Department of Cardiology, Specialistic Hospital in Zabrze, 41-800 Zabrze, Poland.
Second Department of Cardiology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, 40-055 Katowice, Poland.
Int J Environ Res Public Health. 2023 Mar 6;20(5):4625. doi: 10.3390/ijerph20054625.
Pulmonary arterial hypertension (PAH) is defined as an elevated mean pulmonary artery pressure (mPAP) of >20 mmHg together with a pulmonary arterial wedge pressure (PAWP) of ≤15 mmHg and pulmonary vascular resistance (PVR) of>2 Wood units (WU). Although the total mortality of pregnant women with PAH has decreased significantly in recent years and is reported to be around 12% in some databases, total mortality is still at an unacceptably high percentage. Moreover, some subgroups, such as patients with Eisenmenger's syndrome, have a particularly high mortality rate of up to 36%. Pregnancy in patients with PAH is contraindicated; its appearance is an indication for a planned termination. Education of patients with PAH, including counseling on effective contraception, is essential. During pregnancy, blood volume, heart rate, and cardiac output increase, while PVR and systemic vascular resistance decrease. The hemostatic balance is shifted towards hypercoagulability. Among PAH-specific drugs, the use of inhaled or intravenous prostacyclins, phosphodiesterase inhibitors, and calcium channel blockers (in patients with preserved vasoreactivity) is acceptable. Endothelin receptor antagonists and riociguat are contraindicated. Childbirth can take place through either vaginal delivery or caesarean section; similarly, neuraxial and general anesthesia have proven indications. In a situation where all pharmacological options have been used in pregnant or postpartum patients in a serious condition, veno-arterial ECMO is a useful therapeutic option. For PAH patients who want to become mothers, an option that does not endanger their lives is adoption.
肺动脉高压（PAH）定义为平均肺动脉压（mPAP）>20mmHg，同时肺动脉楔压（PAWP）≤15mmHg 和肺血管阻力（PVR）>2 伍德单位（WU）。尽管近年来患有 PAH 的孕妇的总死亡率显著下降，据一些数据库报道约为 12%，但总死亡率仍然高得令人无法接受。此外，一些亚组，如艾森曼格综合征患者，死亡率特别高，高达 36%。PAH 患者怀孕是禁忌的；其出现是计划终止妊娠的指征。PAH 患者的教育，包括关于有效避孕的咨询，是必不可少的。在怀孕期间，血容量、心率和心输出量增加，而 PVR 和全身血管阻力降低。止血平衡向高凝状态转移。在 PAH 特异性药物中，可接受使用吸入或静脉前列腺素、磷酸二酯酶抑制剂和钙通道阻滞剂（在具有血管反应性保留的患者中）。内皮素受体拮抗剂和利奥西呱是禁忌的。分娩可以通过阴道分娩或剖宫产进行；同样，椎管内和全身麻醉已被证明有适应证。在所有药物治疗选择均已用于严重病情的孕妇或产后患者的情况下，静脉动脉 ECMO 是一种有用的治疗选择。对于希望成为母亲的 PAH 患者，一种不会危及生命的选择是收养。
