围产期心肌病课件.pptx

1、围产期心肌病 Peripartum Cardiomyopthy,PPCMSliwa K,Hilfiker-Kleiner D,Petrie M C,et al.Heart Failure Association of the European Society of Cardiology Working Group on Peripartum Cardiomyopathy.Current state of knowledge on aetiology,diagnosis,management,and therapy of peripartum cardiomyopathy:a position

2、statement from the Heart FailurJ.European Journal of Heart Failure,2010,12(8):767778.1围产期心肌病定义Peripartum Cardiomyopthy,PPCM 围产期心肌病：以急性心力衰竭起病或出现扩张型心肌病样改变，发病时间局限在妊娠最后3个月或产后5个月内，既往无心血管系统疾病史，这种特殊的心脏疾病称之为PPCM 中华妇产科学23Definition and Pathophysiology Peripartum cardiomyopathy is an idiopathic cardiomyopathy

3、 presenting with HF secondary to left ventricular(LV)systolic dysfunction towards the end of pregnancy or in the months following delivery,where no other cause of HF is found.It is a diagnosis of exclusion.The LV may not be dilated but the ejection fraction(EF)is nearly always reduced below 45%.Cont

4、ributing factors:General risk factors for cardiovascular disease(such as hypertension,diabetes,and smoking)Pregnancy-related factors(such as age,number of pregnancies,number of children born,use of medication facilitating birth,and malnutrition).qProlactin,16 kDa prolactin,and cathepsin DqOther puta

5、tive pathophysiological mechanismsqInflammationqSerum markers of inflammation including the soluble death receptor sFas/Apo-1,C-reactive protein,interferon gamma(IFN-g),and IL-6 are elevated in patients with PPCM.qViruses qAlthough some reports have implicated cardiotropic enteroviruses in PPCM,othe

6、rs have not found a higher frequency of viral infections in patients with PPCM than in those with IDCM.qAutoimmune systemqSerum derived from PPCM patients affects in vitro maturation of dendritic cells.qGenetic susceptibility to peripartum cardiomyopathy4Clinical presentation and diagnosis qEarly si

7、gns and symptomsqPedal oedema,dyspnoea on exertion,orthopnoea,paroxysmal nocturnal dyspnoea,and persistent cough.qAdditional symptoms qAbdominal discomfort secondary to hepatic congestion,dizziness,praecordial pain,and palpitations,and,in the later stages,postural hypotension can occur.qIn addition,

8、patients may be anaemic.q发病时间：333例患者纳入分析，显示产后3个月内发生者比例最高，占67.3%(224例)，其次是妊娠最后1个月内，占 20.4%(68例)。q合并症：患者合并贫血比例较高，为53.5%(257/480)；其次是高血压，48.5%(233/480)，多表现为血压暂时性升高，终止妊娠或及早积极控制心衰加重，血压均可恢复正常。q并发症：并发妊娠高血压者比例为32.5%(98/302)；血栓性栓塞症者比例为8.6%(27/314)。5岳晓辉,刘楠,薛晓艳.我国围产期心肌病流行病学特点及转归荟萃分析J.中国妇产科临床杂志,2011,12(5):359-3

9、63.v诊断：发生于妊娠最后1个月或产后5个月内的症状性心力衰竭；无其他明确的心力衰竭原因；超声心动图证实为收缩性心力衰竭。67Investigation of peripartum cardiomyopathy Electrocardiogram(ECG)Voltage criteria consistent with LV hypertrophy and ST-T wave abnormalities&susceptible to arrhythmias B-type natriuretic peptide(BNP)or N-terminal pro-BNP(NT-proBNP)Echoc

10、ardiography:LV end-diastolic diameter60 mm(as does a LVEF,30%)predicts poor recovery of LV function&ruling out LV thrombus,particularly where the LVEF is severely depressed.Echocardiography should be repeated before patient discharge and at 6 weeks,6 months,and annually to evaluate the efficacy of m

11、edical treatment.If available,cardiac MRI can also be repeated at 6 months and 1 year.89Management of acute heart failure in peripartum cardiomyopathy Initial management Oxygen:SaO295%,non-invasive ventilation(PEEP 5-7.5 cm H2O)Intravenous diuretics:if congestion and volume overload Intravenous nitr

12、ate:SBP110 mmHg caution in SBP 90-110 mmHg Inotropic agents:low output state,hypoperfusion(cold,clammy skin,vasoconstriction,acidosis,renal impairment,liver dysfunction,and impaired mentation)&persist congestion despite administration of vasodilators and/or diuretics Mechanical ventricular support a

13、nd cardiac transplantation10Management of stable heart failure in peripartum cardiomyopathy Drug therapyAngiotensin-converting enzyme(ACE)-inhibitors and angiotensin-II receptor blocker(ARB)are contraindicated because of serious renal and other foetal toxicity(I-C).Hydralazine and long-acting nitrat

14、es -Blockers Diuretics Aldosterone antagonists Antithrombotic therapy Cardiac resynchronization therapy and implantable cardioverters/defibrillators 11心衰的治疗原则：心衰的治疗原则：v充血性心衰的治疗主要为以下三方面措施(1)改善泵血功能：其目的在于使衰竭心脏的收缩力恢复正常。用于这方面的药物有洋地黄类强心甙和其他正性肌力药物。(2)减轻心脏工作负荷：其目的在于使心脏作功时消耗的能量减少。血管扩张剂、钙通道阻滞剂等为常用于这方面的药物。(3)消

15、除过度的水钠滞留：是为了控制细胞外容量的增加，主要措施为利尿剂的应用。12v一般治疗：一般治疗：安静、半卧位、氧气吸入、限制钠盐与水、镇静剂应用等。v提高心肌收缩力主要应用三类药物：提高心肌收缩力主要应用三类药物：v洋地黄制剂(预防性应用洋地黄类药物：心脏代偿功能在III级以上；有房颤；心脏有明显扩大；心率经常在100次/分以上；轻度心力衰竭)v其他正性肌力药物(如米力农)v某些类交感胺类(如多巴胺及多巴酚丁胺)13洋地黄制剂洋地黄制剂v一般地高辛0.25mg Bid，2-3天后根据效果改为每日一次，亦可每日用地高辛0.25mg，一般在用药天后可达有效负荷，一般只需心率控制在85-95次/分，

16、一般症状改善即可，不要达到饱和量，以备在预防性用药过程中发生心力衰竭时，可有加大剂量的余地。v待产时只用快速洋地黄，一般不用口服洋地黄。国内作者也主张，临产期心脏病孕妇如心率超过100次/分，或双肺底出现中、小湿罗音感轻度气促者，立即静脉注射西地兰，或在分娩前1-2小时静脉注射西地兰0.40mg，效果良好。14其他正性肌力药物其他正性肌力药物 v双吡啶类正性肌力药物胺联吡啶酮(氨力农)(Amrinome,AMR)是一种细胞内磷酸二酯酶抑制剂，能减少cAMP分解，使心肌细胞内钙含量增高。严重心衰者应用AMR后可生许多有益的血流动力学效应，包括心输出量增高，左室充盈压降低，运动能力改善。本药主要用

17、于重度心衰及难治性心衰，静脉注射初始剂量为每日0.25mg/Kg，以后逐日增加至0.75-1.5mg/kg，口服剂量为每日2-4mg/kg，分2-4次服用。15类交感胺类药物类交感胺类药物v多巴胺：具有受体兴奋作用(兴奋心肌)及受体兴奋作用(外周血管收缩)对肾血管具有独立的血管扩张作用。对失代偿性或急性心衰，小剂量多巴胺(5g/kg/min)可增加心输出量及肾血流量，产生有益的血流动力学效应，并有助于利尿，由于多巴胺具有外周血管收缩效应，心衰合并轻-中度低血压中优先选用多巴胺。其始剂量为0.5-1g/kg/min，逐渐增至3-5g/kg/min。16v多巴酚丁胺：具有兴奋1受体，兴奋心脏2受体

18、(扩张外周血管)及较弱的受体肾上腺能受体的外周血管收缩作用，能增强心肌收缩力、提高心输出量、对心率、血压及肾血流量影响很少。其始剂量为2.5g/kg/min。17减轻心脏工作负荷减轻心脏工作负荷 v血管扩张剂最早用于高血压危象的抢救，后用于治疗心衰，晚近扩展到对慢性心衰的治疗，作用机理主要是扩张动静脉血管，减轻心脏前后负荷，或二者兼之，以改善心功能。v血管扩张剂作用机理：v减轻前、后负荷；对心肌耗氧量的影响，特别对缺血的心肌；增强心肌收缩力；提高心电稳定性；有利尿效应，肾功能改善，尿量增多。18血管扩张剂作用部位和机理可分为：v主要扩张容量静脉的药物：硝酸酯类。降低肺压及左室舒张末期压力，降低

19、心脏前负荷，常用的静脉扩张剂为硝酸甘油及消心痛。对重症心衰最初治疗及急性肺水肿紧急处理是可应用大剂量的硝酸甘油，以5-10g/kg/min开始，每5-10分钟递增10g，其总量可以用至100-200g/kg/min，直至达到理想的血流动力学效应。v主要扩张阻力动脉的药物:肼苯达嗪及硝苯定。减轻心脏后负荷，提高心输出量v均衡扩张静脉及动脉的药物:硝普钠、呱唑嗪、巯甲丙脯酸等19利尿剂的应用利尿剂的应用v可降低心脏前负荷，改善心功能：降低细胞充填压，减轻呼吸困难，减少及消除心衰时周围血管的异常反应。速尿常用来治疗严重的充血性心衰及肺水肿。速尿除有利尿作用外，还有扩张静脉作用，能降低周围血管阻力。氨

20、茶碱可维持心排出量、降低静脉压，抑制肾小管对钠的吸收。v用法：酚妥拉明10-20mg加5%葡萄糖250ml静滴，速尿40mg稀释静注或加莫非氏管内滴注。若有中-低度高血压可加入多巴胺20mg，对围产期心肌病或肾功能衰竭，水钠潴留的心衰适用。20治疗急性肺水肿的各种措施治疗急性肺水肿的各种措施v病因治疗v纠正缺氧v吸氧：氧气吸入、加压给氧、膜肺、高压氧舱；去泡沫剂：酒精、二甲基硅油v减少肺血量、降低肺循环压力(1)减轻后负荷：血管扩张剂 硝酸甘油(2)减轻前负荷：坐位垂腿；吗啡；上血带结扎四肢或放血；快速利尿剂；血管扩张剂；高渗腹膜透析；高位硬膜外麻醉21v增强心肌收缩力，改善心肌代谢 v(1)

21、洋地黄：西地兰或毒毛甙K，非洋地黄类，前列腺素E(2)氨茶碱(3)胰高糖素(4)能量合剂ATP-cATP(5)纠正酸碱平衡失调v降低毛细血管渗透性(1)肾上腺皮质激素(2)Vitamin C(3)控制肺部感染(4)颈交感神经节封闭v提高血浆胶体渗透压(1)新鲜血浆(2)人体白蛋白22Prognosis qIn South Africa,case series have demonstrated that mortality rates have slowly improved over time but 6-month and 2-year mortality rates remain at

22、10 and 28%,respectively.Single-centre studies in Brazil and Haiti report mortality rates of 14-16%within 6 months.In Turkey,a single tertiary centre reported a mortality rate of 30%over 4-year follow-up.The proportion of patients in whom LV systolic function returns to normal is more consistent in case series in the USA,Haiti,and Turkey at 23-41%.q我国：31.2%患者治愈，59.7%患者好转，未愈/死亡患者占9.1%。23Thanks!24