Uspešno hirurško zatvaranje aortopulmonalnog prozora udruženog sa Holt-Oramovim sindromom u odraslom dobu
Sažetak
Apstrakt
Uvod. Aortopulmonalni prozor (aortopulmonary window – APW) je retka kongenitalna anomalija uzrokovana nepotpunim razdvajanjem zajedničkog embrionalnog arterijskog stable koja se manifestuje direktnom i neuobičajenom komunikacijom između aorte i plućne arterije. Holt-Oramov sindrom je antozomno-dominantni poremećaj uzrokovan mutacijom TBX5 gena koga odlikuju anomalije kostiju bar na jednom od gornjih ekstremiteta. Njegova udružennost sa APW je veoma retka. Prikaz bolesnika. U radu je prikazana bolesnica u četvrtoj deceniji života sa vrlo retkom kombinacijom Holt-Oramovog sindroma i APW koji nije bio zatvoren do odraslog doba. Bolesnica je hospitalizovana sa znacima teške srčane insuficijencije i plućne hipertenzije. Iako prethodno dijagnostikovana kao inoperabilna, posle odgovarajućeg medicinskog tretmana i detaljnih dijagnostičkih procedura dokazana je još uvek značajno reaktivna plućna vaskularna rezistencija nakon čega je bolesnica uspešno operisana. Zaključak. Ovaj slučaj potvrđuje neophodnost pažljive i sveobuhvatne dijagnostike bolesnika sa hemodinamski značajnim urođenim srčanim manama i sekundarnom plućnom hipertenzijom bez obzira na njihovo životno doba.
Reference
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