{"id":12854,"date":"2022-09-24T19:08:53","date_gmt":"2022-09-24T16:08:53","guid":{"rendered":"http:\/\/blog.ulubat.org\/?p=12854"},"modified":"2022-09-24T19:08:56","modified_gmt":"2022-09-24T16:08:56","slug":"buyuk-arter-transpozisyonu-tga","status":"publish","type":"post","link":"https:\/\/blog.ulubat.org\/index.php\/genel\/buyuk-arter-transpozisyonu-tga\/","title":{"rendered":"B\u00fcy\u00fck Arter Transpozisyonu (TGA)"},"content":{"rendered":"\n

\u0130nsan kalbi, bilimin tarihinin b\u00fcy\u00fck bir b\u00f6l\u00fcm\u00fcnde \u2018cerrahi olarak dokunulmaz\u2019 olarak d\u00fc\u015f\u00fcn\u00fclm\u00fc\u015ft\u00fcr. Bilim ve kardiyolojik cerrahi son 70 y\u0131lda h\u0131zl\u0131 ad\u0131mlarla ilerleme kat etti. B\u00fcy\u00fck arter transpozisyonu cerrahisiyse, kendine kardiyak cerrahinin bu yolda ne kadar ilerleme kaydetti\u011fini sembolize eden bir yer buluyor.<\/p>\n\n\n\n

B\u00fcy\u00fck arter transpozisyonu yenido\u011fanlarda g\u00f6r\u00fclen kompleks bir konjenital defekttir. Sebebi hala anla\u015f\u0131lamam\u0131\u015f olsa da, yenido\u011fanlarda g\u00f6r\u00fclen siyanotik konjenital kalp hastal\u0131klar\u0131 aras\u0131nda en yayg\u0131n oland\u0131r.<\/p>\n\n\n\n

Normal bir insan kalbinde, kalbin d\u0131\u015f\u0131na kan ta\u015f\u0131yan iki b\u00fcy\u00fck arter bulunur: Aorta ve pulmoner arter. Bu hastal\u0131\u011fa sahip \u00e7ocuklarda bu iki arter kalbe anormal bir \u015fekilde ba\u011flan\u0131r; aortan\u0131n sa\u011f ventrik\u00fcl, pulmoner arterin sol ventrik\u00fclden k\u00f6ken almas\u0131yla olu\u015fmu\u015f bir lezyondur. Arterlerin yer de\u011fi\u015ftirmesi, oksijenden yoksun kan\u0131n v\u00fccuttan sa\u011f atriuma, sa\u011f atriumdan sa\u011f ventrik\u00fcle gelip aortaya gitmesine ve v\u00fccuda geri d\u00f6nmesine sebep olur. Oksijenden zengin kansa akci\u011ferlerden sol atriuma d\u00f6ner, oradan sol ventrik\u00fcle ge\u00e7er ve akci\u011ferlere geri pompalan\u0131r. Bunun sonucu olarak, bebek do\u011fduktan hemen sonra siyanoz denilen, mavi renkler ortaya \u00e7\u0131kar. Bu mavi renk en iyi bebe\u011fin dudaklar\u0131nda ve t\u0131rnaklar\u0131n\u0131n alt\u0131nda g\u00f6zlenir. Kalp kaynakl\u0131 siyanozu olan bir bebekte, bu mavilik oksijen kullan\u0131m\u0131yla iyile\u015fmez. E\u011fer bu durum devam ederse, bebek oksijensizlikten \u00f6l\u00fcr. B\u00fcy\u00fck arter transpozisyon olan bir bebe\u011fin do\u011fumdan sonra hayatta kalabilmesinin tek yolu iki kar\u0131nc\u0131k aras\u0131nda bir kan ge\u00e7i\u015fi olmas\u0131d\u0131r ki v\u00fccuda biraz dahi olsa oksijenden zengin kan pompalanabilsin. Semptomlar\u0131n a\u011f\u0131rl\u0131\u011f\u0131ysa, bu kan ge\u00e7i\u015finin d\u00fczeyine g\u00f6re de\u011fi\u015fmektedir.<\/p>\n\n\n\n

\"\"
Resim 1: D-TGA ve L-TGA\u2019da (b\u00fcy\u00fck arter transpozisyonunun iki tipi) kalpte kan ak\u0131\u015f\u0131n\u0131 g\u00f6steren \u015fema<\/figcaption><\/figure>\n\n\n\n

Bundan sebeple intrakardiyak ba\u011flant\u0131 olarak atriyal septal defekt (ASD), ventrik\u00fcler septal defekt (VSD); ekstrakardiyak ba\u011flant\u0131 olarak patent duktus arteriyozus (PDA) ve bronkopulmoner kollateral sirk\u00fclasyon gibi ba\u015fka anormaliler kan\u0131n kar\u0131\u015fabilmesi ve ya\u015famsal faaliyetlerin tan\u0131 – tedavi s\u00fcrecinde devam edebilmesi i\u00e7in hayati \u00f6nem ta\u015f\u0131maktad\u0131r. Ayr\u0131ca D-TGA, VSD\u2019nin varl\u0131\u011f\u0131 (%30) ve yoklu\u011funa (%70) g\u00f6re iki alt t\u00fcre ayr\u0131lmaktad\u0131r.<\/p>\n\n\n\n

D-TGA ile ili\u015fkili anormaliler VSD (%50), sol ventrik\u00fcl \u00e7\u0131k\u0131m yolu darl\u0131\u011f\u0131 (%25), mitral ve trik\u00fcspid kapak rahats\u0131zl\u0131klar\u0131 ve koroner arter varyasyonlar\u0131d\u0131r. TGA-VSD hastalar\u0131 pulmoner stenozis veya artezisi, her iki atriumun AV kapak\u00e7\u0131\u011f\u0131na a\u00e7\u0131lmas\u0131, aorta koarktasyonu gibi rahats\u0131zl\u0131klara di\u011ferlerinden daha yatk\u0131nd\u0131r. Diyabetli annelerin infantlar\u0131nda ve erkeklerde daha yayg\u0131nd\u0131r. \u00d6zellikle pulmoner stenoz ve sa\u011f kardiyak ark gibi di\u011fer kardiyak defektlerle bir arada bulundu\u011fu durumlarda kromozom 22q11. delesyonuyla (DiGeorge sendromu) ba\u011flant\u0131l\u0131 olabilmektedir. Kolektif veya palyatif cerrahinin modern \u00e7a\u011f\u0131 \u00f6ncesinde ilk 1 y\u0131l\u0131n sonuna kadar mortalite %90\u2019dan fazlayd\u0131.<\/p>\n\n\n\n

\"\"
A- D-TGA diyagram\u0131
B-C- 3D Forier SingleShot turbo spinle g\u00f6r\u00fcnt\u00fclenmi\u015f (SSFP) yeniden bi\u00e7imlendirilmi\u015f oblik g\u00f6r\u00fcnt\u00fclemeler
D- Aorta ve ana pulmoner arterin yerle\u015fimi
E- G\u00f6\u011f\u00fcs grafisinde dar mediastinum ve \u201cegg on a string\u201d g\u00f6r\u00fcnt\u00fcs\u00fc<\/figcaption><\/figure>\n\n\n\n

L-TGA ya da di\u011fer bir deyi\u015fle konjenital olarak d\u00fczeltilmi\u015f transpozisyon (ventrik\u00fcler inversiyon) AV ve VA diskordans\u0131n bir arda bulundu\u011fu bir durumdur. Patofizyolojik a\u00e7\u0131dan bakarsak sistemik ve pulmoner sirk\u00fclasyonda bir problem yoktur ancak morfolojik sa\u011f kalp solda, sol kalp sa\u011fdad\u0131r. L-TGA ile ili\u015fkilendirilen baz\u0131 yayg\u0131n patolojiler VSD (%70), tipik \u015fekilde subvalvular olan pulmoner \u00e7\u0131k\u0131m yolu darl\u0131\u011f\u0131 (%40) ve trik\u00fcspit (sistemik AV) kapak anormalileridir (%90). Asemptomatik hasta say\u0131s\u0131 %10\u2019un alt\u0131ndad\u0131r. Fizyolojik ak\u0131m sa\u011fland\u0131\u011f\u0131 i\u00e7in asemptomatik olanlar (izole anomali) belli bir ya\u015fa kadar fark edilmemektedir. Ancak t\u00fcm hastalarda genelde sa\u011f ventrik\u00fcl sol ventrik\u00fcl\u00fcn sistemik sirk\u00fclasyon i\u00e7in \u00fcretti\u011fi iti\u015f bas\u0131nc\u0131 yetersiz kal\u0131r ve sistemik kalp yetmezli\u011fi ya\u015farlar. Sa\u011f kalp yetmezli\u011fine trik\u00fcspit kapak\u00e7\u0131\u011f\u0131n geometrik konfig\u00fcrasyon olarak fazla vol\u00fcm ve bas\u0131nca adaptasyon s\u0131k\u0131nt\u0131s\u0131 \u00e7ekmesi de sebep olabilmektedir.<\/p>\n\n\n\n

Klinik Bulgular:<\/em><\/strong><\/p>\n\n\n\n

  • Takipne (H\u0131zl\u0131 Solunum) (>60 soluk\/dk)<\/li>
  • Siyanoz<\/li>
  • \u00dcf\u00fcr\u00fcm<\/li>
  • S2 sesinin normalden y\u00fcksek olmas\u0131<\/li>
  • Azalan femoral nab\u0131z <\/li><\/ul>\n\n\n\n

    Tan\u0131: <\/em><\/strong><\/p>\n\n\n\n

    Fetal Tan\u0131:<\/strong> Ultrason cihaz\u0131yla yap\u0131lan incelemelerde VSD, AV tam blo\u011fu gibi kolay tespit edilebilen yan rahats\u0131zl\u0131klar olmad\u0131k\u00e7a tespiti zorludur. <\/p>\n\n\n\n

    Postnatal Tan\u0131: <\/strong>Alt\u0131nda yatan di\u011fer siyanotik konjenital kalp defektlerinden klinik anlamda \u015f\u00fcphelenilmesi \u00fcst\u00fcne kurulur ve \u00f6ncelikle ekokardiyografi ile kan\u0131tlan\u0131r. <\/p>\n\n\n\n

    Ekokardiyografi:<\/strong> 2 boyutlu transtorasik ekokardiyografi ve Doppler ekokardiyografisi hastalar\u0131 b\u00fcy\u00fck bir b\u00f6l\u00fcm\u00fc i\u00e7in tan\u0131da yeterli olmaktad\u0131r.<\/p>\n\n\n\n

    \"\"
    TGA\u2019l\u0131 bir hastan\u0131n subksifoid sol anterior oblique b\u00f6lgesinden al\u0131nan 2 boyutlu ekokardiyografik g\u00f6r\u00fcnt\u00fc<\/figcaption><\/figure>\n\n\n\n

    S\u0131kl\u0131kla kullan\u0131lmasa da TGA tan\u0131s\u0131nda kullan\u0131labilecek di\u011fer y\u00f6ntemler:<\/p>\n\n\n\n

    • EKG<\/li>
    • G\u00f6\u011f\u00fcs grafisi<\/li>
    • Kardiyak kateterizasyon ve anjiyogram<\/li>
    • Kardiyovask\u00fcler MR<\/li>
    • Kardiyovask\u00fcler BT<\/li>
    • N\u00fckleer sintigrafi<\/li>
    • Stres ekokardiyografi<\/li><\/ul>\n\n\n\n

      Tedavi Y\u00f6ntemleri:<\/em><\/strong><\/p>\n\n\n\n

      D-TGA<\/strong><\/p>\n\n\n\n

      Durumdan \u015f\u00fcphelenildi\u011finde prostaglandin E1 (PGE1) (alprostadil) tedavisine ba\u015flan\u0131r. Duktus arteriosus a\u00e7\u0131kl\u0131\u011f\u0131ndan oksijenlenmenin korunmas\u0131 i\u00e7in prostaglandin inf\u00fczyonu \u00f6nem ta\u015f\u0131maktad\u0131r. PGE1\u2019in en b\u00fcy\u00fck yan etkisi apnedir, neonatal endotrakeal ent\u00fcbasyon gerekebilir. Bunun i\u00e7in \u00f6ncelik belli bir ya\u015f grubundaki \u00e7ocuklar\u0131 pediatrik kardiyoloji servisine aktarmakt\u0131r. Hasta s\u0131cak tutulmal\u0131d\u0131r.<\/p>\n\n\n\n

      A\u011f\u0131r derecede hipoksik ya da PGE1 inf\u00fczyonuna ra\u011fmen asidotik durumunu koruyan infantlarda Rashkind balon artriyal septostomisi uygulan\u0131r.<\/p>\n\n\n\n

      \"\"
      Balon atriyal septostomisi<\/figcaption><\/figure>\n\n\n\n

      Arteryel switch (Jatene) operasyonu Senning ve Mustang taraf\u0131ndan 1960\u2019larda geli\u015ftirilen TGA tedavisindeki standart cerrahi prosed\u00fcrd\u00fcr. Pulmoner arter ve aorta sin\u00fcslerinin heme alt\u0131ndan kesilerek morfolojik olarak normal anatomik pozisyona reanastomozu ger\u00e7ekle\u015ftirilir. Koroner arterler de eski aort k\u00f6k\u00fcnden al\u0131narak eski pulmoner k\u00f6k\u00fcne (neoaorta) reimplante edilir. Jatene prosed\u00fcr\u00fcn\u00fcn karma\u015f\u0131k olmayan D-TGA hastalar\u0131nda ya\u015fama oran\u0131 %95\u2019in \u00fcst\u00fcndedir.<\/p>\n\n\n\n

      \"\"
      Arteryel switch operasyonunda koroner arterlerin translokalize edilme metodu<\/figcaption><\/figure>\n\n\n\n

      Kaynak\u00e7a:<\/em><\/strong><\/p>\n\n\n\n

      1. https:\/\/www.uptodate.com\/contents\/pathophysiology-clinical-manifestations-and-diagnosis-of-d-transposition-of-the-great-arteries?search=transposition%20of%20the%20great%20arter&source=search_result&selectedTitle=1~63&usage_type=default&display_rank=1#H2924715<\/li>
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      9. https:\/\/www.uptodate.com\/contents\/management-and-outcome-of-d-transposition-of-the-great-arteries?search=transposition%20of%20the%20great%20arter&source=search_result&selectedTitle=2~63&usage_type=default&display_rank=2<\/li>
      10. https:\/\/www.uptodate.com\/contents\/l-transposition-of-the-great-arteries?search=transposition%20of%20the%20great%20arter&source=search_result&selectedTitle=3~63&usage_type=default&display_rank=3#H156826526<\/li>
      11. https:\/\/www.clinicalkey.com\/#!\/content\/3-s2.0-B9780323529501004582?scrollTo=%23sc0010<\/li>
      12. Marathe SP, Talwar S. Surgery for transposition of great arteries: A historical perspective. Ann Pediatr Cardiol. 2015 May-Aug;8(2):122-8. doi: 10.4103\/0974-2069.157025. PMID: 26085763; PMCID: PMC4453180.<\/li><\/ol>\n\n\n\n

        Edit\u00f6rler: Nurbanu Okur ve G\u00fcnsu G\u00fcrg\u00fcn<\/strong><\/p>\n","protected":false},"excerpt":{"rendered":"

        \u0130nsan kalbi, bilimin tarihinin b\u00fcy\u00fck bir b\u00f6l\u00fcm\u00fcnde \u2018cerrahi olarak dokunulmaz\u2019 olarak d\u00fc\u015f\u00fcn\u00fclm\u00fc\u015ft\u00fcr. Bilim ve kardiyolojik cerrahi son 70 y\u0131lda h\u0131zl\u0131<\/p>\n","protected":false},"author":1192,"featured_media":12856,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[1],"tags":[2375,451,727,2376,265,66],"acf":[],"views":717,"_links":{"self":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12854"}],"collection":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/users\/1192"}],"replies":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/comments?post=12854"}],"version-history":[{"count":4,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12854\/revisions"}],"predecessor-version":[{"id":12864,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12854\/revisions\/12864"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media\/12856"}],"wp:attachment":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media?parent=12854"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/categories?post=12854"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/tags?post=12854"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}