w前组病例讨论

病史男性，54岁，活动后气促3年，加重1月，2016-01-08入院3年前出现症状，当时(2012-11-30)我院心超示双房增大，室间隔增厚(12mm)，左室多壁段收缩活动异常，轻度肺动脉高压，LVEF36%。冠脉CTA(-)。诊断心功能不全，给予地高辛、呋塞米、螺内酯、华法林、卡维地洛、贝那普利等治疗后症状好转。3年间症状有反复。2015-11-16我院门诊随访，心超示双房增大，LVDd54mm，轻度二尖瓣反流，左室多壁段收缩活动异常，LVEF42%。长期服用螺内酯导致乳房发育。否认高血压病史，家系中无类似病例。查体HR65次/分，BP92/74mmHg巩膜轻度黄染颈静脉充盈，肝颈静脉反流征(-)肺：双肺呼吸音清，右下肺呼吸音较低，可闻及湿性啰音心：心界不大，HR65次/分，律不齐，杂音未闻及腹部：肝右肋下1-2指，软，无触痛或叩击痛，移动性浊音(-)双下肢不肿实验室检查(2016-01-09)肝功能：TBIL41.5μmol/L，CBIL20.8μmol/L，Alb41g/L，ALT13U/L，AST21U/L，LDH270U/L，γ-GT130U/L肾功能：BUN5.7mmol/L，Cr87μmol/L，eGFR88ml/min血脂：HDL-C0.84mmol/L，LDL-C1.58mmol/L，TG1.11mmol/L电解质：Na136mmol/L，K3.3mmol/L心脏标志物：cTnT0.082ng/ml，CK97U/L，CK-MB11U/L，NT-proBNP10443pg/ml血常规：Hb123g/L，Plt183×109/L，WBC6.95×109/L，N65.2%甲状腺功能：FT34.2pmol/L，FT423.8pmol/L，hs-TSH2.22uIU/ml凝血功能：PT31.0s，APTT45.5s，INR2.59糖代谢：FBG5.9mmol/L，P2hBG10.0mmol/L，HbA1c7.9%血肿瘤标志物：AFP、CEA、CA199、Cyfra211、NSE均(-)，CA125279.2U/ml(＜35U/ml)ECG房颤，心室内传导阻滞，低电压，V1-4呈QS型TTE腹部B超X线胸片心影增大，两侧胸腔积液冠脉CTA诊断心功能不全心功能Ⅲ级心房颤动胸腔积液治疗托拉塞米20mgivbid→20mgivqd→20mgpoqd螺内酯20mgqd卡维地洛2.5mgbid华法林1.25mgqd培哚普利2mgqd硝酸异山梨酯5mgtid氯化钾缓释片1gtid曲美他嗪20mgtid症状明显改善24h尿量1500~2500ml血液指标复查(2016-01-18)肝功能：TBIL29.8μmol/L，CBIL14.1μmol/L肾功能：BUN5.3mmol/L，Cr86μmol/L，eGFR90ml/min电解质：Na137mmol/L，K4.0mmol/L心脏标志物：NT-proBNP4926pg/ml(↓52.8%)凝血功能：PT22.9s，APTT39.8s，INR1.87胸腔穿刺术(2016-01-18)胸水常规：黄色、透明，无凝块，比重1.015，Rivalta试验(+)，RBC2088/mm3，WBC551/mm3，N8%，L92%胸水生化：LDH75U/L，ALB15.33g/L，胸水葡萄糖10.6mmol/L细菌涂片、培养(-)TB(-)真菌涂片(-)Light指标：漏出液可能性较大讨论心功能不全病因：原发性心肌损害：缺血性心脏病、心肌炎和心肌病、心肌代谢障碍性疾病心脏负荷过重：(1)压力负荷过重：高血压病、主动脉瓣狭窄、肺动脉高压、肺动脉瓣狭窄等；(2)容量负荷过重：心脏瓣膜关闭不全、分流性先天性心脏病等限制性心肌病CAUSESOFRESTRICTIVECARDIOMYOPATHIESInfiltrative(BetweenMyocytes)

Amyloidosis

Primary(lightchainamyloid)

Familial(abnormaltransthyretin)Senile(normaltransthyretinoratrialpeptides)SarcoidosisFattyinfiltration

Storage(WithinMyocytes)

Hemochromatosis(iron)

Fabry’sdisease

Glycogenstoragedisease(II,III)

Gaucher’sdiseaseHurler’sdiseaseFibrotic

Radiation

SclerodermaEndomyocardial

Possiblyrelatedfibroticdiseases

Tropicalendomyocardialfibrosis

Hypereosinophilicsyndrome(Löffler’sendocarditis)

Carcinoidsyndrome

Radiation

Drugs:e.g.,serotonin,ergotamine

OverlapwithOtherCMP

Hypertrophiccardiomyopathy

“pseudohypertrophic”

Minimallydilatedcardiomyopathy

Early-stageDCM

PartialrecoveryDCMSarcoidosis

Idiopathic(includefamilial)心脏MRSelvanayagamJBetal.JAmCollCardiol.2007;50:2101-2110.FeaturesofCardiacAmyloidosisBasedonAmyloidTypeTypeofAmyloidosisPrecursorProteinUsualAgeatOnsetMainOrgansInvolvedAverageUntreatedSurvivalAL(primary)Abnormallightchains50+AllexcepttheCNSHeartinvolvedin50%Noncardiac,24mHeartfailure,<9mFamilial(ATTR)MutantTTR20-70+PeripheralandautonomicneuropathyHeart7-10yearsforneuropathySenilesystemicamyloidosisWild-typeTTR70+Heart5-7yearsIsolatedatrialamyloidosisAtrialnatriureticpeptideUnknownCardiacatriaNoeffectonsurvivalAA(secondryamylodosis)SerumamyloidATeensupwordLiver,