Identification of clinically relevant phenotypes in patients with Ebstein anomaly

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dc.contributor.authorCabrera, Rodrigo
dc.contributor.authorCarreño, Marisol
dc.contributor.authorTamar Silva, Claudia
dc.contributor.authorManrique, Diana Carolina
dc.contributor.authorCamacho, Camila
dc.contributor.authorTabares, Sebastián
dc.contributor.authorGarcía, Alberto
dc.contributor.authorMiranda-Fernández, Marta-Catalina
dc.contributor.authorHuertas Quiñones, Victor Manuel
dc.contributor.authorPineda Rodriguez, Ivonne Gisel
dc.contributor.authorRestrepo, Carlos M.
dc.contributor.authorQuero, Rossi
dc.contributor.authorSandoval, Nestor F.
dc.contributor.authorMoreno-Medina, Karen
dc.contributor.authorCano, Juan
dc.contributor.authorDennis, Rodolfo J.
dc.date.accessioned2019-08-05T18:36:36Z
dc.date.available2019-08-05T18:36:36Z
dc.date.issued2018
dc.description.abstractenglishBackground 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.eng
dc.format.mimetypeapplication/pdf
dc.identifier.doihttps://doi.org/10.1002/clc.22870
dc.identifier.instnameinstname:Universidad El Bosquespa
dc.identifier.issn1932-8737
dc.identifier.reponamereponame:Repositorio Institucional Universidad El Bosquespa
dc.identifier.repourlrepourl:https://repositorio.unbosque.edu.co
dc.identifier.urihttps://hdl.handle.net/20.500.12495/1595
dc.language.isoeng
dc.publisherWileyspa
dc.publisher.journalClinical Cardiologyspa
dc.relation.ispartofseriesClinical Cardiology, 1932-8737, Vol. 41, Num. 3, 2018, p. 343-348spa
dc.relation.urihttps://onlinelibrary.wiley.com/doi/full/10.1002/clc.22870
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess
dc.rights.accessrightshttps://purl.org/coar/access_right/c_abf48
dc.rights.creativecommons2018
dc.rights.localAcceso cerradospa
dc.subject.decsEnfermedades cardiovascularesspa
dc.subject.decsAnomalía de Ebsteinspa
dc.subject.decsEpidemiologíaspa
dc.subject.keywordsCongenital Heart Defectspa
dc.subject.keywordsEbstein Anomalyspa
dc.subject.keywordsEpidemiologyspa
dc.titleIdentification of clinically relevant phenotypes in patients with Ebstein anomalyspa
dc.typearticlespa
dc.type.hasversioninfo:eu-repo/semantics/publishedVersion
dc.type.localartículospa

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