Effects of Systolic Dysfunction on Clinical and Diagnostic Parameters in Pediatric Patients with Isolated Left Ventricular Non-compaction İzole Sol Ventrikül Non-Kompaksiyon Tanılı Pediatrik Hastalarda Sistolik Disfonksiyonun Klinik ve Tanısal Parametreler Üzerindeki Etkileri
Turk Kardiyoloji Dernegi Arsivi, cilt.51, sa.5, ss.333-342, 2023 (ESCI, Scopus, TRDizin)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 51 Sayı: 5
- Basım Tarihi: 2023
- Doi Numarası: 10.5543/tkda.2023.09648
- Dergi Adı: Turk Kardiyoloji Dernegi Arsivi
- Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus, Central & Eastern European Academic Source (CEEAS), Directory of Open Access Journals, TR DİZİN (ULAKBİM)
- Sayfa Sayıları: ss.333-342
- Anahtar Kelimeler: Arrhythmias, cardiomyopathies, children, left ventricular non-compaction
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
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.