简介：Hypertrophiccardiomyopathy(HCM)isacommongeneticcardiovasculardiseaseandappearsinallethnicgroups.HCMisdiagnosedonthebasisofleftventricularhypertrophy.EchocardiographyisakeytechniqueinthediagnosisofHCM,theprognosisofpatientswithHCM,themanagementstrategyforHCM,andthefollow-upofpatientswithHCM.ThisreviewbrieflydescribesanddiscussesthepracticaluseofestablishedechocardiographytechniquesandthecurrentandemergingechocardiographicmethodsthatcanhelpphysiciansinthecorrectdiagnosticandpathophysiologicalassessmentofpatientswithHCM.

简介：AbstractDesmoplakin (DSP), encoded by the DSP gene, is the main desmosome component and is abundant in the myocardial tissue. There are three DSP isoforms that assume the role of supporting structural stability through intercellular adhesion. It has been found that DSP regulates the transcription of adipogenic and fibrogenic genes, and maintains appropriate electrical conductivity by regulating gap junctions and ion channels. DSP is essential for normal myocardial development and the maintenance of its structural functions. Studies have suggested that DSP gene mutations are associated with a variety of hereditary cardiomyopathy, such as arrhythmia cardiomyopathy, dilated cardiomyopathy (DCM), left ventricular noncompaction, and is also closely associated with the Carvajal syndrome, Naxos disease, and erythro-keratodermia-cardiomyopathy syndrome with skin and heart damage. The structure and function of DSP, as well as the clinical manifestations of DSP-related cardiomyopathy were reviewed in this article.

简介：Multiplemyeloma(MM)isamalignancyoftheplasmacellcharacterizedbymigrationandlocalizationtothebonemarrowwherecellsthendisseminateandfacilitatetheformationofbonelesions.Itisassociatedwithaconstellationofdiseasemanifestations,apartfromosteolyticlesions,anemiaandimmuno-suppressionduetolossofnormalhematopoieticstemcellfunction,andcardiacamyloidosisduetomonoclonalimmunoglobulinsecretionaswell[1].Amyloidinfiltrationoftheheartmayfrequentlymasqueradeashypertrophiccardiomyopathy(HCM).HCM,ofwhichunderlyingcauseandpathogenesisarelargelyunknown,ischaracterizedbyleftand/orrightventricularhypertrophy,withpredominantinvolvementoftheinterventricularseptumintheabsenceofothercausesofhypertrophy,suchashypertensionorvalvularheartdiseases[2].Whileexcessivehypertrophyofthemyocardiumismostcommonlyassociatedwithmyocytehypertrophy,infiltrationwithamyloidalwaysneedstobeconsidered.Inthisreportwepresentedtwocasesofmultiplemyelomathatmimickedhypertrophiccardiomyopathysocloselythatitrequiredbonemarroworendomyocardialbiopsytoestablishthediagnosis.

简介：AbstractBackground:The detrimental outcomes of right ventricular pacing on left ventricular electromechanical function ultimately result in heart failure, a phenomenon termed pacing-induced cardiomyopathy (PICM) in clinical research. This study aimed to validate prognostic factors that can be used to identify patients with higher susceptibility to progress to the stage of cardiomyopathy before pacemaker implantation.Methods:This observational analysis enrolled 256 patients between January 2013 and June 2016, 23 (8.98%) of whom progressed to PICM after 1 year of follow-up. A Cox proportional hazard model was used to analyze the prognostic factors associated with PICM. Dose-response analysis was used to evaluate the relationship between significant indicators in multifactor analysis and PICM.Results:The mean values of left ventricular ejection fraction before and after pacemaker implantation in 23 patients diagnosed with PICM were 62.3% and 42.7%, respectively. Univariate analysis showed that sex, atrio-ventricular block, paced QRS duration, and ventricular pacing percentage were significantly associated with PICM. In the multivariate analysis, male sex (hazard ratio: 1.20, 95% confidence interval [CI]: 1.09-1.33, P < 0.005), paced QRS duration (hazard ratio: 1.95 per 1 ms increase, 95% CI: 1.80-2.12, P < 0.001), and ventricular pacing percentage (hazard ratio: 1.65 per 1% increase, 95% CI: 1.51-1.79, P < 0.001) were independent prognostic factors associated with the development of PICM. The ventricular pacing percentage and paced QRS duration level defined by the dose-response analysis were positively associated with PICM (P < 0.05).Conclusions:Our findings indicated that paced QRS duration and ventricular pacing percentage were the most sensitive prognostic factors for PICM.

简介：ObjectivesToinvestigatetheclinicalmanifestationsofhypertrophiccardiomyopathy(HCM),andtofindouttheclinicalcluestoavoidmisdiagnosisandprovidereferenceforfutureclinicaldiagnosisandtreatment.MethodsAretrospectiveanalysisof42consecutivepatientswithHCMhospitalizedinourhospitalbetweenJanuary1995andDecember2002wasexplored.BasedonthefamilyhistoryofHCM,clinicalmanifestations,electrocar-diogram,echocardiogram,coronaryangiographyandleftventriculography,theclinicalcharacteristicsbetweenHCMpatientswithleftventricularoutflowtractobstruction(HOCM)andHCMpatientswithoutobstruction(HNOCM)werecompared.Thecausesofmisdiagnosisandlosingdiagnosiswereanalysis.Results13patientswereinHOCMgroupand29patientswereinHNOCMgroup.MorepatientswithsyncopewereinHOCMgroupthaninHNOCMgroup(6/13vs.2/29,P<0.05).PatientswithejectivemurmurwereinHOCMgrouponly(P<0.01).Leftventricularoutflowtractpressuregradient(LVOTPG)onlyobservedinHOCMgroup(P<0.01).Ventriculartachycardiawasseeninbothgroups.28outof42patients(66.67%)hadmisdiagnosis,and4outof42patients(9.53%)hadlosingdiagnosis.Thus,coronaryheartdisease(CHD)hadthehighestrateofmisdiagnosis.Therewere20CHDpatients(71.43%)among28patientswithmisdiagnosis.Hypertensionwasin3,congenitalheartin2,cerebro-embolismin2,andmyocarditisin1.ConclusionsForapatientwithfamilyhistoryorsuddendeathhistoryofHCM,unexplainedsyncopeepisodes,chestpain(angina),especiallyinyoung,anejectionmurmuralongtheleftsternumborder,thepresenceofnarrowanddeepQwaves,orinversionofgiantTwavesinV3-V6,atrialfibrillationand/orcerebra-embolismechocardiogramshouldbegiven.CAGandLVGarenecessaryonlyiftheresultofechoisnegative,andthepatientswithsuspectedHCMorCHD.

简介：无

简介：AbstractBackground:The association between free triiodothyronine (FT3) and long-term prognosis in dilated cardiomyopathy (DCM) patients has not been evaluated. The purpose of this study was to determine whether the level of FT3 could provide prognostic value in patients with DCM.Methods:Data of consecutive patients diagnosed with DCM were collected from October 2009 to December 2014. FT3 was measured by fluoroimmunoassay. Other biochemical markers, such as free thyroxin (FT4), thyroid-stimulating hormone, red blood cell, hemoglobin, blood urea nitrogen, and serum creatinine, were tested at the same time. Follow-up was performed every 3 months. The primary endpoint was all-cause mortality. Pearson analysis was used to evaluate the correlation of FT3 and other lab metrics with DCM patients’ prognosis. The association of long-term mortality in DCM and FT3 was compared using Cox hazards model.Results:Data of 176 patients diagnosed with DCM were collected. Of them, 24 patients missed FT3 values and six patients were lost to follow-up. Altogether, data of 146 patients were analyzed. During the median follow-up time of 79.9 (53.5-159.6) months, nine patients lost, 61 patients died (non-survival group), and 85 patients survived (survival group). FT3 was significantly lower in non-survival group than that in survival group (3.65 ± 0.83 pmol/L vs. 4.36 ± 1.91 pmol/L; P = 0.003). FT3 also showed a significantly positive correlation with red blood cell and hemoglobin, negatively correlated with age, blood urea nitrogen and serum creatinine (P < 0.05), respectively. Patients in the group of lower FT3 levels (FT3 ≤ 3.49 pmol/L) suffered from a higher risk of all-cause mortality (P for log-rank = 0.001). In multivariate Cox regression analysis, FT3 level was significantly associated with all-cause mortality (hazard ratio: 0.70, 95% confidence interval 0.52-0.95, P for trend = 0.021).Conclusion:Low levels of FT3 were associated with increased all-cause mortality in patients with DCM.

简介：Peripartumcardiomyopathy(PPCM)isadisorderinwhichheartfailuredevelopsinthelastmonthofpregnancyorwithinthefirstfivemonthspostpartum.Theexactetiologyisnotknownalthoughrecentstudiessuggestangiogenicimbalanceisakeyfactorwithsolublefms-liketyrosinekinase-1(sFlt1)andacleavedformofprolactinpossiblyplayingimportantroles.Thisreviewdiscussestheepidemiology,riskfactors,diagnosis,treatmentandprognosisofPPCMandhighlightsrecentadvancesinourunderstandingofthisdisorder.

简介：ObjectivesPlasmauricacid(UA)concentrationwassuspectedtoelevateinelderlywithischemiccardiomyopathy(ICM).MethodsWeanalyzedthedataof235elderlyaged60yearsandolderwithcoronaryheartdisease:silentmyocardialischemiaoranginapectorisconfirmedbyangiography.Amongthesepatients,154hadICMdefinedasleftventricularend-diastolicdiameter(LVDd)male>55mm,female>50mm(mean:63.51±7.70mm)measuredbyechocardiography.DifferenceinUAwasanalyzedbetweenpatientswithandwithoutICM.ResultsTherewassignificantincreaseofUAinICMcomparedwithnon-ICM(432.82±143.05umol/Lvs361.06±137.35umol/L,P<0.05);andUAwaspositivelyrelatedtoLVDd(r=0.25,P<0.05).ConclusionsTherewassignificantincreaseofUAinelderlywithICMduetolongtermsilentmyocardialischemiaandanginapectoris.Moreover,UAwaspositivelyrelatedtoLVDd.

简介：Majoradvanceshavebeenmadeoverthelastdecadeinourunderstandingofthemolecularbasisofseveralcardiacconditions.Hypertrophiccardiomyopathy(HCM)wasthefirstcardiacdisorderinwhichageneticbasiswasidentifiedandassuch,hasactedasaparadigmforthestudyofaninheritedcardiacdisorder.HCMcanresultinclinicalsymptomsrangingfromnosymptomstosevereheartfailureandprematuresuddendeath.HCMisthecommonestcauseofsuddendeathinthoseagedlessthan35years,includingcompetitiveathletes.Atleasttengeneshavenowbeenidentified,defectsinwhichcauseHCM.Allofthesegenesencodeproteinswhichcomprisethebasiccontractileunitoftheheart,i.e.thesarcomere.Whilemuchisnowknownaboutwhichgenescausediseaseandthevariousclinicalpresentations,verylittleisknownabouthowthesegenedefectscausedisease,andwhatfactorsmodifytheexpressionofthemutantgenes.StudiesinbothcellcultureandanimalmodelsofHCMarenowbeginningtoshedlightonthesignallingpathwaysinvolvedinHCM,andtheroleofbothenvironmentalandgeneticmodifyingfactors.Understandingthesemechanismswillultimatelyimproveourknowledgeofthebasicbiologyofheartmusclefunction,andwillthereforeprovidenewavenuesfortreatingcardiovasculardiseaseinman.

简介：ObjectivesToinvestigatetheprevalenceofhypertensionanditsprimaryriskfactorsinpatientswithdilatedcardiomyopathy(DCM).MethodsThreehundredandsixty-twopatientswithDCM(DCMgroup)and401age-matchedresidents(controlgroup)wereenrolledrandomlyinthestudy,thehypertensiveprevalenceratewerecalculatedrespectivelyinthetwogroupsandwerecomparedwitheachother;thepatientsintheDCMgroupweredividedintotwosubgroups(hypertensionsubgroupandnon-hypertensionsubgroup)accordingtowhetherthepatientshavehypertension;theclinicaldatarelatedtobloodpressurewascomparedbetweenthetwosubgroups.ResultsTheprevalenceofhypertensioninDCMgroupwassignificantlyhigherthanthatinthecontrolgroup(32.8%vs.20.1%,P<0.01);Therewerenosignificantdifferencesontheage,gender,occupationandleftventricularejectionfraction(LVEF)betweenthetwosubgroups,butthemeanheartrateandthepercentageofpatientswhohadfamilyhistoryofhypertensionweresignificantlyhigherinthehypertensionsubgroupthanthatinthenon-hypertensionsubgroup(P<0.05andP<0.01).ConclusionsTheprevalenceofhypertensioninpatientswithDCMwashigh;TheincreasedactivityofsympatheticnervoussystemandthehypertensivegeneticfactormaybethemainriskfactorsofhypertensioninpatientswithDCM.

简介：ObjectivesItisnotfullyclarifiedhowdiabetesmellitusinducedcardiacdysfunctionandmyocardialultrastructuralchangesintheearlystate.Inthepresentstudy,weprovidedanintegratedapproachtoinvestigateearlychangesinmyocardialfunctionofdiabeticrabbitsandassessedthestructuralalteration.MethodsandResultsDiabeteswasinducedbyalloxaninjection.After30days,echocardio-graphyandleftventricularcannulationwereperformedindia-betic(D,n=8)andcontrolrabbits(C,n=10).Aftercatheterization,animalswerekilledforhistologicalstudies.Hema-toxylin-eosinandMasson’sTrichromestainingoftheheartwereanalyzed.Theultrastructureofleftventriclewasalsoexaminedwithelectronmicroscopy.EchocardiographyrevealedthatearlydiabeticcardiomyopathyhadimpairedLVdiastolicfunctionexpressedbydiminishedE-waves,increasedAwaves,E/AratioreversionandincreasedE-wavedecelerationtime(EDT).Concurrently,LVend-diastolicpressure(LVEDP)anddiastolictimeconstant(T)wereincreased,minimumdP/dt(LV-dp/dt)wasreduced,obtainedthroughcardiaccatheterization.TherewerenosignificantdifferencesinLVejectionfraction(EF),LVpeaksystolicpressure(LVSP),ormaximumdP/dt(LV+dp/dt).Qualitativelightmicroscopyrevealednohistologicchangesinmyocardiumfromdiabeticrabbits.Themostevidentultrastructuralchangewasspottedmyofibrillardamage,whileinterstitialfibrosiswasslight.ConclusionsTheseresultssuggestthatearlydiabeticcardiomyopathyinanimalmodelischaracterizedbyleftventriculardiastolicdysfunction,bothimpairedactiverelaxationandincreasedpassivechamberstiffness.Whereas,leftventricularsystolicfunctioncanremainnormal.Itmightpartlycontributetomyofibrillardamage,butnotmyocardialfibrosis.

简介：Sarcoidosisisarareconditionofgranulomatousinfiltrationofmanytissuesofthebody,includingtheheart.Cardiacsarcoidosishasbeenchallengingtostudy,asitisoftenasymptomatic,althoughtheinitialpresentationcanbesuddencardiacdeath.Theincidenceandprevalencerateshavebeendifficulttoestablishandnoexpertagreeduponguidelinesfordiagnosisandmanagementofcardiacsarcoidosisexist,andclinicalmanifestationsarevaried.Thepathophysiologyofgranulomaformationinthemyocardiumaswellasothertissuesisgovernedbyimmuneresponsetosomeenvironmentalantigen.Geneticsisalsothoughttoplayarole,althoughgenealterationshavenotbeenextensivelystudied,andnospecificsetofgeneticmutationshasbeenidentifiedtoaidinidentificationofindividualsatriskofdevelopingdisease.Epigeneticfactorslikelyplayasignificantroleinmodulationofgeneexpressionwithrespecttoimmuneresponse.Thereisnostandardizedscreeningtoolfortheidentificationofcardiacsarcoidosis.Thepresenceofsystemicsarcoidosisandnew-onsetthird-degreeheartblockorventriculararrhythmiaswarrantsfurtherinvestigationforcardiacsarcoidosis.MRIandPETareusefulinhelpingtoidentifycardiacsarcoidosisbutarenotstand-alonetests.Endomyocardialbiopsyisthegoldstandardbuthasalowyieldowingtothepatchynatureofgranulomaformationinthemyocardium.Therapyshouldbeinstitutedearlyandinvolvesimmunosuppressivetherapywithpredominantuseofcorticosteroids.Arrhythmias,eitherventricularorhigh-gradeheartblocks,aremanagedwithdevicetherapy.Clinicalpresentationmaywarrantuseofantiarrhythmicagentsand/orcatheterablation.Survivalanddiseaseprognosisaredependentonearlydiagnosisandtreatment.Thisreviewdetailsthecurrentunderstandingofcardiacsarcoidosisandhighlightsdiagnosticstrategiesandtreatmentwiththeaimofguidingthecliniciantoearlyidentificationofpatientsandimplementationofappropriatemanagementinthisraredi

简介：TodeterminewhetherthepossessionofcertainHLA-DQA1alleleswasassociatedwiththeriskofdevelopingidiopathicdilatedcardiomyopathy(IDC)andtosubstantiatetheroleofanautoimmunologicpathogenesisinIDC.TypetheallelesofHLA-DQA1bypolymerasechainreactionwithsequence-specificprimers(PCR-SSP)techniquein38patientsofidiopathicdilatedcardiomyopathy(7womenand31men),agedfrom17to56yearsoldwithdiagnosisbeingaccordingtoWorldHealthOrganizationcriteria(IDCgroup),in50patientsofend-stageheartfailureofknownetiology(18womenand32men),withagesrangingfrom34to72(HFgroup),andinthecontrolgroupconsistingofpresumably100healthysubjects(39womenand61men)fromthehealthsurvey,agedfrom30to59yearsold.ThefrequencyofHLA-DQA1*0501intheDCMpatientswassignificantlyelevatedthanthatintheHFandthecontrolgroup.MolecularanalysisoftheDQA1genepolymorphismperformedinthethreesubgroupsshowsanincreasedfrequencyofDQA1*0501amongpatientswithlessEF.TheHFgroupcarriesahighfrequencyofHLA-DQA1*0301.AnincreasedfrequencyofDQA1*0201andDQA1*0103wasfoundinthecontrolgroup.HLA-DQA1*0501isanassociatedgeneofidiopathicdilatedcardiomyopathyandthepossessionofDQA1*0301maybeindicativeoftheknownetiologicheartfailure,suggestingthatthemechanismsinvolvedinthepathogenesisofIDCandotherwiseheartfailurearedifferent.ImmunologicabnormalitiesmaybeamajorcontributortothesusceptibilityofdevelopingofIDC.

简介：ObjectivesToevaluatethechangesoftheplasmaendothelinlevelinpatientswithdilatedcardiomyopathy,andtoinvestigatetherelationshipbetweentheplasmaendothelinlevelandtheseverityofheartfailure,heartsize,leftventricularfunctionandwithornotwithpulmonaryarterialhypertension;thechangesoftheplasmaendothelinlevelbeforeandaftertreatment.MethodsTheplasmaendothelinlevelof30patientswithDCM,30healthycontrolsubjects,andtheirLVEF,PAP,heartsize,plasmaendothelinlevelbeforeandaftertreatmentweredetermined.ResultsTheplasmaendothelinlevelinDCMgroupwassignificantlyhigherthanthatincontrolgroup(135.93±70.65pg/mL)vs(43.65±12.07pg/mL),P<0.05;therewasacorrelationbetweenETlevelandheartsize(r=0.4580,P=0.0109);therewasasignificantnegativecorrelationbetweenLVEFofDCMandETlevel(r=-0.6922,P=0.0021);anditwasasignificantpositivecorrelationbetweenETlevelandpulmonaryarterialhypertension(r=0.8974,P<0.0005).Aftertreatment,theETleveldecreasedsignificantlythanbeforeinDCMgroup(129.15±43.93pg/mL)vs(63.12±22.20pg/mL),P<0.05.ConclusionsPlasmaETlevelmaybeanewparameterofevaluatingseverityofheartfailure,efficacyoftherapyandprognosis.

简介：AbstractSepsis is defined as life-threatening organ dysfunction caused by a dysregulated host response to infection. The heart is one of the most important oxygen delivery organs, and dysfunction significantly increases the mortality of the body. Hence, the heart has been studied in sepsis for over half a century. However, the definition of sepsis-induced cardiomyopathy is not unified yet, and the conventional conception seems outdated: left ventricular systolic dysfunction (LVSD) along with enlargement of the left ventricle, recovering in 7 to 10 days. With the application of echocardiography in intensive care units, not only LVSD but also left ventricular diastolic dysfunction, right ventricular dysfunction, and even diffuse ventricular dysfunction have been seen. The recognition of sepsis-induced cardiomyopathy is gradually becoming complete, although our understanding of it is not deep, which has made the diagnosis and treatment stagnate. In this review, we summarize the research on sepsis-induced cardiomyopathy. Women and young people with septic cardiomyopathy are more likely to have LVSD, which may have the same mechanism as stress cardiomyopathy. Elderly people with ischemic cardiomyopathy and hypertension tend to have left ventricular diastolic dysfunction. Patients with mechanical ventilation, acute respiratory distress syndrome or other complications of increased right ventricular afterload mostly have right ventricular dysfunction. Diffuse cardiac dysfunction has also been shown in some studies; patients with mixed or co-existing cardiac dysfunction are more common, theoretically. Thus, understanding the pathophysiology of sepsis-induced cardiomyopathy from the perspective of critical care echocardiography is essential.

简介：BackgroundArrhythmogenicrightventricularcardiomyopathy(ARVC)isamajorcauseforsuddencardiacdeathduetoventriculartachycardia.Litterisknownaboutitslong-termoutcomesinChineseARVCpatients.Thepurposeofthisstudywastoevaluatethelong-termclinicaloutcomesinpatientswithARVCandtoclarifytheriskfactorsofcardiacevents.MethodsFortysubjectsfulfillingmodifiedTaskForcecriteriawereincludedinthisstudy.Informationonclinicalpresentation,electrocardiographicandcardiacimagingfindings,andlong-termoutcomeofcaseswereinvestigated.ResultsAveragefollow-upperiodfromonsetwas57.5±42.6months.Themeanageatonsetofsymptoms(32.2±12.7years)andmalepredominance(85.0%)weresimilartothatreportedinotherstudies.Palpitationswerethemostfrequentsymptom(82.5%).T-waveinversionwasthemostcommonpresentingabnormalityonresting12-leadECG(75%).Ventriculartachycardiawithleftbundlebranchblockmorphologywassubsequentlydocumentedinatotalof28(70%)subjectsduringastudyperiod.Thecumulativemortalityratewas7.5%.ConclusionClinicalpresentationinChineseARVCpatientswassimilartothatreportedinotherstudies.ARVCisassociatedwithearlymortalitythatisdifferenttoothercountrypopulation.

简介：Ifsystolicanteriormotion(SAM)ofaredundantanteriorleafletofthemitralvalveiscombinedwithsystolicthickeningofanasymmetricseptum,(ASH),anarrowoutflowchanneloftheleftventricleisformed.Thisnarrowedoutflowchannelisthenresponsibleforthemidsystolicpressuredifferencebetweenthebodyoftheleftventricleandtheoutflowareaoftheleftventricle.Thisresultsinhypertrophic“obstructive”cardiomyopathy(HOCM).

简介：无

简介：无