Hypoplastic right heart syndrome is a congenital cardiac anomaly. It is characterized by an underdeveloped right side of the heart, including the right ventricle, ...×ArticlesLogInCasesSignUpCoursesQuizBlogDonateAbout×MenuSearchADVERTISEMENT:Radiopaediaisfreethankstooursupportersandadvertisers.BecomeaGoldSupporterandseenoads.ArticlesCasesCoursesLogInLoginSignup{"url":"/signup-modal-props.json?lang=us\u0026email="}ArticlesCasesCoursesQuizAboutBlogGoad-freeSearchHypoplasticrightheartsyndromeDrMostafaEl-Feky◉andDrYurangaWeerakkody◉etal.Hypoplasticrightheartsyndromeisacongenitalcardiacanomaly. Itischaracterizedbyanunderdevelopedrightsideoftheheart,includingtherightventricle,tricuspidvalve,pulmonaryvalve,andpulmonaryarteries.Onthispage:Article:EpidemiologyClinicalpresentationPathologyRadiographicfeaturesReferencesImages:CasesandfiguresEpidemiologyItmaybepresentinaround1.1%ofstillbirthsandisrarerthanthehypoplasticleftheartsyndrome.ClinicalpresentationNeonatesarecyanoticatbirth.PathologyItiscausedbydecreasedbloodflowinandoutoftherightventricleduringdevelopment.Themostcommonright-sideddefecttocausethissyndromeispulmonaryatresiawithanintactventricularseptum.Coronaryarterialsupplymaybealsoabnormal.RadiographicfeaturesFetalechocardiographyPrenatalultrasoundmayshowasmallrightventriclewithhypertrophyandasmallorabsentpulmonaryartery,withdecreasedorabsentflowthroughthetricuspidvalveandpulmonaryvalve1.References1.RajiahP,MakC,DubinksyTJ,DigheM.Ultrasoundoffetalcardiacanomalies.(2011)AJR.Americanjournalofroentgenology.197(4):W747-60.doi:10.2214/AJR.10.7287-Pubmed2.MohanJC,MohanV,ShuklaM,SethiA.Hypoplasticrightheartsyndrome,absentpulmonaryvalve,andnon-compactedleftventricleinanadult.(2016)Indianheartjournal.68Suppl2:S229-S232.doi:10.1016/j.ihj.2016.03.030-Pubmed3.KadamPG,ShahVR,SaikiaK,PatelRR.ResuscitationofaNewbornwithRightHypoplasticHeartSyndrome:ARareConfrontationforanAnesthesiologist.(2017)Anesthesia,essaysandresearches.11(4):1124-1125.doi:10.4103/0259-1162.194592-Pubmed4.Jacobs