Yenidoğan izole konjenital kalp bloklarında pacemaker implantasyonu: Tek merkez deneyimi

Year 2018, , 227 - 232, 30.09.2018

Murat Koç

https://doi.org/10.18663/tjcl.454171

Abstract

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.

 

Keywords

yenidoğan, kalp bloğu, kalp pili

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

Abstract

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

References

• 1- Michaëlsson M, Engle MA. Congenital complete heart block: an international study of the natural history. Cardiovasc Clin 1972; 4: 85–101
• 2- Brito-Zerón P, Izmirly PM, Ramos-Casals M, Buyon JP, Khamashta MA. The clinical spectrum of autoimmune congenital heart block. Nat Rev Rheumatol 2015; 11: 301–12
• 3- Capone C, Buyon JP, Friedman DM, Frishman WH. Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population. Cardiol Rev 2011; 20: 72–76
• 4- Baruteau AE, Fouchard S, Behaghel A et al. Characteristics and long-term outcome of non-immune isolated atrioventricular block diagnosed in utero or early childhood: a multicentre study. Eur Heart J 2012; 33: 622–29
• 5- Brito-Zerón P, Izmirly PM, Ramos-Casals M, Buyon JP, Khamashta MA Autoimmune congenital heart block: complex and unusual situations. Lupus 2016; 25: 116–28
• 6- Donofrio MT, Moon-Grady AJ, Hornerger LK, et al. Diagnosis and Treatment of Fetal Cardiac Disease: A Scientific Statement From the American Heart Association. Circulation 2014; 129: 2183–242
• 7- Buyon JP, Hiebert R, Copel J et al. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 1998; 31: 1658–66
• 8- Assad RS, Zielinsky P, Kalil R et al. New lead for in utero pacing for fetal congenital heart block. J Thorac Cardiovasc Surg 2003; 126: 300–2
• 9- Vest AN, Zhou L, Huang X et al. Design and Testing of a Transcutaneous RF Recharging System for a Fetal Micropacemaker. IEEE Trans Biomed Circuits Syst 2017; 11: 336–46
• 10- Eronen M, Sirèn MK, Ekblad H, Tikanoja T, Julkunen H, Paavilainen T. Short-and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn. Pediatrics 2000; 106: 86-91.
• 11- Epstein AE, Dimarco JP, Ellenbogen KA et al. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. Journal of the American College of Cardiology, 2008; 51: 1-62.
• 12- Kubus P, Materna O, Gebauer RA et al. Permanent epicardial pacing in children: long-term results and factors modifying outcome. Europace 2012; 14: 509–14
• 13- Silvetti MS, Drago F, De Santis A et al. Single-centre experience on endocardial and epicardial pacemaker system function in neonates and infants. Europace 2007; 9: 426–31
• 14- Moak JP, Hasbani K, Ramwell C et al. Dilated Cardiomyopathy Following Right Ventricular Pacing for AV Block in Young Patients: Resolution After Upgrading to Biventricular Pacing Systems. J. Cardiovasc. Electrophysiol 2006; 17: 1068–71
• 15- Karpawich PP, Singh H, Zelin K. Optimizing paced ventricular function in patients with and without repaired congenital heart disease by contractility-guided lead implant. Pacing Clin. Electrophysiol 2015; 38: 54–62