{"id":12872,"date":"2022-09-24T19:57:51","date_gmt":"2022-09-24T16:57:51","guid":{"rendered":"http:\/\/blog.ulubat.org\/?p=12872"},"modified":"2022-09-24T19:57:55","modified_gmt":"2022-09-24T16:57:55","slug":"fallot-tetralojisi-tani-ve-tedavisi","status":"publish","type":"post","link":"https:\/\/blog.ulubat.org\/index.php\/genel\/fallot-tetralojisi-tani-ve-tedavisi\/","title":{"rendered":"Fallot Tetralojisi: Tan\u0131 ve Tedavisi"},"content":{"rendered":"\n<p>Eskiden Steno-Fallot tetralojisi olarak bilinen fallot tetralojisinin tarihi kardiyolojide, kardiyak cerrahide ve kalbin geli\u015fimini anlamada dramatik evrimin bir par\u00e7as\u0131d\u0131r. Yeni aletlerin ve tedavi konseptlerinin \u00e7o\u011fu Steno olarak bilinen Danimarkal\u0131 me\u015fhur anatomist Nils Stensen taraf\u0131ndan 1673\u2019te ilk defekt tan\u0131mland\u0131\u011f\u0131nda ve 1888\u2019de tetraloji terimi tan\u0131mland\u0131\u011f\u0131nda geli\u015fti. Nils Stensen\u2019in bu tan\u0131m\u0131, tahminen bu kalp malfonksiyonunun en eski tan\u0131m\u0131d\u0131r ve bu tan\u0131m 1944\u2019te Blalock-Taussig shunt\u0131n\u0131n temelini olu\u015fturdu. Tetraloji d\u00f6rtl\u00fc seridir, d\u00f6rt defektin bir arada bulunmas\u0131 anlam\u0131na gelir.<\/p>\n\n\n\n<p>Fallot tetralojisi (FOT) t\u0131bbi anlamda bak\u0131ld\u0131\u011f\u0131nda a\u011f\u0131r oranda ventrik\u00fcler septal defekt (VSD), ventrik\u00fcllere do\u011fru ilerlemi\u015f ata binen aorta, sa\u011f ventrik\u00fcler \u00e7\u0131k\u0131m obstr\u00fcksiyonu ve sa\u011f ventrik\u00fcler hipertrofisiyle karakterize edilebilen siyanotik konjenital bir hastal\u0131kt\u0131r. Fallot tetralojisi 10.000 canl\u0131 do\u011fmdan 3\u2019\u00fcnde g\u00f6r\u00fcl\u00fcr. T\u00fcm konjenital kalp hastal\u0131klar\u0131n\u0131n yakla\u015f\u0131k %7-10\u2019unu kar\u015f\u0131lar ve hayat\u0131n ilk y\u0131l\u0131nda intervasyon gerektiren en yayg\u0131n siyanotik konjenital kalp lezyonlar\u0131ndan biridir. Erkeklerde kad\u0131nlara oranla k\u00fc\u00e7\u00fck bir oranda insidans\u0131 daha y\u00fcksektir. 22q11 delesyonunun genetik etkilerinden \u015f\u00fcphelenilmektedir. Morbidite ve mortalite oranlar\u0131 bu hastalar\u0131n ilk medikal bak\u0131mlar\u0131n\u0131, cerrahi onar\u0131m ve postoperatif komplikasyonlar\u0131n y\u00f6netiminlerini i\u00e7eren kapsaml\u0131 y\u00f6netimlerle birlikte belirgin bir \u015fekilde azalm\u0131\u015ft\u0131r.<\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" width=\"1024\" height=\"1024\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-38-1024x1024.png\" alt=\"\" class=\"wp-image-12873\" srcset=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-38-1024x1024.png 1024w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-38-150x150.png 150w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-38-1536x1536.png 1536w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-38-2048x2048.png 2048w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-38-768x768.png 768w\" sizes=\"(max-width: 1024px) 100vw, 1024px\" \/><figcaption>Fallot tetralojisindeki anomalileri g\u00f6steren \u015fematik ill\u00fcstrasyon<\/figcaption><\/figure>\n\n\n\n<h2>Anatomisi<\/h2>\n\n\n\n<p>Fallot tetralojisine sebep olan ana embriyolojik sebep bilinmemektedir. Bilinen ise geli\u015fim s\u0131ras\u0131nda infundib\u00fcler septumun \u00f6ne ve sefalad deviasyonu kaynakl\u0131 ventrik\u00fcler septal defekt, ata binen aorta defektine sebep olur ve sonradan sa\u011f ventrik\u00fcler \u00e7\u0131k\u0131m obstr\u00fcksiyonuna yol a\u00e7ar.<\/p>\n\n\n\n<p>Kalp geni\u015flemi\u015ftir ve sa\u011f ventrik\u00fcl hipertrofisinin bir sonucu olarak \u201c\u00e7izme \u015fekli\u201d ni alm\u0131\u015ft\u0131r. Kalbin sol taraf\u0131n\u0131n boyutlar\u0131 normaldir.<\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" width=\"1024\" height=\"1024\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-39-1024x1024.png\" alt=\"\" class=\"wp-image-12875\" srcset=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-39-1024x1024.png 1024w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-39-150x150.png 150w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-39-1536x1536.png 1536w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-39-2048x2048.png 2048w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-39-768x768.png 768w\" sizes=\"(max-width: 1024px) 100vw, 1024px\" \/><\/figure>\n\n\n\n<h2>Genetik Fakt\u00f6rler<\/h2>\n\n\n\n<p>Nonsendromik hastalarda y\u00fcr\u00fct\u00fclen \u00e7al\u0131\u015fmalarda a\u015fa\u011f\u0131daki genetik anormaliler rapor edilmi\u015ftir:<\/p>\n\n\n\n<ul><li>114 nonsendromik TOF hastas\u0131n\u0131 i\u00e7eren bir \u00e7al\u0131\u015fmada, hastalar\u0131n %4\u2019\u00fc kardiyak geli\u015fimde rol oynayan transkripsiyon fakt\u00f6r\u00fc NKX2.5\u2019ta mutasyonlar vard\u0131r.<\/li><li>Nonsendromik TOF hastalar\u0131 ve ebeveynleriyle yap\u0131lan genom genelli \u00e7al\u0131\u015fmalarda de novo varyantlar\u0131 bir\u00e7ok kromozal lokasyonda %0.1\u2019den az olmas\u0131na kar\u015f\u0131n sporadik TOF vakalar\u0131n\u0131n yakla\u015f\u0131k %10\u2019unda bulundu\u011fu tahmin edilmektedir.<\/li><li>MTHFR polimorfizm de TOF geli\u015fme riskini artt\u0131rmada ba\u011flant\u0131l\u0131 bulunmu\u015ftur.<\/li><\/ul>\n\n\n\n<p>TOF hastalar\u0131n\u0131n yakla\u015f\u0131k %15\u2019i a\u015fa\u011f\u0131daki sendromlarla birlikte bulunmu\u015ftur:<\/p>\n\n\n\n<ul><li>Down sendromu (trizomi 21)<\/li><li>Alagille sendromu (JAG1 mutasyonu)<\/li><li>DiGeorge ve velokardiyofasiyal sendromlar\u0131 (22q11 kromozomunda delesyon)<\/li><\/ul>\n\n\n\n<p>Ailelerde durumun tekrarlanma olas\u0131l\u0131\u011f\u0131 %3&#8217;t\u00fcr.<\/p>\n\n\n\n<h2>Patofizyolojisi<\/h2>\n\n\n\n<p>Fallot tetralojisinin fizyolojik sonu\u00e7lar\u0131 sa\u011f ventrik\u00fcler \u00e7\u0131k\u0131m obstr\u00fcksiyonunun derecesine b\u00fcy\u00fck oranda ba\u011fl\u0131d\u0131r. VSD tipik olarak geni\u015f ve s\u0131n\u0131rland\u0131r\u0131lmam\u0131\u015fsa, sa\u011f ventrik\u00fcl\u00fcn bas\u0131nc\u0131 sol ventrik\u00fcle de yans\u0131r. Bunun sonucu olarak VSD\u2019den ge\u00e7en kan ak\u0131m\u0131n\u0131n y\u00f6n\u00fc defektin boyutuyla de\u011fil, en az diren\u00e7li yola g\u00f6re olu\u015fur.<\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" width=\"1024\" height=\"1024\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-40-1024x1024.png\" alt=\"\" class=\"wp-image-12876\" srcset=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-40-1024x1024.png 1024w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-40-150x150.png 150w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-40-1536x1536.png 1536w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-40-2048x2048.png 2048w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-40-768x768.png 768w\" sizes=\"(max-width: 1024px) 100vw, 1024px\" \/><figcaption>A.Anomalite diyagram\u0131<br>B.Fiziksel bulgular<br>C.Tipik sa\u011f ventrik\u00fcl hipertrofisini g\u00f6steren g\u00f6\u011f\u00fcs grafisi<br>D.Sa\u011f atriyal ve ventrik\u00fcler hipertrofi ve ra\u011f axis deviasyonunu g\u00f6steren EKG<\/figcaption><\/figure>\n\n\n\n<h2>Tan\u0131<\/h2>\n\n\n\n<p>Faydal\u0131 tan\u0131 testleri g\u00f6\u011f\u00fcs radyografisi, ekokardiyogram, elektrokardiyogram olarak say\u0131labilir. Ekokardiyagram kesin tan\u0131 sa\u011flar ve genelde cerrahi tedavi plan\u0131n\u0131n olu\u015fturulmas\u0131 i\u00e7in \u00f6nemli bilgiler sa\u011flar. Gestasyonun ilk 12 haftas\u0131n\u0131n erken d\u00f6nemlerinde antenatal tan\u0131 konabilmektedir. Yakla\u015f\u0131k olarak hastalar\u0131n yar\u0131s\u0131 do\u011fumdan \u00f6nce tan\u0131 al\u0131yor.<\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" width=\"1024\" height=\"1024\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-41-1024x1024.png\" alt=\"\" class=\"wp-image-12877\" srcset=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-41-1024x1024.png 1024w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-41-150x150.png 150w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-41-1536x1536.png 1536w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-41-2048x2048.png 2048w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2022\/09\/image-41-768x768.png 768w\" sizes=\"(max-width: 1024px) 100vw, 1024px\" \/><\/figure>\n\n\n\n<h2>Prognoz<\/h2>\n\n\n\n<p>Fallot tetralojisinin ilerleyen y\u0131llar\u0131yla ilgili yap\u0131lan \u00e7al\u0131\u015fmalar gayet iyi sonu\u00e7lar veriyor. Fallot tetralojisiyle 30 y\u0131l \u00f6nce do\u011fan hastalarda uzun d\u00f6nemde %85 hayatta kalma oran\u0131 var. Ciddi rezid\u00fclerin yoklu\u011funda normal bir hayat ge\u00e7irebilmekte hatta ba\u015far\u0131l\u0131 hamileliklere sahip olabilmektedirler.<\/p>\n\n\n\n<h2>Tedavi:<\/h2>\n\n\n\n<p>Fallot tetralojisine sahip t\u00fcm bebekler d\u00fczelmek i\u00e7in cerrahi operasyon ge\u00e7irmelidir. Tedavi edilmeyen bebekler geli\u015fimini d\u00fczg\u00fcn tamamlayamamaktad\u0131r. \u00c7ocu\u011fun durumuna g\u00f6re yap\u0131lacak en uygun cerrahi operasyon ve bu operasyonun zaman\u0131 doktoru taraf\u0131ndan belirlenmelidir.<\/p>\n\n\n\n<p>Bu cerrahi operasyon defekti d\u00fczeltmek i\u00e7in intrakardiyak a\u00e7\u0131k kalp ameliyat\u0131 veya ge\u00e7ici bir \u015fant operasyonu olabilmektedir. \u015eant ameliyat\u0131 akci\u011ferlere giden kan oran\u0131n\u0131 artt\u0131rmay\u0131 ama\u00e7larken a\u00e7\u0131k kalp ameliyat\u0131 kalple akci\u011fer aras\u0131 olan kan ak\u0131\u015f\u0131n\u0131n ve kalbin \u00e7al\u0131\u015fmas\u0131n\u0131n olabildi\u011fince normale d\u00f6nd\u00fcr\u00fclmesi i\u00e7in yap\u0131l\u0131r. Premat\u00fcre do\u011fan ya da az geli\u015fmi\u015f bir pulmoner artere sahip olan bebeklerde a\u00e7\u0131k kalp ameliyat\u0131ndan \u00f6nce palyatif operasyon olarak ge\u00e7ici \u015fant operasyonu yap\u0131l\u0131r.<\/p>\n\n\n\n<h2>Kaynak\u00e7a:<\/h2>\n\n\n\n<ol><li><a href=\"https:\/\/www.uptodate.com\/contents\/tetralogy-of-fallot-with-pulmonary-atresia-and-major-aortopulmonary-collateral-arteries-tof-pa-mapcas?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=4~111&amp;usage_type=default&amp;display_rank=4\">https:\/\/www.uptodate.com\/contents\/tetralogy-of-fallot-with-pulmonary-atresia-and-major-aortopulmonary-collateral-arteries-tof-pa-mapcas?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=4~111&amp;usage_type=default&amp;display_rank=4<\/a><\/li><li><a href=\"https:\/\/www.uptodate.com\/contents\/management-and-outcome-of-tetralogy-of-fallot?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=2~111&amp;usage_type=default&amp;display_rank=2\">https:\/\/www.uptodate.com\/contents\/management-and-outcome-of-tetralogy-of-fallot?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=2~111&amp;usage_type=default&amp;display_rank=2<\/a><\/li><li><a href=\"https:\/\/www.uptodate.com\/contents\/pathophysiology-clinical-features-and-diagnosis-of-tetralogy-of-fallot?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=1~111&amp;usage_type=default&amp;display_rank=1\">https:\/\/www.uptodate.com\/contents\/pathophysiology-clinical-features-and-diagnosis-of-tetralogy-of-fallot?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=1~111&amp;usage_type=default&amp;display_rank=1<\/a><\/li><li><a href=\"https:\/\/www.uptodate.com\/contents\/tetralogy-of-fallot-the-basics?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=3~111&amp;usage_type=default&amp;display_rank=3\">https:\/\/www.uptodate.com\/contents\/tetralogy-of-fallot-the-basics?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=3~111&amp;usage_type=default&amp;display_rank=3<\/a><\/li><li><a href=\"https:\/\/www.uptodate.com\/contents\/cardiac-causes-of-cyanosis-in-the-newborn?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=5~111&amp;usage_type=default&amp;display_rank=5\">https:\/\/www.uptodate.com\/contents\/cardiac-causes-of-cyanosis-in-the-newborn?search=tetralogy%20of%20fallot&amp;source=search_result&amp;selectedTitle=5~111&amp;usage_type=default&amp;display_rank=5<\/a><\/li><li>https:\/\/www.clinicalkey.com\/#!\/content\/book\/3-s2.0-B9780323353175000119?scrollTo=%23hl0001165<\/li><li>https:\/\/www.clinicalkey.com\/#!\/content\/67-s2.0-f5005e21-a1f2-4731-971f-492dc0c0c55c<\/li><li>https:\/\/www.clinicalkey.com\/#!\/content\/book\/3-s2.0-B9780323713337008948?scrollTo=%23hl0000415<\/li><li>https:\/\/www.clinicalkey.com\/#!\/content\/journal\/1-s2.0-S0733865120300321<\/li><li>https:\/\/www.clinicalkey.com\/#!\/content\/book\/3-s2.0-B9780323479103000528?scrollTo=%23hl0001496<\/li><li>Bailliard F, Anderson RH. Tetralogy of Fallot. Orphanet J Rare Dis. 2009 Jan 13;4:2. doi: 10.1186\/1750-1172-4-2. PMID: 19144126; PMCID: PMC2651859.<\/li><li>Jacobs, M., &amp; Jacobs, J. (2008). The early history of surgery for patients with tetralogy of Fallot. Cardiology in the Young, 18(S3), 8-11. doi:10.1017\/S1047951108003247<\/li><li>Open Access Journal of Cardiology. Tetralogy of Fallot. Mini Review Vol. 1 Issue 2 Published: September 27 2017 Available at:<\/li><li>Neill CA, Clark EB. Tetralogy of Fallot. The first 300 years. Tex Heart Inst J. 1994;21(4):272-9. PMID: 7888802; PMCID: PMC325189.<\/li><li><a href=\"https:\/\/www.mayoclinic.org\/diseases-conditions\/tetralogy-of-fallot\/diagnosis-treatment\/drc-20353482#:~:text=All%20babies%20who%20have%20tetralogy,your%20or%20your%20child's%20condition.\">https:\/\/www.mayoclinic.org\/diseases-conditions\/tetralogy-of-fallot\/diagnosis-treatment\/drc-20353482#:~:text=All%20babies%20who%20have%20tetralogy,your%20or%20your%20child&#8217;s%20condition.<\/a><\/li><\/ol>\n\n\n\n<p><strong>Edit\u00f6rler: Nurbanu Okur ve G\u00fcnsu G\u00fcrg\u00fcn<\/strong><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Eskiden Steno-Fallot tetralojisi olarak bilinen fallot tetralojisinin tarihi kardiyolojide, kardiyak cerrahide ve kalbin geli\u015fimini anlamada dramatik evrimin bir par\u00e7as\u0131d\u0131r. Yeni<\/p>\n","protected":false},"author":1192,"featured_media":12874,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[1],"tags":[73,2381,451,727,2378,265,66],"acf":[],"views":524,"_links":{"self":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12872"}],"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=12872"}],"version-history":[{"count":1,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12872\/revisions"}],"predecessor-version":[{"id":12878,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/12872\/revisions\/12878"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media\/12874"}],"wp:attachment":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media?parent=12872"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/categories?post=12872"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/tags?post=12872"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}