Kardiyomiyopati tanısı ile takip edilen çocuk hastaların klinik özellikleri- Tek merkez deneyimi

Abstract

Amaç:Kardiyomiyopatiler, ventriküler miyokardın yapısal ve fonksiyonel anormallikleri olarak tanımlanır. Kardiyomiyopatiler çocuklarda nadir görülür. Bu çalışmada hastanemizde 2 yıllık süreçte takip ettiğimiz kardiyomiyopati tanısı alan hastaların verileri incelenerek sonuçları değerlendirilmiştir. Yöntem:2020-2022 Temmuz tarihleri arasında Mersin Üniversitesi Tıp Fakültesi Hastanesi Çocuk Kardiyoloji bölümünde kardiyomiyopati tanısı alan hastaların verileri geriye dönük olarak incelendi. Bulgular:2 yıllık süreçte 17 hastada kardiyomiyopati tespit edildi. Hastaların tanı esnasındaki yaş ortancası 5 yıl ( 2 ay- 14 yıl) idi. 5 hasta kız , geri kalan 12 hasta erkek idi. 10 hasta (%58.8) dilate kardiyomiyopati (DKMP) tanısı alırken, 5 hasta (%29.4) hipertrofik kardiyomiyopati (HKMP), 2 hasta (%11.7) ise restriktif kardiyomiyopati(RKMP) tanısı aldı. DKMP tanısı alan hastalarda, viral miyokardite sekonder DKMP, nörometabolik hastalıklar, sol koroner arterin pulmoner arterden anormal çıkışı ve taşikardiyomiyopati etiyolojik nedenler arasında yer almaktaydı. Bir hastada Friedrich ataksisine sekonder HKMP ve kronik miyokardit görülürken, üç hastada ise Pompe hastalığı ve HKMP tespit edildi. RKMP tanısı alan iki hastada ise şu ana kadar spesifk bir etiyolojik faktör bulunamadı. İzlemde DKMP olan hastalardan üç hasta ex oldu. DKMP olan bir hastaya kalp transplantasyonu yapıldı. Tartışma:Kardiyomiyopatiler çocuklarda önemli bir morbidite ve mortalite nedenidir. Etiyolojide nörometabolik ve genetik faktörlere, koroner arter hastalıklarına ve aritmilere dikkat edilmelidir. Erken tanı ve tedavi sağkalımda önemlidir.

Keywords

• Çocuk
• kalp yetmezliği
• kardiyomiyopati
• genetik
• nörometabolik
• hastalıklar

Clinical findings of pediatric patients with cardiomyopathy – A single center experience

Abstract

Aim: Cardiomyopathies are defined as structural and functional abnormalities of the ventricular myocardium. Cardiomyopathies are rare in children. In this study, the data of patients diagnosed with cardiomyopathy that we followed in our hospital for a 2-year period were examined and their results were evaluated. Method: The data of patients diagnosed with cardiomyopathy between July 2020 and 2022 in the pediatric cardiology department were examined retrospectively. Results: Cardiomyopathy was detected in 17 patients during the 2-year period. The median age of the patients at diagnosis was 5 years (2 months - 14 years). 5 patients were female and the remaining 12 patients were male. While 10 patients (58.8%) were diagnosed with dilated cardiomyopathy (DCMP), 5 patients (29.4%) were diagnosed with hypertrophic cardiomyopathy(HCMP), and 2 patients (11.7%) were diagnosed with restrictive cardiomyopathy(RCMP). In patients with DCMP, viral myocarditis, neurometabolic diseases, abnormal origin of the left coronary artery from the pulmonary artery, and tachycardiomyopathy were among the etiological causes. HCMP and chronic myocarditis secondary to Friedrich's ataxia were observed in one patient, while Pompe disease was detected in 3 patients with HCMP. No specific etiological factor has been found so far in two patients diagnosed with RCMP. During the follow-up, 3 patients with DCMP died. A patient with DKMP underwent heart transplantation. Conclusion: Cardiomyopathies are an important cause of morbidity and mortality in children. In etiology, attention should be paid to neurometabolic and genetic factors, coronary artery diseases and arrhythmias. Early diagnosis and treatment are important for survival.

Keywords

• Cardiomyopathy
• childhood
• heart failure
• genetic
• neurometabolic diseases.

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