Evren SEMİZEL, Özlem Mehtap BOSTAN, Ergün ÇİL

Konjenital Tam Atriyoventriküler Bloklu Hastalarımızın Özellikleri

Tanımlanan süreler içerisinde konjenital A-V tam blok tanısı almış olan 12 hasta çalışmaya dahil edildi Tablo 1 . Hastaların yedisi erkek, beşi kız olup, tanı anındaki yaşları bir gün ila altı yaş arasında değişi- yordu ortalama 16 ay, ortanca 12 ay . Oniki hastanın yedisi bradikardi ve ritim problemi, dördü üfürüm duyulması, ve biri de senkop nedeni ile tarafımıza gönderilmişti. Bradikardisi olan yedi hastadan dör- dünde intrauterin bradikardi de saptanmıştı. Bu dört hastadan üçünde bradikardi kadın doğum uzmanı, birinde ise çocuk kardiyoloji uzmanı tarafından sap- tanmıştı. Ekokardiyografik incelemeleri gözden geçi- rildiğinde, hastalardan birinde cTGA, üçünde de pa- tent duktus arteriyosuz PDA olduğu görüldü. Altı hastanın annesinde spesifik anti Ro ve anti La anti- korunun pozitif olduğu ve bu altı hastadan birinde ayrıca neonatal lupus sendromunun diğer bulguları olan, diskoid döküntü, karaciğer fonksiyon bozuklu- ğu, ve sitopenin de olduğu saptandı. Neonatal lupus sendromu olan bu hastanın altı aylık izlem içerisinde şikayetlerinin ortadan kalktığı, ancak A-V tam blo- ğun devam ettiği görüldü. Oniki hastanın tanı anın- daki elektrokardiyografilerinin incelenmesi sonucu, bu hastaların tanı anındaki ortalama ventriküler ve atriyal hızlarının sırası ile 54 ± 11 atım/dk ve 116 ± 24 atım/dk olduğu bulundu Sekiz hastaya kalp pili tak›lm›flt›. Takip süresi 1 ay ila 11 yafl aras›nda de¤iflen hastalar›n son kontrollerindeki yafllar› 6 ay ila 13 yafl aras›ndayd›. Sonuç: Fetal hayatta bradiaritmisi saptanan ve/veya annelerinde anti-Ro/La antikoru pozitif olan fetüslerin tümüne seri ekokardiyografi ile takip yap›lmas› önerilir. Güncel Pediatri 2007; 5: 47-51

Anahtar Kelimeler:

Konjenital, tam atriyoventriküler blok

Konjenital Tam Atriyoventriküler Bloklu Hastalarımızın Özellikleri

SSUUMMAARRYY AAiimm:: Evaluation of the clinical features of the children with congenital complete atrioventricular block. Matteerriiaall aanndd Metthhooddss:: The hospital records of children who had been admitted to the Uludag University Pediatric Car- Maatteerriiaall aanndd Metthhooddss:: The hospital records of children who had been admitted to the Uludag University Pediatric Car- diology Unit between January 1994 and Nowember 2005 were reviewed retrospectively and a total of 12 patients who were diagnosed as congenital complete A-V block between this period were involved the study. Medical records of the- se patients were reviewed. RReessuullttss;; A total of 12 patients 7 male and 5 female were involved the study. The children ranged in age from 1 day to 6 years with a mean age of 16 months and a median age of 12 months at the time of diagnosis. Seven of 12 pati- ents were referred to our unit due to bradycardia and rhythym problem; four of them due to murmur, and the rema- ining one due to syncope. Intrauterine bradycardia was also detected in four of seven patients with the symptom of bradycardia. On echocardiographic examination, corrected transposition of great arteries was found in one patient, and patent ductus arteriosus in three. Totally, specific anti-Ro and anti-La antibody were detected in the mother of six patients and one of them also had the other symptoms of neonatal lupus syndrome. Mean ventricular and atrial rates of 12 patients, obtained from electrocardiography at the time of diagnosis, were 54 ± 11 and, 116 ± 24 respectively. Pacemaker was implanted in eight patients. The follow up period ranged from 1 months to 11 years. Age at last eval- uation ranged from 6 months old to 13 years old. CCoonncclluussiioonn:: In all fetuses with bradyarrhythmias detected in utero and all fetuses of women with anti-Ro/La an- tibodies, serial echocardiographic monitoring is strongly advised.

Keywords:

Congenital, complete atrioventricular block,

PDF

___

1. Friedman DM, Duncanson LJ, Glickstein J, Buyon JP. A review of congenital heart block. Images Pediatr Cardiol 2003;16:36-48.
2. JC Steele1, LJ Dawson1, RJ Moots2, EA Field. Congenital heart block associated with undiagnosed maternal Primary Sjögren's Syndrome a case report and discussion. Oral Diseases 2005;11:190-4.
3. Michaelsson M, Engle MA. Congenital complete heart block: an international study of the natural history. Cardiovasc Clin 1972;4:85-98.
4. Jaeggi ET, Hornberger LK, Smallhorn JF, Fouron JC. Prenatal diagnosis of complete atrioventricular block associated with structural heart disease: combined experience of two tertiary care centers and review of the literature. Ultrasound Obstet Gynecol 2005;26:16-21.
5. Berg C, Geipel A, Kohl T, et al. Atrioventricular block detected in fetal life: associated anomalies and potential prognostic markers. Ultrasound Obstet Gynecol 2005;26:4-15.
6. Wahren-Herlenius M, Sonesson SE. Specificity and effector mechanisms of autoantibodies in congenital heart block. Curr Opin Immunol 2006;18:690-6.
8. Watson R, Kang JE, May M, Hudak M, Kickler T, Provost TT. Thrombocytopenia in the neonatal lupus syndrome. Arch Dermatol 1988;124:560-3.
9. Buyon JP, Waltuck J, Kleinman C, et al. In utero identification and therapy of congenital heart block. Lupus 1995;4:116-21.
10. Waltuck J, Buyon JP. Autoantibody-associated congenital heart block: outcome in mothers and children. Ann Intern Med 1994;120:544-51.
11. Buyon JP. Congenital complete heart block (review). Lupus 1993;2:291-5.
12. Eronen M, Siren MK, Ekblad H, et al. Short- and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn. Pediatrics 2000;106:86-91.
13. Buyon J, 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.
14. Friedman DM, JP Buyon. Complete atrioventricular block diagnosed prenatally: anything new on the block? Ultrasound Obstet Gynecol 2005;26:2-3.
15. Friedman DM, Rupel A, Glickstein J, Buyon JP. Congenital heart block in neonatal lupus: The pediatric cardiologist’s perspective. Indian J Pediatr 2002;69:517-22.
16. Escamilla SA, Pettersen MD. Transient Heart Block in a Neonate Associated with Previously Undiagnosed Maternal AntiRo/SSA and Anti-La/SSB Antibodies. Pediatr Cardiol. 2007;20:[Epub ahead of print].
17. GREGORATOS ET AL., ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices. http://www.acc.org/clinical/guidelines/pacemaker/index.htm; 2002.
18. A. Çeliker, S. Çicek, S. Özme. Long term results of patient with congenital complete atrioventricular block. Turk J Pediatr 1993;35:93-9.
19. Richard A. Friedman. Congenital AV Block , Pace Me Now or Pace Me Later? Circulation. 1995;92:283-5.
20. Balmer C, Bauersfeld U. Do all children with congenital complete atrioventricular block require permanent pacing? Indian Pacing Electrophysiology J 2003;3:178-83.
21. Jayaprasad N, Johnson F, Venugopal K. congenital complete heart block and maternal connective tissue disease. Int J Cardiol 2006;112:153-8.