{"id":9526,"date":"2021-01-11T19:54:53","date_gmt":"2021-01-11T16:54:53","guid":{"rendered":"http:\/\/blog.ulubat.org\/?p=9526"},"modified":"2021-01-11T19:54:55","modified_gmt":"2021-01-11T16:54:55","slug":"tek-ventrikulle-yasam-hipoplastik-sol-kalp-sendromu","status":"publish","type":"post","link":"https:\/\/blog.ulubat.org\/index.php\/genel\/tek-ventrikulle-yasam-hipoplastik-sol-kalp-sendromu\/","title":{"rendered":"Tek Ventrik\u00fclle Ya\u015fam: Hipoplastik Sol Kalp Sendromu"},"content":{"rendered":"\n

Hipoplastik Sol Kalp Sendromu (HLHS), konjenital kalp hastal\u0131klar\u0131 i\u00e7erisinde en a\u011f\u0131r seyreden ve tedavisi en g\u00fc\u00e7 olan hastal\u0131klardan bir tanesidir. Peki sizce geli\u015fmemi\u015f sol kalp yap\u0131lar\u0131na sahip bir kalbin fonksiyonel hale gelme \u015fans\u0131 var m\u0131d\u0131r? Bu yaz\u0131mda, bu sava\u015f\u00e7\u0131 bebeklerin hayata tutunma \u00f6yk\u00fclerini sizlerle payla\u015fmaya \u00e7al\u0131\u015faca\u011f\u0131m.<\/em><\/p>\n\n\n\n

<\/p>\n\n\n\n

Hipoplastik Sol Kalp Nedir?<\/strong><\/h2>\n\n\n\n

Hipoplastik sol kalp, geli\u015fmemi\u015f bir sol ventrik\u00fcl ve sistemik dola\u015f\u0131m\u0131 sa\u011flama yetisinden yoksun sol kalp yap\u0131lar\u0131 ile karakterize edilmi\u015f konjenital bir sendromdur. HLHS tedavi edilmedi\u011fi takdirde kesin olarak \u00f6l\u00fcmc\u00fcld\u00fcr. HLHS\u2019de g\u00f6r\u00fclen yap\u0131sal bozukluklar\u0131 ayr\u0131nt\u0131l\u0131 inceleyecek olursak kar\u015f\u0131m\u0131za \u00e7\u0131kan bozukluklar \u015funlard\u0131r:<\/p>\n\n\n\n

Geli\u015fmemi\u015f bir sol ventrik\u00fcl mevcuttur.<\/p>\n\n\n\n

Mitral ve aortik kapaklar atretik, hipoplastik ya da stenotiktir.<\/p>\n\n\n\n

Aortik koarktasyon e\u015flik edebilir.<\/p>\n\n\n\n

Asendan aorta b\u00fcy\u00fck oranda hipoplastik olup sadece koroner kan ak\u0131m\u0131n\u0131 sa\u011flayabilmektedir.<\/p>\n\n\n\n

Sistemik kan ak\u0131m\u0131n\u0131 da sa\u011flama g\u00f6revini \u00fcstlenen sa\u011f kalp hipertrofik yap\u0131dad\u0131r.<\/p>\n\n\n\n

Patent foramen ovale e\u015flik edebilir.<\/p>\n\n\n\n

<\/p>\n\n\n\n

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Commons.wikimedia.org<\/figcaption><\/figure>\n\n\n\n

Kimlerde g\u00f6r\u00fcl\u00fcr?<\/strong><\/h2>\n\n\n\n

Her 10.000 yeni do\u011fandan 2-3\u2019\u00fc HLHS ile d\u00fcnyaya gelmektedir. Erkeklerde daha y\u00fcksek s\u0131kl\u0131kla g\u00f6r\u00fcl\u00fcr. Baz\u0131 ailelerde HLHS yatk\u0131nl\u0131\u011f\u0131 oldu\u011fu bilinse de hastal\u0131\u011f\u0131n genetik temeli tam olarak a\u00e7\u0131klanabilmi\u015f de\u011fildir.<\/p>\n\n\n\n

<\/p>\n\n\n\n

Ya\u015fam\u0131n \u0130lk G\u00fcnleri<\/strong><\/h2>\n\n\n\n

Oksijen ile sat\u00fcre olmu\u015f pulmoner ven kan\u0131 sol atriyum ve sol ventrik\u00fcl aras\u0131nda yer alan mitral kapaktaki t\u0131kan\u0131kl\u0131k dolay\u0131s\u0131yla sol ventrik\u00fcle ge\u00e7emez, sa\u011f atriuma ge\u00e7erek sistemik ven\u00f6z kan ile kar\u0131\u015f\u0131r. Buradan sonra aortaya g\u00f6nderilecek kan ak\u0131m\u0131 duktus arteriosustan ge\u00e7ecek kan miktar\u0131na ba\u011fl\u0131d\u0131r. duktus arteriosus,\u00a0 fetal d\u00f6nemde pulmoner arter ve aorta aras\u0131nda ba\u011flant\u0131 sa\u011flayan bir yap\u0131d\u0131r ve do\u011fumdan k\u0131sa s\u00fcre sonra kapanmaktad\u0131r. Dolay\u0131s\u0131yla bu bebeklerde ameliyat i\u00e7in beklenebilecek s\u00fcre duktusun a\u00e7\u0131k kalaca\u011f\u0131 s\u00fcre ile k\u0131s\u0131tl\u0131d\u0131r.<\/p>\n\n\n\n

<\/p>\n\n\n\n

Tan\u0131<\/strong><\/h2>\n\n\n\n

Tan\u0131, fetal d\u00f6nemde fetal ekokardiyogram (EKO) ile konulabilece\u011fi gibi do\u011fumun ard\u0131ndan yine EKO ile konulabilir. HLHS, \u00fclkemizde fetal d\u00f6nemde tespit edildi\u011finde terminasyon \u00f6nerilen tek kalp hastal\u0131\u011f\u0131<\/strong> olarak \u00f6ne \u00e7\u0131kmaktad\u0131r. Bu durum da sendromun ne kadar ciddi sorunlara yol a\u00e7abildi\u011fini g\u00f6zler \u00f6n\u00fcne sermektedir.<\/p>\n\n\n\n

<\/p>\n\n\n\n

Tedavi<\/strong><\/h2>\n\n\n\n

Hastal\u0131\u011f\u0131n tedavisi i\u00e7in 3 farkl\u0131 opsiyon mevcuttur:<\/p>\n\n\n\n

Kalp Nakli:<\/strong> Bu ya\u015f grubu i\u00e7in don\u00f6r say\u0131s\u0131n\u0131n olduk\u00e7a az olmas\u0131 ve \u00f6m\u00fcr boyu al\u0131nmas\u0131 gereken imm\u00fcnosupresif tedavilerin tehlikelerinden dolay\u0131 olduk\u00e7a az tercih edilmektedir.<\/p>\n\n\n\n

Destekleyici Tedavi:<\/strong> Ciddi bir ya\u015fam uzat\u0131c\u0131 etkisi bulunmamaktad\u0131r.<\/p>\n\n\n\n

3 A\u015famal\u0131 Cerrahi Onar\u0131m:<\/strong> \u00a0Bu sendromun en \u00e7ok tercih edilen\u00a0 ve en efektif tedavisidir.<\/p>\n\n\n\n

3 a\u015famal\u0131 cerrahi onar\u0131m y\u00f6ntemi ( Norwood Prosed\u00fcr\u00fc) ilk olarak 1981\u2019de Dr. William Imon Norwood, Jr. ve arkada\u015flar\u0131 taraf\u0131ndan ba\u015far\u0131yla uygulanm\u0131\u015ft\u0131r.<\/p>\n\n\n\n

<\/p>\n\n\n\n

Norwood Prosed\u00fcr\u00fc<\/strong><\/h3>\n\n\n\n

1.A\u015fama: Norwood Ameliyat\u0131: <\/strong>Hipoplastik vaziyette olan aorta, pulmoner arter arac\u0131l\u0131\u011f\u0131 ile sa\u011f ventrik\u00fcle ba\u011flan\u0131r ve art\u0131k kalp tam anlam\u0131yla tek ventrik\u00fcll\u00fc hale gelmi\u015ftir. 2 atriyum aras\u0131 ge\u00e7i\u015fi devam ettirecek olan atriyal septektomi a\u00e7\u0131l\u0131r ve aorta ve pulmoner arter aras\u0131 kan ge\u00e7i\u015finin devaml\u0131l\u0131\u011f\u0131 i\u00e7in bir \u015fant yerle\u015ftirilir. Bu operasyon duktus arteriosus kapanmadan \u00f6nce yani do\u011fumdan k\u0131sa s\u00fcre sonra ger\u00e7ekle\u015ftirilmelidir.<\/p>\n\n\n\n

2.A\u015fama: Hemi-Fontan\/ Glenn Ameliyat\u0131:\u00a0\u00a0 <\/strong>Sa\u011fl\u0131kl\u0131 bir kalpte hem sa\u011f hem sol ventrik\u00fcl\u00fcn \u00fcstlendi\u011fi g\u00f6revi tek ba\u015f\u0131na \u00fcstlenen sa\u011f ventrik\u00fcl\u00fcn y\u00fck\u00fcn\u00fc azaltmak \u00e7ocu\u011fun fonksiyonel bir kalbe sahip olmas\u0131n\u0131 sa\u011flamak istiyorsak b\u00fcy\u00fck \u00f6nem arz etmektedir. Bu ama\u00e7la, prosed\u00fcr\u00fcn bu a\u015famas\u0131nda v\u00fccudun \u00fcst k\u0131sm\u0131ndan gelen deoksijene kan\u0131 kalbe ta\u015f\u0131yan superior vena kava direkt olarak kalpten sonraki dura\u011f\u0131 olan pulmoner artere ba\u011flan\u0131r.\u00a0 Bu operasyon bebek 4-6 ayl\u0131kken ger\u00e7ekle\u015ftirilir.<\/p>\n\n\n\n

3.A\u015fama: Fontan Ameliyat\u0131: <\/strong>Son a\u015fama olan bu ameliyatta, deoksijene kan\u0131n kalbe gelmesini sa\u011flayan di\u011fer bir b\u00fcy\u00fck damar olan inferior vena kava pulmoner artere direkt olarak ba\u011flan\u0131r ve sa\u011f ventrik\u00fcl sadece sistemik dola\u015f\u0131ma oksijene kan\u0131 pompalayan bir yap\u0131 haline gelerek asl\u0131nda sa\u011fl\u0131kl\u0131 bir kalpte sol ventrik\u00fcl\u00fcn g\u00f6revi olan bu hayati sorumlulu\u011fu \u00fczerine al\u0131r. Bu operasyon genellikle \u00e7ocuklar 4-5 ya\u015f\u0131na geldiklerinde yap\u0131l\u0131r.<\/p>\n\n\n\n

\"\"

Guseh, S., Friedman, K., & Wilkins\u2010Haug, L. (2020, January 09). OBGYN. Retrieved January 10, 2021, 418-425,from https:\/\/doi.org\/10.1002\/pd.5631<\/a><\/figcaption><\/figure>\n\n\n\n

<\/p>\n\n\n\n

Yukar\u0131daki operasyonlar sonucunda art\u0131k dola\u015f\u0131m\u0131 sa\u011flayabilen fonksiyonel bir kalp olu\u015fturulmu\u015f olur. Bu ameliyatlar\u0131n hepsi \u00e7e\u015fitli k\u0131sa- uzun vadeli komplikasyonlara gebe ve olduk\u00e7a zorlu ameliyatlar olsalar da do\u011fumdan \u00e7ok k\u0131sa s\u00fcre sonra kaybedilecek olan bu \u00e7ocuklara yeti\u015fkinlik \u00e7a\u011flar\u0131n\u0131 ya\u015fayabilme imkan\u0131 sunan mucizevi operasyonlard\u0131r.<\/p>\n\n\n\n

G\u00fcn\u00fcm\u00fczde t\u0131bb\u0131n ve cerrahinin geldi\u011fi noktada, ge\u00e7mi\u015f zamanlarda \u00e7aresiz oldu\u011fu d\u00fc\u015f\u00fcn\u00fclen bir\u00e7ok konjenital hastal\u0131k i\u00e7in bir \u00e7\u0131k\u0131\u015f yolu bulunmu\u015f olsa da daha ilerleyece\u011fimiz \u00e7ok yol bulunuyor. Hi\u00e7bir bebe\u011fin \u00f6lmedi\u011fi bir d\u00fcnya dile\u011fiyle\u2026<\/p>\n\n\n\n

Kaynak\u00e7a<\/strong><\/h2>\n\n\n\n

Jacobs, Marshall and William Norwood, Pediatric Cardiac Surgery Current Issues,1992<\/p>\n\n\n\n

Si, Ming Sing, Medscape, 2014<\/p>\n\n\n\n

Hypoplastic Left Heart Syndrome: Management and Outcome,UptoDate,Waltham,MA,2019<\/p>\n\n\n\n

Gobergs, Roberts, Elza Salputra, Inguna Lubaua, Hypoplastic Left Heart Syndrome: A Review, Acta Medica Lituanica, 2016<\/p>\n\n\n\n

Hypoplastic Left Heart Syndrome: Anatomy, clinical feautres and diagnosis, UptoDate, Waltham,MA,2019<\/p>\n\n\n\n

https:\/\/www.sciencedirect.com\/science\/article\/pii\/S1092912611000111<\/a>: Jonas, R. (2019). The Intra\/Extracardiac Conduit Fenestrated Fontan. https:\/\/doi.org\/10.1053\/j.pcsu.2011.01.010<\/a><\/p>\n\n\n\n

https:\/\/www.cdc.gov\/ncbddd\/heartdefects\/hlhs.html<\/a> https:\/\/emedicine.medscape.com\/article\/890196-overview<\/a>  Emedicine.medscape.com. (2019). Pediatric Hypoplastic Left Heart Syndrome: Background, Pathophysiology, Etiology<\/p>\n\n\n\n

https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC2817797\/<\/a>:Fruitman, D. (2019). Hypoplastic left heart syndrome: Prognosis and management options. https:\/\/dx.doi.org\/10.1093%2Fpch%2F5.4.219<\/a><\/p>\n\n\n\n

https:\/\/www.cambridge.org\/core\/journals\/cardiology-in-the-young\/article\/hypoplastic-left-heart-syndrome-from-fetus-to-fontan\/055F6F897A4490A30B5D80043B1128B1\/core-reader<\/a>:Roeleveld, P., Axelrod, D., Klugman, D., Jones, M., Chanani, N., Rossano, J. and Costello, J. (2019). Hypoplastic left heart syndrome: from fetus to fontan. https:\/\/doi.org\/10.1017\/S104795111800135X<\/a><\/p>\n\n\n\n

57 Long-Term Neurologic Outcomes in Children With Congenital Heart Disease, ( 2018) Peyvandi S, Mcquillen P, Avery’s Diseases of the Newborn https:\/\/www.sciencedirect.com\/topics\/biochemistry-genetics-and-molecular-biology\/fetus-circulation<\/a><\/p>\n\n\n\n

Hypoplastic Left Heart Syndrome, (2013), Blackwell W., Pediatric Cardiac Surgery, Fourth Edition<\/p>\n","protected":false},"excerpt":{"rendered":"

Hipoplastik sol kalp, geli\u015fmemi\u015f bir sol ventrik\u00fcl ve sistemik dola\u015f\u0131m\u0131 sa\u011flama yetisinden yoksun sol kalp yap\u0131lar\u0131 ile karakterize edilmi\u015f konjenital bir sendromdur. <\/p>\n","protected":false},"author":390,"featured_media":9528,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[1,25],"tags":[1725,1726,1727,1728],"acf":[],"views":1739,"_links":{"self":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/9526"}],"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\/390"}],"replies":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/comments?post=9526"}],"version-history":[{"count":5,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/9526\/revisions"}],"predecessor-version":[{"id":9535,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/9526\/revisions\/9535"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media\/9528"}],"wp:attachment":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media?parent=9526"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/categories?post=9526"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/tags?post=9526"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}