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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorLandi, Filippo
dc.contributor.authorSandoval, Elena
dc.contributor.authorMartinez, Julia
dc.contributor.authorBlasi, Annabel
dc.contributor.authorArguis, Maria Jose
dc.contributor.authorColmenero, Jordi
dc.contributor.authorDos Subirá, Laura
dc.date.accessioned2022-03-10T07:37:23Z
dc.date.available2022-03-10T07:37:23Z
dc.date.issued2021-11
dc.identifier.citationLandi F, Sandoval E, Martinez J, Blasi A, Arguis MJ, Colmenero J, et al. Combined Heart and Liver Transplantation for Uhl’s Anomaly: A Case Report. Transplant Proc. 2021 Nov;53(9):2751–3.
dc.identifier.issn0041-1345
dc.identifier.urihttp://hdl.handle.net/11351/7132
dc.descriptionCombined heart-liver transplant; Cardiomyopathy
dc.description.abstractBackground Uhl's anomaly is an extremely rare congenital heart defect characterized by absence of the right ventricle myocardium and preserved left ventricular myocardium. Although the disease has a poor prognosis and is generally fatal in the perinatal period, some patients may reach adulthood. Methods We describe a case of Uhl's anomaly complicated with heart failure and decompensated cardiac cirrhosis in a 42-year-old man treated by combined heart-liver transplant. Results The patient underwent heart transplant using the bicaval technique followed by subsequent liver transplant with the piggyback technique without venovenous bypass. Total ischemia time was 108 minutes for the heart and 360 and 25 minutes of cold and warm ischemia, respectively, for the liver. No intraoperative complications occurred. The patient was discharged without severe complications on postoperative day 22. Pathologic examination of the organs reported advanced cirrhosis of the liver and severe dilated myocardiopathy of right ventricle with absence of myocardium and a normal left ventricle. Twenty-seven months after the transplant the patient has been free from hospital admissions, with normal function of both transplanted organs. Conclusions We report the first successful combined heart-liver transplant for Uhl's anomaly indication in an adult patient. Despite of the insufficient knowledge of natural history of this exceptional disease, we successfully apply the management principles of other end-stage right heart disorders complicated with liver failure.
dc.language.isoeng
dc.publisherElsevier
dc.relation.ispartofseriesTransplantation Proceedings;53(9)
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.sourceScientia
dc.subjectCardiopatia congènita
dc.subjectCor - Trasplantació
dc.subjectFetge - Trasplantació
dc.subject.meshHeart Defects, Congenital
dc.subject.meshOrgan Transplantation
dc.titleCombined Heart and Liver Transplantation for Uhl's Anomaly: A Case Report
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1016/j.transproceed.2021.08.036
dc.subject.decscardiopatías congénitas
dc.subject.decstrasplante de órganos
dc.relation.publishversionhttps://doi.org/10.1016/j.transproceed.2021.08.036
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[Landi F] Department of General and Digestive Surgery, Hospital Clinic, CIBEREHD, IDIBAPS, University of Barcelona, Barcelona, Spain. [Sandoval E] Department of Cardiovascular Surgery, Hospital Clinic, University of Barcelona, Barcelona, Spain. [Martinez J, Blasi A, Arguis MJ] Department of Anesthesiology, Hospital Clinic, University of Barcelona, Barcelona, Spain. [Colmenero J] Department of Hepatology, Hospital Clinic, University of Barcelona, Barcelona, Spain. [Dos L] Servei de Cardiologia, Unitat de Cardiopaties Congènites de l’Adult, Vall d'Hebron Hospital Universitari, Barcelona, Spain. Sant Pau Hospitals, Barcelona, Spain. Grup de Recerca en Malalties Cardiovasculars, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. CIBERCV, Barcelona, Spain
dc.identifier.pmid34593248
dc.identifier.wos000755390700028
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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