1、儿童与成人肺动脉高压特点比较吴炳祥哈尔滨医科大学附属第一医院PAH的定义PAH流行病学及自然病程PAH的病理改变PAH的临床表现PAH的诊断流程PAH的治疗及预后主要内容肺动脉高压的定义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.Pulm
2、onary Arterial Hypertension:a comparison between between 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 disea
3、se in the first year of life and others remaining free of 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
4、and idiopathic pulmonary arterial hypertension.Curr Opin Pulm Med 16(Suppl1):S35-S41.流行病学及自然病程病理学成人 :内膜增生 ,纤维化 ,丛样病变 ,扩张样病变 ;外膜增厚 ,中膜肥厚 ,炎细胞浸润 ,局部血栓形成等 .儿童 :中膜肥厚 ,内膜纤维化 ,非肌型小动脉肌化 ,丛样病变 ,扩张样病变等 . The presenting symptoms are usually more severe in children , with syncopal episodes occuring more freque
5、ntly than in adults,and a rate of disease progression that is typically faster.临床表现劳力性呼吸困难 ,乏力 ,晕厥 ,胸痛 ,右心功能不全伴外周水肿 . 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儿童患者对症状表述不准确 ,多需要家长的观察 儿童患者运
6、动量大 ,且患上呼吸道感染的机会高 ,故儿童在疾病的早期即可出现相应的症状 ,有利于早期诊断 .儿童 IPAH/HPAH,APAH-CHD患者在诊断时 ,其心功能分级和血流动力学指标好于成人 .儿童患者的特殊性严重程度评估1. 临床特点WHO心功能分级的评估对成人及大龄儿童适用 ,但不适宜应用于新生儿或低龄儿童 .uTime to clinical worsening is a reliable and appropriate means of assessing disease progression. (though not adopted in children)uUsing clini
7、cal 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 etal,Assessment of endpoint in pediatric population:congenital heart disease and idiopathic pulmonary arterial hypertension.Curr Opin Pulm Med 16(Suppl1):S35-S41.
