ISSN 1016-5169 | E-ISSN 1308-4488
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Effects of Systolic Dysfunction on Clinical and Diagnostic Parameters in Pediatric Patients with Isolated Left Ventricular Non-compaction [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2023; 51(5): 333-342 | DOI: 10.5543/tkda.2023.09648

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

Fatma Sevinç Şengül1, Pelin Ayyıldız1, Sezen Ugan Atik1, Sinem Aydin2, Alper Güzeltaş1, Yakup Ergül1
1Department of Pediatric Cardiology, University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Türkiye
2Department of Radiology, University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Türkiye


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


METHODS
We retrospectively reviewed children with isolated left ventricular non-compaction at Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital 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 (1 month-17.9 years). The median follow-up of the study was 19 months (1-121 months). Fourteen (25.5%) presented with systolic dysfunction (ejection fraction < 45%), and 2 presented with resuscitated/aborted cardiac arrest. Electrocardiographic abnormalities were present in 78.2%. Fragmented QRS was observed in 6 patients, and QTc duration was 450 milliseconds and above in 17 patients (30.9%). Electrocardiographic abnormalities, low QRS voltage, fragmented QRS, and thrombus were common in patients with ejection fraction < 45% group. Atrial and ventricular arrhythmias (including ventricular fibrillation-VF) were found with similar frequency in both ejection fraction < 45% and ≥45% groups. One patient with a complete atrioventricular block and 1 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. 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, even in patients with preserved ejection fraction.

Keywords: Arrhythmias, cardiomyopathies, children, left ventricular non-compaction

How to cite this article
Fatma Sevinç Şengül, Pelin Ayyıldız, Sezen Ugan Atik, Sinem Aydin, Alper Güzeltaş, Yakup Ergül. Effects of Systolic Dysfunction on Clinical and Diagnostic Parameters in Pediatric Patients with Isolated Left Ventricular Non-compaction. Turk Kardiyol Dern Ars. 2023; 51(5): 333-342

Corresponding Author: Yakup Ergül, Türkiye
Manuscript Language: English


Journal Metrics

Journal Citation Indicator: 0.18
CiteScore: 1.1
Source Normalized Impact
per Paper:
0.22
SCImago Journal Rank: 0.348

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