摘要
目的探讨妊娠合并结缔组织病相关肺动脉高压(connective tissue disease related pulmonary arterial hypertension,CTD-PAH)的临床特征及妊娠结局。方法回顾性分析2012年7月至2017年3月间本院诊断为妊娠合并CTD-PAH的8例住院患者的临床资料,分析不同心功能分级的CTD-PAH患者的妊娠分娩方式、分娩时机、麻醉方式及母婴结局。结果 (1)8例CTD-PAH患者,7例为系统性红斑狼疮,1例为混合结缔组织病,妊娠后诊断PAH者共7例,6例患者出现PAH相关症状后急诊入院,其中3例PAH为CTD的首发表现。(2)8例患者中,心功能Ⅰ~Ⅱ级为2例,心功能Ⅲ~Ⅳ级有6例,轻度PAH 2例,中度PAH2例,重度PAH 4例,(3)3例患者死亡,均是心功能Ⅲ~Ⅳ级患者,5例患者治疗后病情好转,新生儿并发症多。结论 CTD-PAH早期无特异性临床症状。CTD患者妊娠期宜规范产检,定期复查超声心动图。妊娠合并CTD-PAH患者多急诊入院,母婴并发症发生率高,预后差,需在多学科医师的指导下,加强母婴的监护,适时结束分娩。推荐硬膜外麻醉或腰硬联合麻醉下行剖宫产术,围术期及产后患者死亡率高,应注意围分娩期及产后的监测及管理。
Objective To investigate the clinical features and outcome of pregnant women with connective tissue disease related pulmonary arterial hypertension(CTD-PAH). MethodsClinical data of 8 pregnant women with CTD-PAH from July 2012 to March 2017 in Fujian Provincial Hospital were retrospectively analyzed. The mode and time of delivery, type of anesthesia and maternal-neonatal outcomes in patients with different cardiac functions were analyzed. Results (1) Among 8 female patients with CTD-PAH,7 were diagnosed with systemic lupus erythematosus, one was diagnosed with mixed connective tissue disease. Seven cases were diagnosed after pregnancy. Six patients were admitted to the emergency department for the PAH related symptoms. PAH occurred in 3 cases after initial CTD manifestations.(2) Among 8 cases, 2 cases were in NYHA class Ⅰ or Ⅱ, while others were in NYHA class Ⅲ or Ⅳ. They were divided into mild PAH group(2 cases), moderate PAH group(2 cases), and severe PAH group(4 cases).(3) Three dead cases were all in NYHA class Ⅲ or Ⅳ. Five patients' conditions improved after in-time treatment. The newborns had various complications. Conclusion As there are no specific clinical symptoms at the early stage of CTD-PAH, standard checked-up regular echocardiography should be conducted on the patients with CTD during pregnancy. For most pregnancy women with CTD-PAH were admitted into hospital as emergency cases with high rates of maternal and infant complications as well as poor prognosis, we should intensively monitor the mother and fetus, timely terminate delivery under the guidance of a multidisciplinary team. It is recommended that a cesarean section under epidural anesthesia or combined spinalepidural anesthesia should be performed. In addition, these patients often have high mortalityduring perioperative period and postpartum, so we must pay attention to the monitoring and management of childbirth and postpartum.
作者
陈璐
陈宇清
CHEN Lu;CHEN Yu-qing(Department of Obstetrics, Fujian Provincial Hospital, Fuzhou 350001, China)
出处
《创伤与急诊电子杂志》
2017年第4期193-196,共4页
Journal of Trauma and Emergency(Electronic Version)
关键词
妊娠
结缔组织病
肺动脉高压
Pregnancy
Connective tissue disease
Pulmonary arterial hypertension