抽象
辅导妊娠vasoresponder IPAH需要慎重考虑http://ow.ly/qunk30fXCRH
致编辑:
肺动脉高血压(PAH)导致肺血管阻力增加,右心室衰竭和过早死亡。大约一半的患者的PAH被诊断为特发性肺动脉高压(IPAH),其中没有明显的原因被确定[1,2]。与IPAH在世界卫生组织(WHO)的功能类别I-III可与血管扩张的挑战,如吸入一氧化氮,患者右心脏导管插入术期间(RHC)的研究[3]。“Vasoresponsive” IPAH被普遍认为是与无反应者从一个完全不同的病程[优异的预后指标4]。怀孕是明确的患者,因为它已与产妇和胎儿死亡的高利率相关的肺动脉高压(PH),建议不要[1,2]。但是,也有在vasoresponders有限报告。
向我们的专家肺动脉高压中心,一名27岁的白人女子已经移居到英国。她报告呼吸困难增加和减少运动耐受只有几码的6个月的历史。此前,她已经适应和积极的。小号he was in WHO functional class IV with a history of four syncopal episodes in the previous 6 months. There was no past medical history of note except sertraline taken for anxiety. She denied anorexigenic or illicit drug use. She underwent standard investigations [1,2]为确认IPAH(表格1)。[RHC revealed a mean pulmonary arterial pressure (mPAP) of 52 mmHg with a cardiac output of 3.9 L·min-1通过热稀释和11.5木材单位肺血管阻力。Upon inhalation of 20 ppm NO, mPAP fell to 27 mmHg. Her cardiac output was preserved at 4.2 L·min-1在以积极的血管反应试验是一致的。通气/灌注扫描排除慢性血栓栓塞PH。Amlodipine was commenced and titrated up to a dose of 20 mg once daily. Anticoagulation with warfarin was commenced. She was strongly advised against pregnancy and encouraged to use dual methods of contraception.
Although functional class IV patients would not routinely be recommended to proceed with oral therapy, 4 months after initiation of therapy, the patient reported resolution of symptoms and was assessed as WHO class I. This was reflected in significant improvements in right heart function and haemodynamics (表格1)。
患者搬回国外怀孕了。她决定怀孕,同时继续钙通道拮抗剂进行,但停止华法林。The pregnancy was uneventful until 10 days prior to planned, elective caesarean section, when she developed chest pain and was started on a course of low molecular weight heparin. Imaging was not performed at this time to assess for pulmonary embolism. 8 weeks after delivery an elective RHC showed worsening haemodynamics (mPAP 40 mmHg). Sildenafil (20 mg three times daily) was initiated. Despite this, she functionally deteriorated and a further RHC 3 months later revealed an mPAP of 56 mmHg. She was started on bosentan whilst continuing on amlodipine. Warfarin was reinitiated.
患者迁回英国,又在我中心进行了评估。尽管联合治疗,她仍然在WHO III级。她报告中央胸部不适劳力时解除由休息,心悸持续分钟,每周大约三次,而不是与晕厥或晕厥有关。进行了24小时的带和无异常。鉴于她的恶化,她接受计算机断层肺动脉造影。这并没有表现出血栓栓塞性疾病,冠心病压缩或将指向额外的或替代的诊断发现任何证据。目前还没有临床体征或调查结果提出一个误判初步诊断,如结缔组织病相关的PAH或肺静脉闭塞病,和促甲状腺激素水平正常。At repeat RHC, mPAP was found to be 56 mmHg with no significant response to inhalation of 40 ppm NO (表格1)。病人被转移到静脉注射前列腺素治疗,但不幸的是突然死亡,而住院,从心脏骤停。远程监护病房之前没有发现任何心律失常她逮捕。
怀孕是在对患者建议用PH [1,2]。妊娠在1978年和1996年之间的PH患者病例的系统评价报告在怀孕30%的产妇死亡率在IPAH患者[五]。目前还不太清楚如何律师vasoresponders,与文献有些含糊其词。妊娠患者的回顾性,多中心分析1999年到2009年间PAH在美国报告了一组12名妇女与PAH谁选择继续妊娠的2例(16.7%)死亡。They reported no deaths (within their follow up interval of 2 months) in the four women in the cohort who had a diagnosis of IPAH [6]。据英国中心进行妇女PH的回顾性分析谁怀孕了,并选择继续妊娠超过7年的时间。They reported one (10%) maternal death associated with 10 pregnancies in nine women during their median follow up interval of 3.2 years [7]。在10年间1997年至2007年病例系统评价发现改进的死亡率为17%,女性IPAH经受怀孕[8]。怀孕的妇女PAH的最大注册表确定在13个欧洲中心26周妊娠超过2007年和2010年之间的3年期,并报告死亡率或移植率在女性IPAH谁怀孕了[24%9]。患者vasoresponsive IPAH该小组没有表现出产妇死亡率和都认为有一个成功怀孕。在一年的随访产后,没有这一批经验丰富,需要改变治疗临床恶化。作者发现,这些患者的表型不同,有那么严重PH谁比那些死亡或需要移植。他们的结论是怀孕能成功的患者有良好和积极管理这个选择亚群,但目前有数据不足以产生值来确定何时它可能是“安全”。
Here, we present a case of a patient who responded well to vasodilator therapy, became pregnant and although she progressed through pregnancy with no overt problems, she converted to a nonresponder phenotype and died within 2 years from rapidly progressive disease. We highlight that although there are examples of improved outcomes in vasoresponders, pregnancy may have the potential to dramatically change patient prognosis. We recognise that there are certain vasoresponsive patients who develop a progressive nonresponder phenotype in the absence of pregnancy. Although the temporal relationship of events in this case suggests that pregnancy had a role in the patient's deterioration, there is potential that these events were coincidental. Volume overload in the third trimester may have played a triggering role but we note that the patient continued to deteriorate over the medium term and lost her vasodilatory response, and therefore, regardless of the pathophysiological trigger, this carried profound prognostic implications.
这种情况下的亮点,在患者vasoresponsive IPAH的背景下,这是至关重要的不确定性围绕产后结果进行了讨论。有这方面的一些高质量的前瞻性数据,并给出vasoresponder群的稀有性,谁只占5%左右所有IPAH的,它是不太可能在未来推出。这种情况下,强调需要咨询的患者,即使在没有充分的证据,有可能是或怀孕后期间死亡或发病的一个显著机会,当是很清楚的。
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- 收到2017年6月8日。
- 公认2017年9月9日。
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