Yenidoğan izole konjenital kalp bloklarında pacemaker implantasyonu: Tek merkez deneyimi

Amaç: Yenidoğan döneminde izole konjenital tam kalp bloğu oldukça nadir görülen bir hastalıktır. Genellikle anne kanında altta yatan immun sistem hastalıklarına bağlı gelişen otoantikorların plasenta yoluyla geçişi ile bebeğin kalp ileti sisteminde fibrozis ile sonuçlanmasının neden olduğu bilinmektedir. Erken dönemde tanı konulmayan ve tedavi edilmeyen hastalarda yüksek oranda ani bebek ölümü ve ciddi morbidite ile kendini gösterir. Bu çalışmadaki amacımız kliniğimizin konu ile ilgili tecrübelerini aktarmaktır.Gereç ve Yöntemler: Çalışmada 2011-2017 yılları arasında Dr. Sami Ulus Kadın Doğum Çocuk Sağlığı ve Hastalıkları Eğitim ve Araştırma Hastanesi Kalp ve Damar Cerrahisi Kliniğinde izole kalp bloğu nedeniyle cerrahi olarak epikardiyal kalp pili implante edilen 9 hasta (1 erkek, 8 kız) retrospektif olarak incelendi. Bulgular: Altı hastada (%67) kalp bloğu tanısı intrauterin hayatta iken yapılan fetal ekokardiyografi incelemesi ile kondu. 3 (%33) hastamızda ise doğumdan sonra ortaya çıkan bradikardi sonrası çekilen elektrokardiyografiler (EKG) ve transtorasik ekokardiyografi( TTE) incelemeleri ile tanı konuldu. Hastaların 8 (%89)’inde annede otoimmün bir hastalık bulunmaktaydı. Bunların %62’si Sistemik Lupus Eritamatozus (SLE) (n=5), %38’i Sjögren Sendromu (n=3) tanıları ile takip edilmekteydi. Tüm hastalara epikardiyal kalp pili implante edildi. Bir hastada postoperatif 2. ayda sepsis nedeniyle mortalite görüldü. Diğer hastaların takipleri sorunsuz olarak devam etmektedir.Sonuç: Yenidoğan dönemi tam kalp bloğu yüksek mortalite ve morbidite ile seyreden bir durumdur. Artan tecrübe ile birlikte fetal ekokardiyografi prenatal dönemde birçok konjenital kalp hastalığının tanısının konulmasını sağlamaktadır. Epikardiyal kalp pili implantasyonu mortalite ve morbiditede belirgin derecede azalma sağlayan tek tedavi yöntemidir.

Anahtar Kelimeler:

yenidoğan, kalp bloğu, kalp pili

Pacemaker implantation in newborn isolated congenital heart blocks: A single center experience

Aim: isolated congenital complete heart block in the neonatal period is a very rare disease. Generally, fibrosis in the conduction system of newborn caused by antibodies of mother with autoimmune disease is responsible for this complete heart block. This situation is related with high risk of sudden infant death and serious morbidities in undiagnosed and untreated newborns. In this study we aim to share our experience about these patients.Material and Methods: 9 patients (1 male and 8 female) whom implanted epicardial pacemaker due to isolated complete heart block were retrospectively evaluated in Dr. Sami Ulus Maternity and Child Health Research and Training Hospital Cardiovascular Surgery clinic between 2011-2017were evaluated retrospectively in this study.Results: Disease was diagnosed in 6 (67%) patients during intrauterine period with fetal echocardiography and in 3 (33%) patients diagnosis was achieved with electrocardiograpy and transthroacic echocardiography after bradycardia was seen. There were autoimmune disorders in mothers of 8 (89%) infants. 62% of them were already had Systemic Lupus Erythematosus (SLE) (n=5) and 38% of them had sjören's syndrome (n= 3). Epicardial pacemaker implantation was performed in all of our patients. One postoperative mortality was seen due to sepsis at 2nd month. Other patients' early and midterm follow-ups have been favorable. Conclusion: Isolated heart block in newborn has high mortality and morbidity rates. After improvements in technology fetal echocardiography can help us to diagnose a lot of congenital heart disease. Epicardial pacemaker implantation is the only treatment option which can decrease mortality and morbidity rates.

Keywords:

newborn, heart block, pacemaker,

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