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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.

Background Pulmonary vascular changes in postoperative pulmonary artery hypertension (PAH) are similar to those seen in idiopathic PAH. Data are sparse on direct comparative midterm outcomes for these 2 high-risk populations. Methods Patients with idiopathic or postoperative PAH referred to a large tertiary hospital between June 2005 and July 2019 were retrospectively evaluated. Results A total of 364 consecutive patients were studied, including 201 postoperative PAH patients and 163 patients with idiopathic PAH, with a median age of 18.7 (interquartile range 10.0, 31.5) and 7.3 (IQR: 2.9, 18.3) years, respectively. PAH-specific drugs were used in 77.7% of patients; 31.4% received combination therapy. Patients with idiopathic PAH had a shorter 6-mintue walk distance, lower percutaneous oxygen saturation, and higher B-type natriuretic peptide levels than those with postoperative PAH at diagnosis (all P < 0.001), During a median follow-up time of 3.4 (interquartile range: 2.1, 5.8) years, 56 patients (15.4%) died, and one underwent bilateral lung transplantation. Patients with postoperative PAH had better survival than those with idiopathic PAH, according to age (hazard ratio [HR] 0.128, 95% confidence interval [CI]: 0.07-0.22, P < 0.0001); Kaplan–Meier survival estimates at 5 years for idiopathic and postoperative PAH patients were 74.3% and 92.6%, respectively. Patients in New York Heart Association functional class III–IV had an over 4-fold increased risk of death (HR 4.85, 95% CI: 2.61-9.00, P < 0.0001). Patients < 18 years of age at idiopathic PAH diagnosis had a worse survival compared to adult patients (HR 6.90, 95% CI: 4.19-15.56, P = 0.040). Conclusions Postoperative-PAH patients had better midterm survival compared to patients with idiopathic PAH. Mortality was significant in both PAH groups, reinforcing the need for early diagnosis and optimal individualized management to improve outcomes.