Pregnancy outcome in women with Eisenmenger’s syndrome: a case series from west China

Duan, Ruiqi; Xu, Xinjiang; Wang, Xiaodong; Yu, Haiyan; You, Yong; Liu, Xinghui; Xing, Aiyun; Zhou, Rong; Xi, Mingrong

doi:10.1186/s12884-016-1153-z

Cited by 45 publications

(41 citation statements)

References 25 publications

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“…12 Similar to our finding, the presence of Eisenmenger syndrome was associated with worst outcome, both for maternal and fetal survival. 12 RV dysfunction and failure are also associated with worsened outcome for pregnant women with CHD-PH. 13 Reduced TAPSE (mean TAPSE < 19 mm) was observed in nonsurvivors, which indicated the reduced RV systolic function during current pregnancy.…”

Section: Discussionsupporting

confidence: 88%

Severe pulmonary hypertension and reduced right ventricle systolic function associated with maternal mortality in pregnant uncorrected congenital heart diseases

et al. 2019

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Background: Pregnant uncorrected congenital heart disease patients, especially those who already developed pulmonary hypertension, have increased risk for maternal mortality. The pulmonary hypertension severity and right ventricle function may be associated with higher maternal mortality. The study aimed to investigate the mortality rate of pregnant uncorrected congenital heart disease and the impact of pulmonary hypertension severity on mortality. Methods: This is the sub study of COngenital HeARt Disease in adult and Pulmonary Hypertension Registry. The data of pregnant uncorrected congenital heart disease patients were analyzed from registry database. The maternal mortality was recorded. The data of demography, clinics, obstetrics, and transthoracic echocardiography were collected. The factors that influenced maternal mortality were analyzed. A statistical significance was determined when p value < 0.05. Results: From 2012 until 2017, there were 78 pregnant congenital heart disease patients. Of them, 56 patients were eligible for analyses. The majority of congenital heart disease was atrial septal defect (91.1%). The maternal mortality rate was 10.7% (6 of 56). Pulmonary hypertension occurred in 48 patients, therefore the maternal mortality rate among congenital heart diseasepulmonary hypertension with majority of atrial septal defect was 12.5% (6 of 48). Among nonsurvivors, 100% suffered from severe pulmonary hypertension as compared to survivors (56.0%), p ¼ 0.041. Most nonsurvivors were Eisenmenger syndrome (83.3%), significantly higher compared to survivors (22.0%), p ¼ 0.006. Nonsurvivors had significantly worsened WHO functional class, reduced right ventricle systolic function, and right heart failure. The modes of maternal death were severe oxygen desaturation (66.7%) and respiratory failure and sepsis (33.3%). Most of the maternal deaths occurred within 24 h postpartum period. Conclusion: Maternal mortality rate among pregnant uncorrected congenital heart disease with majority of atrial septal defect was 10.7% and among congenital heart disease-pulmonary hypertension with majority of atrial septal defect was 12.5%. Factors related with maternal mortality were severe pulmonary hypertension, Eisenmenger syndrome, and reduced right ventricle systolic function.

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Section: Discussionsupporting

confidence: 88%

Severe pulmonary hypertension and reduced right ventricle systolic function associated with maternal mortality in pregnant uncorrected congenital heart diseases

et al. 2019

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“…The advantages of a Cesarean delivery include avoiding hemodynamic instability related to uterine contractions and pain, but this should be performed by an expert specialist multidisciplinary team. 20,21 Two patients had unplanned general anesthesia in this study and died. As in other studies, general anesthesia was associated with a high risk of death in pregnant patients with PAH-CHD, especially in ES, attributable to the adverse effects of intubation and associated hemodynamic changes.…”

Section: Discussionmentioning

confidence: 81%

Peripartum outcomes in a large population of women with pulmonary arterial hypertension associated with congenital heart disease

Dimopoulos

Liu

et al. 2019

Eur J Prev Cardiolog

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Aims 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). Methods 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. Results 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 ( n = 34, 36.2%) were more likely to have Eisenmenger syndrome or repaired defects ( p < 0.001). Other risk factors in the multivariate model included lower arterial blood oxygen saturation, higher brain natriuretic peptide, and pericardial effusion on echocardiography. Conclusion 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.

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“…We included 13 studies, with a total of 272 pregnancies in 258 women with PAH. 5 – 17 A total of 214 pregnancies were advanced beyond 20 weeks. The description of included studies is shown in Table 1 .…”

Section: Resultsmentioning

confidence: 99%

“…In the eight studies that reported the cause of prematurity, most were iatrogenic; 84% ( n = 63/75) secondary to a planned premature delivery usually for maternal indications. 5 , 6 , 9 , 11 , 12 , 14 , 16 , 17 In the eight studies that reported birthweights, 5 – 7 , 9 , 10 , 13 , 14 , 17 the mean birthweight was at 1922 ± 292 g and 36% of babies were reported to be small for gestational age.…”

Section: Resultsmentioning

confidence: 99%

Pulmonary arterial hypertension in pregnancy—a systematic review of outcomes in the modern era

et al. 2021

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Background: Pregnancy is hazardous with pulmonary arterial hypertension (PAH), but the risks may have improved in recent years. We sought to systematically evaluate PAH and pregnancy-related outcomes in the last decade. Methods: We searched for articles describing outcomes in pregnancy cohorts published between 2008-2018. 3658 titles were screened and 13 studies included for analysis. Pooled incidences and percentages of maternal and perinatal outcomes were calculated.Â Results: Out of 272 pregnancies, 214 pregnancies advanced beyond 20 gestational weeks. The mean maternal age was 28Â±2 years, mean pulmonary artery systolic pressure on echocardiogram was 76Â±19mmHg. Aetiologies include idiopathic PAH 22%, congenital heart disease 64%, and others 15%. Majority (74%) had good functional class I/II. Only 48% of women received PAH-specific therapy. Premature deliveries occur in 58% of pregnancies at mean of 34Â±1 weeks, most (76%) had caesarean section. Maternal mortality rate was 12% overall (n=26); even higher for idiopathic PAH aetiology alone (20%). Reported causes of death included right heart failure, cardiac arrest, PAH crises, pre-eclampsia and sepsis. 61% of maternal deaths occur at 0-4 days post-partum. Stillbirths rate was 3% and neonatal mortality rate 1%. Conclusions: PAH in pregnancy continues to be perilous with high maternal mortality rate. Continued prospective studies are needed.

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Pregnancy outcome in women with Eisenmenger’s syndrome: a case series from west China

Cited by 45 publications

References 25 publications

Severe pulmonary hypertension and reduced right ventricle systolic function associated with maternal mortality in pregnant uncorrected congenital heart diseases

Severe pulmonary hypertension and reduced right ventricle systolic function associated with maternal mortality in pregnant uncorrected congenital heart diseases

Peripartum outcomes in a large population of women with pulmonary arterial hypertension associated with congenital heart disease

Pulmonary arterial hypertension in pregnancy—a systematic review of outcomes in the modern era

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