1 Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, China.
2 Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College, UK.
Eur J Prev Cardiol. 2019 Jul;26(10):1067-1076. doi: 10.1177/2047487318821246. Epub 2019 Apr 11.
Pulmonary arterial hypertension is a severe complication in patients with congenital heart disease and poses a significant risk to women wishing to become pregnant. This study describes the clinical presentation, maternal outcomes and risk factors for the peripartum period in women with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD).
All pregnant women with PAH-CHD who were admitted for delivery in a tertiary center between February 2011-September 2016 were included. Logistic regression analysis was used to identify predictors of the combined endpoint of maternal death, severe heart failure requiring treatment, or pulmonary hypertensive crisis.
Ninety-three women (94 pregnancies) were included. Average age was 27.5 ± 4.4 years. Thirty (31.9%) patients had Eisenmenger syndrome, 51 (54.3%) had pulmonary arterial hypertension associated with systemic-to-pulmonary shunts, and 13 (13.8%) had pulmonary arterial hypertension with corrected congenital heart disease. Twenty-three (24.5%) women required admission for delivery within two days from presentation. Elective Cesarean section was performed in 95.7% of women, with intravertebral anesthesia in 93.6%. Fifty-one (54.2%) patients received pulmonary arterial hypertension therapies during pregnancy. Six (6.4%) women died, 33 (35.1%) developed heart failure and 10 (10.6%) had a pulmonary hypertensive crisis. Patients who met the combined endpoint ( = 34, 36.2%) were more likely to have Eisenmenger syndrome or repaired defects ( < 0.001). Other risk factors in the multivariate model included lower arterial blood oxygen saturation, higher brain natriuretic peptide, and pericardial effusion on echocardiography.
Maternal mortality and morbidity remain high in PAH-CHD patients, who should be counseled on the risks of pregnancy and managed in a tertiary multidisciplinary environment to improve prognosis.
肺动脉高压是先天性心脏病患者的一种严重并发症，对希望怀孕的女性构成重大风险。本研究描述了与先天性心脏病相关的肺动脉高压（PAH-CHD）患者围产期的临床表现、母婴结局和危险因素。
纳入 2011 年 2 月至 2016 年 9 月期间在一家三级中心分娩的所有患有 PAH-CHD 的孕妇。采用逻辑回归分析识别母婴死亡、严重心力衰竭需要治疗或肺动脉高压危象的复合终点的预测因素。
93 名女性（94 例妊娠）纳入研究。平均年龄为 27.5±4.4 岁。30 名（31.9%）患者患有艾森曼格综合征，51 名（54.3%）患有与体肺分流相关的肺动脉高压，13 名（13.8%）患有校正型先天性心脏病合并肺动脉高压。23 名（24.5%）患者在就诊后 2 天内需要入院分娩。95.7%的女性接受选择性剖宫产，93.6%采用椎管内麻醉。51 名（54.2%）患者在妊娠期间接受肺动脉高压治疗。6 名（6.4%）女性死亡，33 名（35.1%）发生心力衰竭，10 名（10.6%）发生肺动脉高压危象。符合复合终点（ = 34，36.2%）的患者更有可能患有艾森曼格综合征或修复性缺陷（ < 0.001）。多变量模型中的其他危险因素包括动脉血氧饱和度降低、脑利钠肽水平升高和超声心动图检查有心包积液。
PAH-CHD 患者的母婴死亡率和发病率仍然很高，应向其提供有关妊娠风险的咨询，并在三级多学科环境中进行管理，以改善预后。
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