2020
Subaortic Membranes in Patients With Hereditary Hemorrhagic Telangiectasia and Liver Vascular Malformations
Kim AS, Henderson KJ, Pawar S, Kim MJ, Punjani S, Pollak JS, Fahey JT, Garcia‐Tsao G, Sugeng L, Young LH. Subaortic Membranes in Patients With Hereditary Hemorrhagic Telangiectasia and Liver Vascular Malformations. Journal Of The American Heart Association 2020, 9: e016197. PMID: 33054561, PMCID: PMC7763373, DOI: 10.1161/jaha.120.016197.Peer-Reviewed Original ResearchMeSH KeywordsActivin Receptors, Type IICardiac Output, HighDiscrete Subaortic StenosisEchocardiographyFemaleHeart Defects, CongenitalHeart FailureHumansLiverMaleMiddle AgedMutationPrognosisRetrospective StudiesSurvival AnalysisTelangiectasia, Hereditary HemorrhagicUnited StatesVascular MalformationsConceptsHigh-output cardiac failureHereditary hemorrhagic telangiectasiaLeft ventricular outflow tractVentricular outflow tractHemorrhagic telangiectasiaMild obstructionSubaortic membraneVascular malformationsOutflow tractActivin receptor-like kinase 1 mutationsHereditary hemorrhagic telangiectasia patientsLiver vascular malformationsMild aortic insufficiencyPulmonary artery pressureRight heart catheterizationCohort of patientsRetrospective observational analysisHigh cardiac outputKinase 1 mutationsArtery pressureHeart catheterizationPulmonary hypertensionAortic insufficiencyBackground PatientsTricuspid regurgitation
2008
Hepatic Vascular Malformations in Hereditary Hemorrhagic Telangiectasia
Khalid S, Garcia-Tsao G. Hepatic Vascular Malformations in Hereditary Hemorrhagic Telangiectasia. Seminars In Liver Disease 2008, 28: 247-258. PMID: 18814078, DOI: 10.1055/s-0028-1085093.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsHereditary hemorrhagic telangiectasiaHepatic arteryVascular malformationsHepatic veinPortal veinHemorrhagic telangiectasiaHigh-output cardiac failureAbnormal vascular communicationAggressive medical therapyHepatic vascular malformationsPredominant clinical presentationCommon hepatic arteryNodular regenerative hyperplasiaIntensive medical treatmentFocal nodular hyperplasiaAutosomal dominant vascular disorderAbdominal anginaBiliary ischemiaLiver transplantationPortal hypertensionRegenerative hyperplasiaSymptomatic patientsCommon presentationMedical therapyCardiac failure
2000
Liver Disease in Patients with Hereditary Hemorrhagic Telangiectasia
Garcia-Tsao G, Korzenik J, Young L, Henderson K, Jain D, Byrd B, Pollak J, White R. Liver Disease in Patients with Hereditary Hemorrhagic Telangiectasia. New England Journal Of Medicine 2000, 343: 931-936. PMID: 11006369, DOI: 10.1056/nejm200009283431305.Peer-Reviewed Original ResearchConceptsHereditary hemorrhagic telangiectasiaHigh-output heart failureHemorrhagic telangiectasiaHeart failureMedian periodPortal hypertensionLiver involvementClinical findingsBiliary diseasePulmonary capillary wedge pressureElevated alkaline phosphatase levelsHepatic sinusoidal pressureSymptomatic liver involvementTypical clinical presentationAlkaline phosphatase levelsAutosomal dominant disorderHyperdynamic circulationAngiodysplastic lesionsCardiac indexLiver transplantationVariceal bleedingWedge pressureMedical therapyRendu-OslerClinical presentation