Pregnancy in women with pulmonary arterial hypertension (PAH) is considered to be associated with prohibitive maternal mortality. During the past decade, new advanced therapies for PAH have emerged and progress in high-risk pregnancy management has been made. We examined whether these changes have improved outcomes in parturients with PAH. A systematic review of all cases of parturients with idiopathic pulmonary hypertension (iPAH), congenital heart disease associated with PAH (CHD-PAH), or PAH of other aetiology (oPH) published in the past decade (1997-2007) was performed. Outcome data from this study were then compared with relevant data published between 1978 and 1996. Forty-eight case reports or case series met the inclusion criteria, totalling 73 parturients with PAH. Seventy-two per cent of patients with iPAH were receiving advanced therapies, compared with 52% of CHD-PAH and 47% of oPH. Although a publication bias cannot be excluded, overall maternal mortality was significantly lower compared with previous era (25 vs. 38%, P = 0.047) and was 17% in iPAH, 28% in CHD-PAH, and 33% in oPH. Seventy-eight per cent of deaths occurred within the first month after delivery. Primigravidae and parturients who received general anaesthesia were at higher risk of death (OR 3.70, 95% CI 1.15-12.5, P = 0.03 and OR 4.37, 95% CI 1.28-16.50, P = 0.02, respectively). Maternal mortality in parturients with PAH remains prohibitively high, despite lower death rates than previous decades. Early advice on pregnancy risks, including contraception, remains paramount. Women with PAH who become pregnant warrant a multidisciplinary approach with consideration of advanced therapies.