逸大医 1肺动脉高压 3.肺病/低氧所致肺高压 特发性肺动脉高压 COPD 遗传性肺动脉高压 间质性肺病 ·药物和毒物 其他肺部疾病(混合性通气功能障碍) 相关因素所致肺动脉高压 睡眠呼吸疾病 结缔组织疾病 肺泡低通气 HV感染 慢性高原病 门静脉高压 ·肺泡一毛细血管发育不良 先天性心脏病 一血吸虫病 4慢性血栓栓塞所致肺高压 慢性溶血性贫血 新生儿持续性肺动脉高压 5不明和或多种机制所致肺高压 1肺静脉闭塞病和/或肺毛细血管瘤 ·血液系统疾病:骨髓增生性疾病,脾切除术 系统性疾病:结节病,血管炎 2.左心疾病所致肺高压 代谢性疾病:糖原沉积症,甲状腺疾病 其他罕见疾病 收缩功能异常 舒张功能异常 心瓣膜疾病 肺循环高压临床分类
6 Company Logo 1.肺动脉高压 2.左心疾病所致肺高压 3.肺病/低氧所致肺高压 4.慢性血栓栓塞所致肺高压 5不明和/或多种机制所致肺高压 •特发性肺动脉高压 •遗传性肺动脉高压 •药物和毒物 •相关因素所致肺动脉高压 --结缔组织疾病 --HIV感染 --门静脉高压 --先天性心脏病 --血吸虫病 --慢性溶血性贫血 •新生儿持续性肺动脉高压 1’.肺静脉闭塞病和/或肺毛细血管瘤 •收缩功能异常 •舒张功能异常 •心瓣膜疾病 •COPD •间质性肺病 •其他肺部疾病(混合性通气功能障碍) •睡眠呼吸疾病 •肺泡低通气 •慢性高原病 •肺泡—毛细血管发育不良 •血液系统疾病: 骨髓增生性疾病,脾切除术 •系统性疾病:结节病,血管炎 •代谢性疾病:糖原沉积症,甲状腺疾病 •其他罕见疾病… •肺循环高压临床分类
逸大医 肺动脉高压概念不断发弯化 Venice Clinical Classification Table 2 Updated Clinical Classification of Pulmonary Hypertension (Dana Point, 2008) of Pulmonary Hypertension(2003) 1 Pulmonary arterial hypertension(PAH) 1 IdiopathIc PAH 12 Heritable 11 Idiopathic(IPAH) 121日MP2 12 Familial (FPAH) 122ALK endogin (wath or without be 13. Associated with(APAH) telangiectaninl 13.1 Collagen vascular disease 1.23 Unknown 13.2, Congenital systemic to-puImonary shunts 13. Drug and soxin-nauced 14 Assayed wn? 13.3. Portal hypertension 141. 3.4. HiV infection 42 Hiv infection 13.5. Drugs and toxins 143. Portal hypertension 13.6. Other (thyroid disorders, glycogen storage disease, Gaucher disease, 144. Consents hear dese hereditary hemorrhagic telangiectasia, hemoglobinopathies 1.45. Schistosomiasis 1.46 Chroni myeloproliferative disorders, splenectomy) 15P crenson ot the newt 14. Associated with significant venous or capillary involvement 1.Pulmonary veno-occlusive disese (PwOD) and/or ry capillary 14.1 Puimonary veno-occlusive disease(PvOD (PCH) 14.2 Pulmonary capillary hemangiomatosis(PCH) 2. Puimonary hypertension owing to en hear disease 15. Persistent pulmonary hypertension of the newborn 2⊥ Systole dysfuncton 2. Pulmonary hypertension with left heart disease 2.2 Diastole dysfunction 23. VeNular die 2.1 Left-sided atrial or ventricular heart disease 3 on owing to ang dlsease and/or my 2.2 Left-sided valvular heart disease 3.⊥ chronk cbcruatlve p. 3. Pulmonary hypertension associated with lung diseases and/or hypoxemia 3.2 Inter 3.1 Chronic obstructive pulmonary disease 3.3 other pulmonany dseases with mixed restnctrve obstructive pettern 3.2 Interstitial lung disease 3. 4 sleep-dbord ered breaths 3.5 ANe ar hypoventilation disorden 3.3. Sleep-disordered breathing 16 Chronk exposure to hig atitude 3. 4. Alveolar hypoventilation disorders 37.1 3.5. Chronic exposure to high altitude on ICTEPH) factorial m 5.1 Hematologio disorders: myelopwolife 4. Pulmonary hypertension owing to chronic thrombotic and/or embolic disease 5.2 systemic disorden: sarcoidosis, pulmonary Langerhans cel 4.1 Thromboembolic obstruction of proximal pulmonary arteries hiabocytos lymphangaielomyamatos. neurofibromatosis, vasculitis 42. Thro olic obstruction of distal pulmonary arteries 5.3. Metaboeo disorders sfyoocen storage diease, Gaucher disease, throid 4.3 Nonthrombotic pulmonary embolism (tumor, parasites. foreign materia 54 5. Miscellaneous on wsis Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary
肺动脉高压概念不断发展变化
逸大医 慢性肺源性心脏病 呼吸系统的常见疾病,发病率高,远超过 00 目前缺乏理想治疗措施,病死率1015%左 石。积洽狞同以延长寿命,提高生活质
8 慢性肺源性心脏病 • 呼吸系统的常见疾病，发病率高，远超过 4.4%o • 目前缺乏理想治疗措施， 病死率10–15%左 右。积极治疗可以延长寿命，提高生活质 量
逸大医 病因 1、支气管、肺疾病 慢支并发肺气肿(COPD)占80-90% -其次为支气管哮喘、支气管扩张、重 症肺结核、尘肺、慢性弥漫型肺间质 纤维化、结节病、过敏性肺泡炎、嗜 酸性肉芽肿
9 病因 1、支气管、肺疾病 –慢支并发肺气肿（COPD）占80-90%; –其次为支气管哮喘、支气管扩张、重 症肺结核、尘肺、慢性弥漫型肺间质 纤维化、结节病、过敏性肺泡炎、嗜 酸性肉芽肿
逸大医 病因 2、神经肌肉及胸壁疾病 3、肺血管疾病 反复的肺动脉栓塞,结缔组织疾病,原 发性肺动脉高压 °4、通气驱动失常的疾病 原发性肺泡通气不足、先夭性口咽畸形、 睡眠呼吸暂停综合症
10 病因 • 2、神经肌肉及胸壁疾病 • 3、肺血管疾病 反复的肺动脉栓塞，结缔组织疾病，原 发性肺动脉高压 • 4、通气驱动失常的疾病 原发性肺泡通气不足、先天性口咽畸形、 睡眠呼吸暂停综合症