Better Pregnancy Outcomes in Patients With Pulmonary Arterial Hypertension

The contemporary outcomes of pregnancies in patients with pulmonary arterial hypertension (PAH) have improved substantially in patients with well-controlled disease, according to the results of a small study conducted in France.¹
   
PAH is a rare disease that is associated with right ventricular failure and premature death. Although PAH can develop in anyone at anytime, the disease occurs twice as often in women than in men and symptoms associated with the disease typically begin in early adulthood. Current recommendations suggest that women with PAH should avoid pregnancy, because complications such as hemodynamic instability or sudden death during delivery and the postpartum period are common.
   
According to the study authors, previous reports have shown that pregnancy in patients with PAH is associated with mortality rates of up to 56%.¹ However, the management of PAH has changed considerably in recent years. To assess whether pregnancy outcomes in patients with PAH have improved under these newer management strategies, the study authors followed 26 women with PAH who became pregnant during a 3-year period.
   
Of the 26 women, 3 (12%) died and 1 (4%) required an urgent heart-lung transplantation because of right heart failure. There were 8 abortions, 6 of which were induced and 2 were spontaneous. A total of 16 women delivered healthy newborns without any complications.
   
The 16 women whose pregnancies were successful had well-controlled PAH (defined as pulmonary vascular resistance of 500 ± 352 dyn•s•cm5). Half of them (8 women) were long-term responders to calcium channel blockers, reported the study authors. The 4 patients who died/needed a heart-lung transplantation had poorly controlled disease (pulmonary vascular resistance of 500 ± 209 dyn•s•cm5).
   
Although calcium channel blockers were very effective in nearly a third of the patients in this study, this class of medication typically is beneficial to only a small number of patients with PAH.² Also, the type of PAH seems to play an important role in which treatment is most effective. For example, calcium channel blockers seem to work best in patients with idiopathic PAH.
   
Overall, the outcome of pregnancy in women with PAH has improved, especially in those with well-controlled disease. However, the mortality rate associated with pregnancy and PAH remains substantial, and patients with PAH should continue to be counseled to avoid pregnancy.

Pertinent Points:
- Newer management strategies for pulmonary arterial hypertension (PAH) have resulted in improved pregnancy outcomes for women with PAH.
- Women with PAH who have well-controlled disease, especially long-term responders to calcium channel blockers, have the best pregnancy outcomes (delivery of healthy baby with no complications).
- The general recommendation to avoid pregnancy in all patients with PAH remains prudent.
 

References:

1. Jais X, Olsson KM, Barbera JA, et al. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J. 2012;40:881-885.
2. Robbins I. The role of calcium channel blockers in pulmonary arterial hypertension. Available at: http://www.medscape.org/viewarticle/523338. Accessed May 17, 2013.