Konjenital Tam Atriyoventriküler Bloklu Hastalarımızın Özellikleri

Yıl 2007, Cilt: 5 Sayı: 2, 47 - 51, 01.09.2007

Evren Semizel Özlem Mehtap Bostan Ergün Çil

Öz

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

Öz

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.

Anahtar Kelimeler

Congenital, complete atrioventricular block

Kaynakça

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