Volume 10: Issue 3

Deborah Jo Levine, MD

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Kelly Chin, MD

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Although pulmonary hypertension is not a uniquely female problem, women living with pulmonary hypertension often have female-specific concerns; such as, whether they should get pregnant; what types of contraception are safe and effective in PH; and whether routine health-maintenance care and exams are necessary. Separately, the increased incidence and prevalence of pulmonary hypertension in women vs men has led to considerable discussion over the years as to the role for sex hormones in the pulmonary vasculature and in the development of pulmonary hypertension.

The Clinical Trials Update highlights new and ongoing research trials that are relevant to the diagnosis and treatment of PAH. In this issue, Corey Ventetuolo, MD, MS, describes a study on the relationships among genetic predictors of sex hormone processing, sex hormones and their metabolites, and RV measures both at baseline and longitudinally in male and female participants from the Multi-Ethnic Study of Atherosclerosis–Right Ventricle Study.

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by pulmonary vascular remodeling of the distal pulmonary vasculature, ultimately leading to destruction and loss of the smallest pulmonary arteries.1 The ensuing syndrome of PAH is clinically characterized by reduced pulmonary arterial circulatory flow, resulting in increased pulmonary vascular resistance, which ultimately results in failure of the right heart.2 In both children and adults, PAH presents as a primary disease or in association with a diverse range of diseases such as connective tissue diseases, portal hypertension, and congenital heart disease.3 Nearly all forms of World Health Organization (WHO) Group 1 PAH demonstrate a skewed gender ratio with significantly more females diagnosed with PAH than males.4–6

While the mechanistic details behind the female predominance remain unclear, this gender discrepancy may represent an opportunity for advanced biologic understanding and future therapeutic development. This article will briefly discuss the intersection of human, in vitro, and animal studies of PAH, and highlight the conflicting data that others have discussed and elegantly elaborated upon as the “estrogen paradox” in PAH.7–9

The World Health Organization describes contraception as desirable for many reasons. It allows families to make informed choices about their reproductive years. It allows family planning, with spacing of children, leading to better bonding between mother and child, higher exclusive breastfeeding rates, and improved health of the mother for a future pregnancy.1

Unintended pregnancies continue to occur at an astronomically high rate in the US, as high as 35% by most recent data,2 and of course, since women with pulmonary arterial hypertension (PAH) are not exempt from this statistic, it is imperative that as care providers, we help our patients to plan ahead. In their case, maternal health is our main concern.

Weiss and colleagues, reporting on pregnancy outcomes with PAH between 1978 and 1996, found a maternal mortality of 36% in Eisenmenger syndrome, 30% in idiopathic PAH, and 56% in secondary PAH.3 A more recent systematic review reported slightly more optimistic pregnancy outcomes from 1997 to 2007. Still, maternal mortality ranged from 17% to 33%, depending on the exact etiology of pulmonary hypertension.4 It is therefore very clear that in the setting of PAH, contraception is important and potentially lifesaving.

The Uneasy Conversation

Guest editors' memo

Preventive Health Care for Women with Pulmonary Hypertension

The Gender/Estrogen Paradox and the Right Ventricle in Pulmonary Vascular Disease

Gender, Sex Hormones, and Pulmonary Arterial Hypertension

Contraceptive Options for the Patient with Pulmonary Arterial Hypertension

PAH and Pregnancy: Physiologic Changes, Challenges, and Outcomes

The Challenges of PAH in Pregnancy

PAH Therapies in Men: Often Wondered, Seldom Asked

Conference Abstracts

Guest Editor’s Memo

A Roundtable Discussion Addressing Patient-Centered Care and Quality of Life in Patients With Pulmonary Arterial Hypertension

Editor’s Memo

Pulmonary Hypertension Associated With Parenchymal Lung Disease