Electrocardiographic findings at initial diagnosis in children with isolated left ventricular noncompaction

Abstract

Background: The aim of this study was to comprehensively evaluate electrocardiographic (ECG) findings of isolated left ventricular noncompaction (IVNC) patients at initial diagnosis and to explore the correlation between them and the clinical, echocardiographic, and magnetic resonance imaging (MRI) findings.

Methods: Twenty-three patients diagnosed with IVNC by echocardiography and cardiac MRI between January 2006 and June 2010 were enrolled in this study. The patients were examined with standard ECG and 24-hour Holter ECG. For comparison purpose, ECGs of 50 healthy children of similar ages and demographic characteristics were taken.

Results: In 87% of patients, ECG abnormalities were found. The most frequently seen ECG findings were left ventricular hypertrophy, ST-segment depression, and negative T wave related to abnormal repolarization particularly in DII, DIII, and V(4-6) leads, as well as prolonged PR and QTc intervals. No ECG features or patterns were found that were specific to the disease. In contrast to adult patients, while no intraventricular conduction defects (particularly in the left bundle brach) were found in any of our patients, 13% had considerable bradycardia and one required a pacemaker. The Holter ECG recordings showed supraventricular tachycardia attacks in two patients and a short ventricular tachycardia attack in one. Patients whose echocardiograms and MRI showed left ventricular systolic dysfunction and left ventricular dilatation had signs of left ventricular hypertrophy and repolarization abnormality on their ECGs, but there was no significant difference in PR, QRS, and QTc intervals.

Conclusion: Regardless of how frequently left ventricular hypertrophy and repolarization abnormalities are found on IVNC patients' initial ECGs, we think that they are not unique to the disease but are related to the severity of the cardiomyopathy.

Figure 1

Noncompacted myocardium characterized by a pronounced increase in trabeculation in the left ventricle apex and the free wall on 2D echocardiography, and flow in deep recesses shown by color Doppler ultrasound.

Figure 2

ECG of an 8‐day‐old newborn admitted with dyspnea and cyanosis and diagnosed with IVNC by echocardiography. Significant left ventricular hypertrophy was seen.

Figure 3

The most frequent ECG findings of IVNC patients at the time of admission. (*P < 0.05; **P < 0.01; ***P < 0.001).

Figure 4

Initial ECG of a 2 7/12 y.o. male patient who was admitted with bradycardia and sinus node disfunction, diagnosed with IVNC, and implanted with a permanent pacemaker due to left ventricular dysfunction.