Advances in Pulmonary Hypertension

Erika Berman Rosenzweig, MD

The conversation never comes easily as I counsel a young woman with newly-diagnosed pulmonary arterial hypertension (PAH) about the extreme risks of pregnancy. In that moment, I know I've shattered a life-long dream. Despite the many medical advances in the field of pulmonary hypertension, pregnancy and PAH remain a lethal combination. And, in a disease that affects more women than men, most experts believe that hormonal influences play a key role in the pathophysiology of the disease; however the exact mechanisms remain unclear. In this issue of Advances, guest editors Drs Kelly Chin and Deborah Levine call on authors to address this delicate topic. Dr Dianne Zwicke's article provides readers with a review of the normal physiology of pregnancy and highlights the pathophysiological derangements that make pregnancy so dangerous for women with Group 1 PAH. Given the risks of pregnancy to both mother and fetus, including potential teratogenic effects of some of the targeted PH agents, Dr Patricia Santiago-Munoz also provides a comprehensive review of contraceptive options for women with PAH. In Dr Eric Austin's article about sex hormones and PAH, the reader can learn about potential mechanisms of hormonal influences in PH. Finally, in the roundtable discussion, experts discuss controversies surrounding the topic of pregnancy and PH. All acknowledged that while pregnancy can be fatal and should be avoided in women with PAH, management guidelines should still be developed for practitioners who find themselves caring for a woman presenting with PAH during pregnancy. Another key message that emerged from this discussion was that the management requires a multidisciplinary team of experts with experience taking care of PH patients at a major medical center, as these patients require meticulous monitoring through all stages of pregnancy including the early post-partum period for sudden cardiovascular collapse. One can only hope that in the future we will have a better understanding of these hormonal influences, which may uncover potential therapeutic targets, and that we may be able to counsel women with PAH differently. Until then, despite the many advances in management of PAH, the conversation remains sobering.