathies
Heart Failure
Cardiac Arrhythmias
Thromboembolism
Sudden Cardiac Death
Implantable Defibrillators
Heart Transplantation
Ventricular Tachycardia
Morphogenesis
Age of Onset
Tachycardia
Geriatrics
Hypertrophy
Heart Ventricles
Echocardiography
Cause of Death
Myocardium
Mutation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Isolated noncompaction cardiomyopathy presenting with paroxysmal supraventricular tachycardia : Case report and literature review. / Khan, Ijaz A.; Biddle, William P.; Najeed, Syed A.; Abdul-Aziz, Syed; Mehta, Nirav J.; Salaria, Vikrant; Murcek, Annette L.; Harris, David M.

In: Angiology, Vol. 54, No. 2, 01.01.2003, p. 243-250.

Research output: Contribution to journalArticle

Khan, Ijaz A. ; Biddle, William P. ; Najeed, Syed A. ; Abdul-Aziz, Syed ; Mehta, Nirav J. ; Salaria, Vikrant ; Murcek, Annette L. ; Harris, David M. / Isolated noncompaction cardiomyopathy presenting with paroxysmal supraventricular tachycardia : Case report and literature review. In: Angiology. 2003 ; Vol. 54, No. 2. pp. 243-250.
@article{aac35582e166421185ddc314511086e5,
title = "Isolated noncompaction cardiomyopathy presenting with paroxysmal supraventricular tachycardia: Case report and literature review",
abstract = "Isolated noncompaction cardiomyopathy is an exceedingly rare congenital cardiomyopathy. A case of isolated noncompaction cardiomyopathy is reported and the literature on the subject collected through a comprehensive literature search is reviewed. Fewer than 100 cases of this condition have been reported. Isolated noncompaction cardiomyopathy is caused by a defect in cardiac morphogenesis resulting in an arrest of compaction of loose interwoven meshwork of myocardial fibers during intrauterine life, which results in severe systolic dysfunction as well as undue hypertrophy of the involved walls of the ventricles. Although the most frequent sites involved are left ventricular apex and inferior wall, involvement of other left ventricular walls and right ventricle has also been reported. Etiology of the isolated noncompaction of myocardium is not clear. Familial cases have been reported and the mode of inheritance is heterogeneous. In X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene. The age of onset of symptoms ranges from infancy to the geriatric age. Patients with isolated noncompaction cardiomyopathy have a high incidence of heart failure, arrhythmias, and thromboembolism. The most common presentation is congestive heart failure. Arrhythmias include atrial arrhythmias, ventricular tachycardia, and sudden cardiac death. The patient reported in this article presented with paroxysmal supraventricular tachycardia. Echocardiography is the procedure of choice to establish diagnosis. Due to the lack of associated cardiac anomalies, antenatal detection is difficult. The treatment is that for congestive heart failure, arrhythmias, and thromboembolism. The end-stage congestive heart failure is managed with heart transplantation and potential life-threatening ventricular tachyarrhythmias with an implantable cardioverter defibrillator. Prognosis is poor and the common causes of death are intractable heart failure and sudden cardiac death.",
author = "Khan, {Ijaz A.} and Biddle, {William P.} and Najeed, {Syed A.} and Syed Abdul-Aziz and Mehta, {Nirav J.} and Vikrant Salaria and Murcek, {Annette L.} and Harris, {David M.}",
year = "2003",
month = "1",
day = "1",
doi = "10.1177/000331970305400216",
language = "English (US)",
volume = "54",
pages = "243--250",
journal = "Angiology",
issn = "0003-3197",
publisher = "SAGE Publications Inc.",
number = "2",

}

TY - JOUR

T1 - Isolated noncompaction cardiomyopathy presenting with paroxysmal supraventricular tachycardia

T2 - Case report and literature review

AU - Khan, Ijaz A.

AU - Biddle, William P.

AU - Najeed, Syed A.

AU - Abdul-Aziz, Syed

AU - Mehta, Nirav J.

AU - Salaria, Vikrant

AU - Murcek, Annette L.

AU - Harris, David M.

PY - 2003/1/1

Y1 - 2003/1/1

N2 - Isolated noncompaction cardiomyopathy is an exceedingly rare congenital cardiomyopathy. A case of isolated noncompaction cardiomyopathy is reported and the literature on the subject collected through a comprehensive literature search is reviewed. Fewer than 100 cases of this condition have been reported. Isolated noncompaction cardiomyopathy is caused by a defect in cardiac morphogenesis resulting in an arrest of compaction of loose interwoven meshwork of myocardial fibers during intrauterine life, which results in severe systolic dysfunction as well as undue hypertrophy of the involved walls of the ventricles. Although the most frequent sites involved are left ventricular apex and inferior wall, involvement of other left ventricular walls and right ventricle has also been reported. Etiology of the isolated noncompaction of myocardium is not clear. Familial cases have been reported and the mode of inheritance is heterogeneous. In X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene. The age of onset of symptoms ranges from infancy to the geriatric age. Patients with isolated noncompaction cardiomyopathy have a high incidence of heart failure, arrhythmias, and thromboembolism. The most common presentation is congestive heart failure. Arrhythmias include atrial arrhythmias, ventricular tachycardia, and sudden cardiac death. The patient reported in this article presented with paroxysmal supraventricular tachycardia. Echocardiography is the procedure of choice to establish diagnosis. Due to the lack of associated cardiac anomalies, antenatal detection is difficult. The treatment is that for congestive heart failure, arrhythmias, and thromboembolism. The end-stage congestive heart failure is managed with heart transplantation and potential life-threatening ventricular tachyarrhythmias with an implantable cardioverter defibrillator. Prognosis is poor and the common causes of death are intractable heart failure and sudden cardiac death.

AB - Isolated noncompaction cardiomyopathy is an exceedingly rare congenital cardiomyopathy. A case of isolated noncompaction cardiomyopathy is reported and the literature on the subject collected through a comprehensive literature search is reviewed. Fewer than 100 cases of this condition have been reported. Isolated noncompaction cardiomyopathy is caused by a defect in cardiac morphogenesis resulting in an arrest of compaction of loose interwoven meshwork of myocardial fibers during intrauterine life, which results in severe systolic dysfunction as well as undue hypertrophy of the involved walls of the ventricles. Although the most frequent sites involved are left ventricular apex and inferior wall, involvement of other left ventricular walls and right ventricle has also been reported. Etiology of the isolated noncompaction of myocardium is not clear. Familial cases have been reported and the mode of inheritance is heterogeneous. In X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene. The age of onset of symptoms ranges from infancy to the geriatric age. Patients with isolated noncompaction cardiomyopathy have a high incidence of heart failure, arrhythmias, and thromboembolism. The most common presentation is congestive heart failure. Arrhythmias include atrial arrhythmias, ventricular tachycardia, and sudden cardiac death. The patient reported in this article presented with paroxysmal supraventricular tachycardia. Echocardiography is the procedure of choice to establish diagnosis. Due to the lack of associated cardiac anomalies, antenatal detection is difficult. The treatment is that for congestive heart failure, arrhythmias, and thromboembolism. The end-stage congestive heart failure is managed with heart transplantation and potential life-threatening ventricular tachyarrhythmias with an implantable cardioverter defibrillator. Prognosis is poor and the common causes of death are intractable heart failure and sudden cardiac death.

UR - http://www.scopus.com/inward/record.url?scp=0037344259&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0037344259&partnerID=8YFLogxK

U2 - 10.1177/000331970305400216

DO - 10.1177/000331970305400216

M3 - Article

C2 - 12678202

AN - SCOPUS:0037344259

VL - 54

SP - 243

EP - 250

JO - Angiology

JF - Angiology

SN - 0003-3197

IS - 2

ER -