Mustafa Kemal DEMİRAĞ, Hasan Tahsin KEÇELİGİL, Kemal BAYSAL

Situs inversus totalis ve tam bloklu yenidoğanda kalıcı kalp pili yerleştirilmesi

Atriyoventriküler tam blok ile birlikte görülen komplet atriyoventriküler kanal defekti oldukça nadir rastlanan doğumsal kalp anomalilerindendir. Otuz iki yaşında bir anne, gebelik sırasında yapılan fetal ultrasonografide doğuştan kalp anomalisinden şüphelenilerek merkezimize sevk edildi. Doğum sonrası ikinci saatte yapılan ekokardiyografide yenidoğanda dekstrokardi, geniş atriyal septal defekt, tek atriyoventriküler kapak, ventriküler septal defekt ve aort yetmezliği saptandı. Komplet atriyoventriküler kanal, atriyoventriküler tam blok ve situs inversus totalis tanıları konan bebeğe öncelikli olarak epikardiyal kalp pili yerleştirilmesine karar verildi. Hastaya subdiyafragmatik pozisyonda VVI kalp pili jeneratörü ve sağ ventrikül apeksine yakın bir bölgeye pediyatrik epikardiyal led yerleştirildi. Ameliyat sonrası beşinci günde ekstübe edilen hasta genel durumunun düzelmesi üzerine taburcu edildi. Atriyoventriküler tam blok ve komplet atriyoventriküler kanal defektine situs inversus totalisin de eşlik ettiği bir olguya literatürde rastlamadık.

Permanent pacemaker implantation in a newborn with situs inversus totalis and complete heart block

Complete atrioventricular canal defect accompanied by complete atrioventricular block is one of the rare congenital heart abnormalities. A 32-year-old mother was referred to our clinic as her fetal ultrasonography was suggestive of a congenital heart abnormality. Echocardiographic examination of the newborn showed dextrocardia, a large atrial septal defect, single atrioventricular valve, ventricular septal defect, and aortic insufficiency. The newborn was diagnosed as having complete atrioventricular canal defect, complete atrioventricular block, and situs inversus totalis, and implantation of an epicardial pacemaker was decided as the initial intervention. A VVI pacemaker generator was implanted in the subdiaphragmatic position and a pediatric epicardial lead was implanted in a region close to the right ventricular apex. The patient was extubated on the fifth postoperative day and was then discharged with improved general condition. A literature review showed no reported case of complete atrioventricular canal defect and complete atrioventricular block accompanied by situs inversus totalis.

PDF

___

1) Kutsal A. Atriyal septal defekt ve atriyoventriküler kanal defektleri. In: Bozer AY, editör. Kalp hastalıkları ve cerrahisi. Ankara: Ayyıldız Matbaası; 1985. s. 283-309.
2) Batra AS, Wells WJ, Hinoki KW, Stanton RA, Silka MJ. Late recovery of atrioventricular conduction after pacemaker implantation for complete heart block associated with surgery for congenital heart disease. J Thorac Cardiovasc Surg 2003;125:1291-3.
3) Johansen AS, Herlin T. Neonatal lupus syndrome. Association with complete congenital atrioventricular block. Ugeskr Laeger 1998;160:2521-5. [Abstract]
4) Grolleau R, Leclercq F, Guillaumont S, Voisin M. Congenital atrioventricular block. Arch Mal Coeur Vaiss 1999;92:47-55 [Abstract]
5) Siller Rodríguez J, Villegas Cabello O, Martínez Salinas R. Placement of permanent epicardial pacemaker in a newborn with congenital complete AV block. Arch Inst Cardiol Mex 2000;70:180-6. [Abstract]
6) Minassian VA, Jazayeri A. Favorable outcome in a pregnancy with complete fetal heart block and severe bradycardia. Obstet Gynecol 2002;100:1087-9.
7) Lin MC, Fu YC, Tseng JJ, Jan SL, Chi CS. Congenital complete heart block. Acta Paediatr Taiwan 2001;42:42-5.
8) Finkelstein Y, Adler Y, Harel L, Nussinovitch M, Youinou P. Anti-Ro (SSA) and anti-La (SSB) antibodies and complete congenital heart block. Ann Med Interne 1997;148:205-8.
9) Herreman G, Sauvaget F, Généreau T, Galezowski N.Congenital atrioventricular block and maternal autoimmune diseases. Ann Med Interne 1990;141:234-8. [Abstract]
10) Eronen M. Long-term outcome of children with complete heart block diagnosed after the newborn period. Pediatr Cardiol 2001; 22:133-7.
11) Baschat AA, Gembruch U, Knöpfle G, Hansmann M. Firsttrimester fetal heart block: a marker for cardiac anomaly. Ultrasound Obstet Gynecol 1999;14:311-4.
12) Olguntürk R, Tunaoğlu FS, Oğuz D, Cinaz P, Bideci A. Complete atrioventricular heart block in congenital hypothyroidism. Turk J Pediatr 1998;40:431-5.
13) Young JN, Bacaner TJ, Powell CA. Preperitoneal suprahepatic pacemaker generator placement in the pediatric population. Ann Thorac Surg 1997;63:1486 8.
14) Ohmi M, Tofukuji M, Sato K, Nakame T, Sato N, Haneda K, et al. Permanent pacemaker implantation in premature infants less than 2,000 grams of body weight. Ann Thorac Surg 1992; 54:1223-5.