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Identification of clinically relevant phenotypes in patients with Ebstein anomaly

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dc.contributor.gruplacGENIUROSspa
dc.creatorCabrera, Rodrigo
dc.creatorMiranda-Fernández, Marta-Catalina
dc.creatorHuertas Quiñones, Victor Manuel
dc.creatorCarreño, Marisol
dc.creatorPineda, Ivonne
dc.creatorRestrepo Fernández, Carlos Martín
dc.creatorSilva, Claudia Tamar
dc.creatorQuero, Rossi
dc.creatorCano, Juan David
dc.creatorCabrera, Rodrigo
dc.creatorMiranda-Fernández, Marta-Catalina
dc.creatorHuertas Quiñones, Victor Manuel
dc.creatorCarreño, Marisol
dc.creatorPineda, Ivonne
dc.creatorRestrepo Fernández, Carlos Martín
dc.creatorSilva, Claudia Tamar
dc.creatorQuero, Rossi
dc.creatorCano, Juan David
dc.creatorManrique, Diana Carolina
dc.creatorCamacho, Camila
dc.creatorTabares, Sebastián
dc.creatorGarcía, Alberto
dc.creatorSandoval, Néstor
dc.creatorMoreno-Medina, Karen
dc.creatorDennis Verano, Rodolfo José
dc.creator.googleCabrera, Rodrigospa
dc.creator.googleMiranda-Fernández, Marta Catalina
dc.creator.googleHuertas-Quiñones, Victor Manuel
dc.creator.googleCarreño, Marisol
dc.creator.googlePineda, Ivonne
dc.creator.googleRestrepo, Carlos M.
dc.creator.googleSilva, Claudia Tamar
dc.creator.googleQuero, Rossi
dc.creator.googleCano, Juan David
dc.creator.googleManrique, Diana Carolina
dc.creator.googleCamacho, Camila
dc.creator.googleTabares, Sebastián
dc.creator.googleGarcía, Alberto
dc.creator.googleSandoval, Néstor
dc.creator.googleMoreno Medina, Karen Julieth
dc.creator.googleDennis Verano, Rodolfo José
dc.date.accessioned2019-10-11T20:46:18Z
dc.date.available2019-10-11T20:46:18Z
dc.date.created2018
dc.date.issued2018
dc.description.abstractBackground: Ebstein anomaly (EA) is a heterogeneous congenital heart defect (CHD), frequently accompanied by diverse cardiac and extracardiac comorbidities, resulting in a wide range of clinical outcomes. Hypothesis: Phenotypic characterization of EA patients has the potential to identify variables that influence prognosis and subgroups with distinct contributing factors. Methods: A comprehensive cross-sectional phenotypic characterization of 147 EA patients from one of the main referral institutions for CHD in Colombia was carried out. The most prevalent comorbidities and distinct subgroups within the patient cohort were identified through cluster analysis. Results: The most prevalent cardiac comorbidities identified were atrial septal defect (61%), Wolff-Parkinson-White syndrome (WPW; 27%), and right ventricular outflow tract obstruction (25%). Cluster analysis showed that patients can be classified into 2 distinct subgroups with defined phenotypes that determine disease severity and survival. Patients in cluster 1 represented a particularly homogeneous subgroup with a milder spectrum of disease, including only patients with WPW and/or supraventricular tachycardia (SVT). Cluster 2 included patients with more diverse cardiovascular comorbidities. Conclusions: This study represents one of the largest phenotypic characterizations of EA patients reported. The data show that EA is a heterogeneous disease, very frequently associated with cardiovascular and noncardiovascular comorbidities. Patients with WPW and SVT represent a homogeneous subgroup that presents with a less severe spectrum of disease and better survival when adequately managed. This should be considered when searching for genetic causes of EA and in the clinical setting. © 2018 Wiley Periodicals, Inc.eng
dc.format.mimetypeapplication/pdf
dc.identifier.doi10.1002/clc.22870
dc.identifier.issn0160-9289
dc.identifier.urihttps://repository.urosario.edu.co/handle/10336/20434
dc.language.isoengspa
dc.relation.citationEndPage348
dc.relation.citationStartPage343
dc.relation.citationTitleClinical Cardiology
dc.relation.citationVolumeVol. 41
dc.relation.ispartofClinical Cardiology, ISSN:1609-289, Vol. 41 (2018) pp. 343-348spa
dc.relation.urihttps://onlinelibrary.wiley.com/doi/abs/10.1002/clc.22870spa
dc.rights.accesRightsinfo:eu-repo/semantics/openAccess
dc.rights.accesoAbierto (Texto Completo)spa
dc.source.bibliographicCitationKouchoukos, N.T., Kirklin, J.W., Ebstein anomaly (2013) Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results, and Indications, pp. 1576-1598. , In, Kouchoukos NT, Blackstone EH, Hanley FL, eds., 4th ed, Philadelphia, PA, Elsevier/Saundersspa
dc.source.instnameinstname:Universidad del Rosario
dc.source.reponamereponame:Repositorio Institucional EdocUR
dc.subjectFurosemidespa
dc.subjectSpironolactonespa
dc.subjectAdolescentspa
dc.subjectCatheter Ablationspa
dc.subjectChildspa
dc.subjectCluster Analysisspa
dc.subjectCohort Analysisspa
dc.subjectColombiaspa
dc.subjectCross-Sectional Studyspa
dc.subjectDisease Severityspa
dc.subjectEbstein Anomalyspa
dc.subjectEndocardial Cushion Defectspa
dc.subjectFallot Tetralogyspa
dc.subjectFontan Procedurespa
dc.subjectHealth Care Facilityspa
dc.subjectHeart Atrium Septum Defectspa
dc.subjectHeart Failurespa
dc.subjectHeart Right Bundle Branch Blockspa
dc.subjectHeart Right Ventricle Outflow Tract Obstructionspa
dc.subjectHeart Transplantationspa
dc.subjectHumanspa
dc.subjectInfantspa
dc.subjectMajor Clinical Studyspa
dc.subjectMalespa
dc.subjectMortalityspa
dc.subjectNewbornspa
dc.subjectObservational Studyspa
dc.subjectPacemaker Implantationspa
dc.subjectPatent Ductus Arteriosusspa
dc.subjectPatent Foramen Ovalespa
dc.subjectPatient Referralspa
dc.subjectPhenotypespa
dc.subjectPreschool Childspa
dc.subjectPrognosisspa
dc.subjectPulmonary Valve Atresiaspa
dc.subjectPulmonary Valve Replacementspa
dc.subjectPulmonary Valve Stenosisspa
dc.subjectSchool Childspa
dc.subjectSupraventricular Tachycardiaspa
dc.subjectSurvival Ratespa
dc.subjectTricuspid Valve Repairspa
dc.subjectTricuspid Valve Replacementspa
dc.subjectWolff Parkinson White Syndromespa
dc.subjectEbstein Anomalyspa
dc.subjectEchocardiographyspa
dc.subjectElectrocardiographyspa
dc.subjectHeart Septum Defectspa
dc.subjectPhenotypespa
dc.subjectSupraventricular Tachycardiaspa
dc.subjectTrendsspa
dc.subjectWolff Parkinson White Syndromespa
dc.subjectYoung Adultspa
dc.subjectAdolescentspa
dc.subjectChildspa
dc.subjectChildspa
dc.subjectColombiaspa
dc.subjectCross-Sectional Studiesspa
dc.subjectEbstein Anomalyspa
dc.subjectEchocardiographyspa
dc.subjectElectrocardiographyspa
dc.subjectHeart Septal Defectsspa
dc.subjectInfantspa
dc.subjectInfantspa
dc.subjectMalespa
dc.subjectPhenotypespa
dc.subjectSurvival Ratespa
dc.subjectTachycardiaspa
dc.subjectWolff-Parkinson-White Syndromespa
dc.subjectYoung Adultspa
dc.subjectFurosemidaspa
dc.subjectLa espironolactonaspa
dc.subjectAdolescentespa
dc.subjectAdultospa
dc.subject.ddcEnfermedadesspa
dc.subject.keywordCongenital heart defectspa
dc.subject.keywordEbstein anomalyspa
dc.subject.keywordEpidemiologyspa
dc.subject.keywordAdultspa
dc.subject.keywordArticlespa
dc.subject.keywordComorbidityspa
dc.subject.keywordFemalespa
dc.subject.keywordMiddle Agedspa
dc.subject.keywordAdultspa
dc.subject.keywordComorbidityspa
dc.subject.keywordFemalespa
dc.subject.keywordHumansspa
dc.subject.keywordMiddle Agedspa
dc.subject.lembCardiopatías congénitasspa
dc.subject.lembAnormalidades del corazónspa
dc.subject.lembAnomalía de Ebsteinspa
dc.titleIdentification of clinically relevant phenotypes in patients with Ebstein anomalyspa
dc.typearticleeng
dc.type.hasVersioninfo:eu-repo/semantics/publishedVersion
dc.type.spaArtículospa
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