Mandel R I, Bruner J P
Medical Corps, United States Army, Department of Obstetrics and Gynecology, Tripler Army Medical Center, Honolulu, Hawaii, USA.
Am J Perinatol. 1995 Sep;12(5):319-21. doi: 10.1055/s-2007-994484.
Pulmonary hypertension due to endocardial fibroelastosis is usually diagnosed during infancy and childhood and is almost uniformly lethal when severe. Since females with this disorder rarely reach reproductive age, no cases of successful pregnancy in the presence of this severe cardiopulmonary disease have been reported. A 23-year-old Caucasian primigravida with a history of congenital endocardial fibroelastosis and severe pulmonary hypertension presented at 20 weeks' gestation. Following cardiac catheterization, the pregnancy was managed with bed rest, oral theophylline and digoxin, and low-flow oxygen therapy. After spontaneous onset of labor at 35 weeks, invasive hemodynamic monitoring and epidural anesthesia were initiated. Worsening of maternal pulmonary artery pressures postpartum was relieved by intravenous nitroglycerin infusion. Recent advances in medical care have resulted in more women with endocardial fibroelastosis reaching reproductive age. Successful pregnancy outcome is possible using established techniques of modern obstetric care.
心内膜弹力纤维增生症所致的肺动脉高压通常在婴幼儿期被诊断出来，病情严重时几乎无一例外会致命。由于患有这种疾病的女性很少能达到生育年龄，因此尚未有在这种严重心肺疾病情况下成功妊娠的病例报道。一名23岁有先天性心内膜弹力纤维增生症和严重肺动脉高压病史的白种初产妇在妊娠20周时前来就诊。心脏导管检查后，对该孕妇采取卧床休息、口服茶碱和地高辛以及低流量氧疗等措施进行管理。35周时自然发动分娩后，开始进行有创血流动力学监测和硬膜外麻醉。产后通过静脉输注硝酸甘油缓解了产妇肺动脉压力的恶化。医疗护理方面的最新进展使更多患有心内膜弹力纤维增生症的女性达到了生育年龄。采用现代产科护理的既定技术有可能实现成功的妊娠结局。
