儿童与成人肺动脉高压特点比较PPT学习教案

1、会计学1儿童与成人肺动脉高压特点比较儿童与成人肺动脉高压特点比较主要内容第1页/共38页Rich S,etal.Primary pulmonary hypertension.A national prospective study.Ann Intern Med 1987;107:216-223.成人:在海平面状态下,静息时,右心导管检查肺 动脉平均压(mPAP) 25mmHg,肺毛细血管楔压(PCWP)15mmHg,且肺血管阻力(PVR) 3WU.Barst RJ,etal.Pulmonary Arterial Hypertension:a comparison between between

2、 children and adults.Eur Respir.2011,37:665-677.婴儿:平均肺动脉压/平均体循环动脉压,或肺动脉收缩压/体循环动脉收缩压0.4第2页/共38页儿童患者:APAH-CHD,IPAH/HPAH高发成人患者:APAH-CTD则相对较常见.CHD-PAHThere is a heterogeneity in the nature of CHD-PAH,with some patients developing irreversible disease in the first year of life and others remaining free o

3、f symptoms untill their second or third decade, or even later.IPAH/HPAHThe natural history of untreated IPAH/HPAH is most often rapidly progressive and fatal.Sheila G etal,Assessment of endpoint in pediatric population:congenital heart disease and idiopathic pulmonary arterial hypertension.Curr Opin

4、 Pulm Med 16(Suppl1):S35-S41.流行病学及自然病程流行病学及自然病程第3页/共38页病理学第4页/共38页临床表现劳力性呼吸困难,乏力,晕厥晕厥,胸痛,右心功能不全伴外周水肿.第5页/共38页治疗原发病与肺高压严重程度不匹配病史/症状/体征怀疑肺高压ECG,胸片,超声心动图,肺功能,HRCT确诊为第二或第三大类肺通气灌注扫描与肺高压严重程度匹配节段性灌注缺损PAH可能,建议RHC检查CTEPHPVOD/PCHmPAP25mmHg,PCWP15mmHg其它原因是是是否否否是诊断流程是第6页/共38页儿童患者的特殊性第7页/共38页严重程度评估严重程度评估第8页/共38页

5、uTime to clinical worsening is a reliable and appropriate means of assessing disease progression. (though not adopted in children)uUsing clinical worsening as an endpoint in children could be particularly helpful because of the faster rate of disease progression in many children with IPAH.Sheila G e

6、tal,Assessment of endpoint in pediatric population:congenital heart disease and idiopathic pulmonary arterial hypertension.Curr Opin Pulm Med 16(Suppl1):S35-S41.第9页/共38页CPET,in contrast to the 6-minute walk test,may be particularly relevant to identify those children who have an exaggerated response

7、 of the pulmonary vascular bed to exercise and appear to have good exercise capacity with normal activities of daily living,but have a history of syncopal episodes with over-exertion or in response to mild hypoventilation with sleepRosenzweig EB,etal.Pulmonary arterial hypertension in children.Pedia

8、tr Pulmonol 2004;38:2-22.第10页/共38页3.生化标记物NT-proBNP , BNP , 血尿酸,C反应蛋白等指标也可应用于儿童病情严重程度及预后的评估.Lower NT-proBNP and BNP concentrations have been reported in children compared with adults,consistent with right heart failure being less frequent in children as compared with adult patients.Lammer AE,etal.Pro

9、gnostic value of B-type natriuretic peptide in children with pulmonary arterial hypertension.Int J Cardiol 2009;135:21-26第11页/共38页由于非侵入性评估指标在儿童患者中难以执行,所得结果可信度差,因此,血流动力学指标仍然作为儿童患者评估疾病严重程度及疗效的金标准.Barst RJ,etal.Pulmonary Arterial Hypertension:a comparison between between children and adults.Eur Respir.

10、2011,37:665-677.第12页/共38页治治 疗疗p一般治疗p支持治疗p靶向药物治疗p其它第13页/共38页第14页/共38页儿童患者抗凝风险评估有争议,可在特殊情况时考虑抗凝,如右心功能不全,高凝状态,中心静脉置管等.第15页/共38页第16页/共38页It is important to remember that vasoreactive patients require constant re-evaluation, as reactivity can be lost at any time.Sheila G etal,Assessment of endpoint in pe

11、diatric population:congenital heart disease and idiopathic pulmonary arterial hypertension.Curr Opin Pulm Med 16(Suppl1):S35-S41.阳性标准:mPAP下降幅度超过10mmHg并且绝对 值40mmHg,CO增加或至少不变.(儿童是否适用有争议)第17页/共38页In children with IPAH,the probality of having a positive response to the pulmonary vasoractivity test appea

12、rs to be age dependent,with the youngest having the greatest likelihood of pulmonary vasodilation;the percentage of responders in children is 30-40% compared with 10-15% in adults.Barst RJ,etal.Vasodilator therapy for primary pulmonary hypertension in children.Circulation 1999;99:1197-1208.血管舒张试验阳性率

13、第18页/共38页Yung D,etal.Outcomes in Children With Idiopathic Pulmonary Arterial Hypertension.Circulation 2004:110:660-665.儿童儿童Long-term CCB responders represented 54% of acute responders and 6.8% (95% CI, 4.7% to 8.9%) of the patient sample.Sitbon O,etal.Response to Calcium Channel Blockers in Idiopath

14、ic Pulmonary Arterial Hypertension . Circulation. 2005;111:3105-3111.成人成人第19页/共38页伊洛前列素,曲前列环素,贝前列环素在儿童患者应用相关报道较少.第20页/共38页u依前列醇(Epoprostenol)在儿童及成人患者中均有较好疗效,但在儿童患者中其最佳剂量常较高.由于其用药途径特殊,故在儿童患者中应用不便.Epoprostenol has also been used in children of all ages with similar safety and efficacy as shown in adul

15、t ptients. However,optimal dosing in children is frequently higher in a nanograms per kilogram per minute basis.Barst R. How has epoprostenol changed the outcome for patients with pulmonary arterial hypertension?Int J Clin.PractSuppl.2010,64(168):23-32.第21页/共38页成人成人儿童儿童一年生存率80-91%94%三年生存率49-65%88%五年

16、生存率47-55%81%Yung D,etal.Outcomes in children with idiopathic pulmonary arterial hypertension.Circulation 2004;110:660-665.依前列醇依前列醇第22页/共38页塞他生坦,安贝生坦在儿童患者应用相关报道较少.第23页/共38页在儿童及成人中均有较好疗效,可改善WHO心功能分级和血流动力学等指标.Gatzoulis MA,etal. Pulmonary arterial hypertension in paediatric and adult patients with conge

17、nital heart disease.Eur Respir Rev.2009,18(113):154-61.第24页/共38页Ivy DD, etal.Long-Term Outcomes in Children With Pulmonary Arterial Hypertension Treated With Bosentan in Real-World Clinical Settings.Am J Cardiol. 2010 Nov 1;106(9):1332-8. 波生坦(儿童)第25页/共38页 波生坦(成人) Long-term outcome with first-line bo

18、sentan therapy in idiopathic pulmonary arterial hypertension Eur Heart J. 2006 Mar;27(5):589-95. 第26页/共38页 伐地那非,他达那非在儿童患者应用相关报道较少.西地那非在儿童及成人中均有较好疗效,可改善血流动力学,提高运动耐量.第27页/共38页Xu XQ,etal.The efficacy and safety of sildenafil in Chinese patients with pulmonary arterial hypertension. Hypertension Reseach

19、(2009)32,911-915.西地那非(成人)第28页/共38页Oliveira EC,etal.Sildenafil in the management of idiopathic pulmonary arterial hypertension in children and adolescents.J Pediatr,2005;81(5):390-4.西地那非(儿童)第29页/共38页靶向药物治疗后的生存率Haworth SG,etal.Treatment and survival in children with pulmonary arterial hypertension:the

20、 UK Pulmonary Hypertension Service for children 2001-2006.Heart 2009;95:312-317.第30页/共38页药物药物剂量剂量给药途径给药途径适应症适应症副作用副作用氧气保持SaO2为94%吸入低氧血症儿童用量过多安全性差NO2-20ppm吸入NICU,PICU低血压CCB(硝苯地平)0.2mg/kg ,每8小时口服IPAH低血压5-磷酸二酯酶抑制剂(西地那非)0.25mg/kg,每6小时口服新生儿慢性肺疾病,艾森曼格综合症低血压,视觉障碍内皮素受体拮抗剂(波生坦)15-125mg(按体重),每12小时口服IPAH,艾森曼格综

21、合症低血压,肝功损害前列环素(依前列醇)2-60ng/kg/min静脉注射艾森曼格综合症低血压,出血前列环素(伊洛前列环素)5ug,每2-3小时,逐渐增至最大耐受量吸入慢性肺部疾病,艾森曼格综合症低血压儿童患者肺动脉高压治疗药物儿童患者肺动脉高压治疗药物Robert T.Etiology,Diagnosis,and Pharmacologic Treatment of Pediatric Pulmonary Hypertension.Pediatr Drugs,2009,11(2):115-128第31页/共38页肺动脉高压支持疗法:抗凝,吸氧,改善生活习惯血管舒张试验阳性阴性CCB稳定性WH

22、O/不稳定性WHO/波生坦,前列环素类依前列醇持续监测症状性WHO,进行性呼吸困难,难治性心衰,晕厥(CCB阳性患者:治疗数月后对CCB反应性下降)联合药物治疗移植房间隔造瘘术治疗流程National Pulmonary Hypertension Centres of the UK and Ireland.Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland.Heart 2008;94(Supp1):i1-i41.第32页/共38页预

23、后美国国立卫生研究院(NIH)对194例特发性PAH 患者的统计，平均生存期仅为年, 1年、3年和5年的生存率分别为68、48和34.第33页/共38页IPAHAPAH一年生存率85.6%92.3%三年生存率79.9%83.8%五年生存率71.9%56.9%Haworth SG,etal.Treatment and survival in children with pulmonary arterial hypertension:the UK Pulmonary Hypertension Service for children 2001-2006.Heart 2009;95:312-317.儿童靶向药物治疗后IPAH,APAH一年,三年,五年总体生存率分别为90.5%,82.8%,和64.2%. IPAH和APAH生存率无明显差别.成人IPAH患者靶向药物治疗后一年，三年生存率分别为92.1% 和75.1%,(Zhang R,etal.Survival of Chinese Patients with Pu