Dilate kardiyomyopatili çocuk hastalarda klinik ve prognostik faktörler

Öz

Amaç: Dilate kardiyomiyopati, sol ventrikül kontraktilitesinde bozulma ve kardiyak dilatasyon ile seyreden bir hastalıktır. Tanısal ilerlemelere rağmen çocuklardaki çoğu vaka idiyopatiktir. Çalışmamızda dilate kardiyomiyopatili çocuk hastalarda prognozu etkileyen faktörleri değerlendirmeyi amaçladık. Yöntemler: Dilate kardiyomiyopati tanısı konulan 59 hasta, Ocak 2005-Ocak 2013 tarihleri arasında retrospektif olarak incelendi. Prognoz açısından; hastalarda semptomların başlama yaşı, hastaların cinsiyeti, anne-baba akrabalığı, elektrokardiyografik bulgu ve telekardiyogramda kardiyomegali varlığı, ekokardiyografide ejeksiyon fraksiyonu ve kısalma fraksiyonu değerlendirildi. Bulgular: Hastalarımızın ortalama yaşları 49.2±59.2 ay olup, erkek/kız oranı 1.03/1 idi. Hastalar en sık kalp yetersizliği semptom ve klinik bulguları ile başvurmuştu. En sık elektrokardiyografi bulgusu sol ventrikül hipertrofisi (%32.2) idi ve telekardiyografide hastaların %77.8’inde kardiyomegali saptandı. Ekokardiyografik incelemede; hastaların sol ventrikül ejeksiyon ve kısalma fraksiyonları tanı anında %38.4±14.5 ve %18.8±8.4 olup, takip süresi sonunda ise %51±18.6% ve %26.8±11.6 idi. Ortalama takip süresi 27.5±27.7 ay ve mortalite oranı %42.4 olarak bulundu. Prognozu etkileyen ana faktörler incelendiğinde; tanıda hastalarda elektrokardiyografik değişiklik ve kardiyomegali olması ve ejeksiyon fraksiyonlarının düşük olmasının sağkalımı etkilediği görüldü. Sonuç: Dilate kardiyomiyopati tedavisi zor bir hastalıkdır ve prognozu kötüdür. Tanı anında elektrokardiyografik bulgu ve telekardiyografide kardiyomegali olması ve EF değerlerinin düşük olması hastaların sağkalımını etkileyen en önemli belirteçlerdir.

Anahtar Kelimeler

• Dilate kardiyomiyopati
• Çocuk
• Prognostik faktörler

Clinical and prognostic factors in children with dilated cardiomyopathy

Abstract

Purpose: Dilated cardiomyopathy is characterized by impaired myocardial contractility and cardiac dilatation. Most cases in children are idiopathic despite diagnostic developments. In this study, we aimed to review children with dilated cardiomyopathy and attempt to determine the prognostic factors. Materials and Methods: A total of 59 children with a diagnosis of dilated cardiomyopathy were evaluated retrospectively between January 2005 to January 2013. The age of onset of symptoms, gender, parental consanguinity, electrocardiography findings, presence of cardiomegaly in the telecardiograpy, left ventricular ejection fraction and shortening fraction on prognosis were evaluated. Results: The mean age of patients was 49.2 ± 59.2 months and the male/female ratio was 1.03/1. Heart failure symptoms and signs were the most common cause of admission. The most common electrocardiographic finding was left ventricular hypertrophy in 32.2% and cardiomegaly was observed on telecardiography in 77.8%. The left ventricular ejection and shortening fraction of patients’ were 38.4± 14.5% and 18.8± 8.4% at the time of the diagnosis and 51± 18.6% and 26.8± 11.6% at the end of the follow-up, respectively. The mean follow-up period was 27.5± 27.7 months and the mortality rate was 42.4%. The main factors affecting prognosis have been found as detection of electrocardiographic changes, cardiomegaly, and lower left ventricular ejection fraction at the time of the diagnosis. Conclusion: Dilated cardiomyopathy remains a challenging disease and the prognosis is poor. In our study, the most important prognostic markers affecting survival were determined by the presence of electrocardiographic changes, cardiomegaly, and a reduced ejection fraction.

Keywords

• Children
• dilated cardiomyopathy
• prognostic factors

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