Effects of Systolic Dysfunction on Clinical and Diagnostic Parameters in Pediatric Patients with Isolated Left Ventricular Non-Compaction

OBJECTIVES
Left ventricular non-compaction is rare cardiomyopathy following an early arrest in endomyocardial morphogenesis. This study aimed to present the clinical and electrocardiographic (ECG) characteristics, diagnostic features, treatment strategies, effects of systolic dysfunction on clinical and diagnostic parameters, and follow-up of pediatric patients diagnosed with LVNC.


METHODS
We retrospectively reviewed children with isolated left ventricular non-compaction from January 2010 to June 2020.


RESULTS
Fifty-five children were diagnosed with left ventricular non-compaction. Thirty-two patients (58.2%) were male, and the median age of presentation was 8.5 years (1month-17.9years). The median follow-up of the study was 19months (1-121months). Fourteen (25.5%) presented with cardiac dysfunction (EF<45%), and two presented with resuscitated/aborted cardiac arrest. ECG abnormalities were present in 78.2%. Fragmented QRS (fQRS) was observed in the ECGs of six patients, and the QTc duration was 450ms and above in 17 patients (30.9%). ECG abnormalities, low QRS voltage, fQRS, and thrombus were common in patients with EF<45% group. Atrial and ventricular arrhythmias (including VF) were found with similar frequency in both EF<45% and ≥45% groups. One patient with a complete AV block and one with Long QT syndrome and severe bradycardia underwent permanent pacemaker implantation. Five (9.1%) patients died.


CONCLUSIONS
Left ventricular non-compaction has heterogeneous clinical findings in childhood. Congestive heart failure clinic may develop in patients during follow-up. It is essential to follow-up with the patients closely for the development of ventricular dysfunction or arrhythmias due to the progressive course of the disease. Further studies are needed since life-threatening ventricular arrhythmias can be seen, also in patients with preserved EF.