Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge

Abstract Background: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Methods: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. Results: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. Conclusions: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.

摘要 背景：外科技术的进步使得患有复杂性先天性心脏病（Complex Congenital Heart Disease, CCC）的育龄女性数量逐年攀升。然而，由于残留心脏病变类型繁杂多样，针对该人群的妊娠咨询方案尚未形成统一规范。研究目的：本研究旨在评估复杂性先天性心脏病孕妇的胎儿与母体妊娠结局，并分析预后相关的预测变量。研究方法：本研究对10年间纳入的435例先天性心脏病患者的连续妊娠病例进行随访，其中选取40例患者的42次妊娠数据进行分析，患者平均年龄为25.5±4.5岁，均确诊为复杂性先天性心脏病且曾被医师建议避免妊娠。患者的基础心脏病变类型包括：大动脉转位（D-Transposition of the great arteries）、肺动脉闭锁、三尖瓣闭锁、单心室、双出口心室及永存动脉干。妊娠前接受的手术方式包括Fontan术、Jatene术、Rastelli术、Senning术、Mustard术，以及Blalock、Taussing、Glenn等其他手术术式。其中8例（20.0%）患者未接受过术前手术治疗。19例（47.5%）患者存在低氧血症。临床随访方案涵盖血氧饱和度监测、血红蛋白与血细胞比容检测；根据妊娠进程调整用药方案，必要时给予抗凝治疗；重症患者需自妊娠28周起住院观察。本研究设定的统计学显著性阈值为p<0.05。研究结果：仅17例（40.5%）妊娠实现了母体与胎儿的平稳结局。共发生13例（30.9%）母体并发症，2例（4.7%）母体死亡，死因为产后出血与重度子痫前期，两例均为低氧血症患者。本研究中共出现7例（16.6%）死胎，17例（40.5%）早产婴儿，2例（4.1%）婴儿被确诊患有先天性心脏病。低氧血症患者的母体与胎儿并发症发生率显著更高（p<0.05）。研究结论：复杂性先天性心脏病女性妊娠时面临较高的母体及子代风险。低氧血症是预测母体与胎儿不良结局的重要变量。即使在专业医疗中心接受治疗，复杂性先天性心脏病女性也应被充分告知妊娠相关风险，并建议避免妊娠。