斜卧呼吸-直立性低氧血症综合征机制与诊疗研究进展  

Mechanism,Diagnosis and Treatment of Platypnea-Orthodeoxia Syndrome

作　　者：刘瑶[1] 张丽娟[1] 雍永宏[2] 孙海建 LIU Yao;ZHANG Lijuan;YONG Yonghong;SUN Haijian(Department of Ultrasound Medicine,The Fourth Affiliated Hospital of Nanjing Medical University,Nanjing 210031,Jiangsu,China;Department of Cardiology,The First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,Jiangsu,China;Wuxi School of Medicine,Jiangnan University,Wuxi 214028,Jiangsu,China)

机构地区：[1]南京医科大学第四附属医院超声医学科,江苏南京210031 [2]南京医科大学第一附属医院心内科,江苏南京210029 [3]江南大学无锡医学院,江苏无锡214028

出　　处：《心血管病学进展》2023年第8期752-756,共5页Advances in Cardiovascular Diseases

基　　金：国家自然科学基金(82170424)。

摘　　要：斜卧呼吸-直立性低氧血症综合征的特征是站立位时引发呼吸困难和低氧血症,仰卧位可缓解。目前该疾病的具体机制尚未明确,但大多数已报道的病例都和心内分流或肺内分流相关,前者主要包括房间隔解剖学缺损,伴随其他解剖异常和右心压力升高,后者主要包括通气/血流不匹配和血管畸形。诊断该疾病通常需充分的临床评估和完善的相关检查,对于大多数心内分流和部分肺内分流患者,手术治疗后效果尚可。现就该疾病的机制及诊疗相关研究进展进行综述。Platypnea-orthodeoxia syndrome is characterized by dyspnea and hypoxemia in the standing position and resolved in the supine position.The mechanism of this disease is not clear,but most reported cases are associated with intracardiac shunts and intrapulmonary shunts.The former mainly includes gaps in the atrial septum,accompanying other anatomical abnormalities,and elevated right heart pressure.The latter mainly included ventilation/perfusion mismatch and vascular abnormalities.Adequate clinical evaluation and comprehensive related examinations are helpful for the diagnosis of this disease.Interventional and surgical treatments are beneficial for most patients with intracardiac shunts and some patients with intrapulmonary shunts.This article reviews the research progress in the pathophysiological mechanism,diagnosis and treatment of this disease.

关 键 词：斜卧呼吸-直立性低氧血症综合征 分流 卵圆孔未闭 肺动静脉畸形 

分 类 号：R597[医药卫生—内科学]