Sexual Health-related Quality of Life in Women with Pulmonary Arterial Hypertension: Compensating for Loss.

Rationale: Health-related quality of life in patients with pulmonary arterial hypertension (PAH) has become increasingly important in disease management as numerous treatment options have improved prognosis and time to clinical worsening. Sexual health-related quality of life (SHRQoL) is poorly understood in patients with PAH, but previous work has shown that patients may face unrecognized challenges, especially related to parenteral prostanoid analogue therapies. Objectives: Using qualitative methods, to describe challenges and perspectives related to SHRQoL among women with PAH. Methods: We conducted 13 semistructured in-depth interviews at the Pulmonary Hypertension Association's International Pulmonary Hypertension Conference and Scientific Sessions among female attendees with World Symposium on Pulmonary Hypertension group 1 PAH. A coding structure using both deductive and inductive coding was developed to organize and analyze data using applied thematic analysis. Salient themes were identified and are presented here using summary and illustrative quotations. Results: Ninety-two percent (12 of 13) of participants reported declines in the frequency of sex after diagnosis of PAH. A significant portion (62% [8 of 13]) experienced fear of having sexual intercourse because of cardiopulmonary symptoms. All participants (100% [13 of 13]) reported compensatory behaviors/strategies during and around sexual intercourse; some participants on subcutaneous prostanoids also reported timing intercourse to coincide with infusion site changes and, as a result, interrupted treatment during this time. Participants reported changing positions during sex to reduce breathlessness, and some reported removing oxygen to avoid interrupting intimacy. Most participants endorsed negative body image related to their medications, external oxygen supplementation, and/or body weight fluctuations (54% [7 of 13]). Many participants revealed that they had never discussed sexual practices with healthcare professionals and desired increased communication and discussion with their providers. Conclusions: Women with PAH face significant burdens and challenges regarding SHRQoL. PAH therapies directly affect SHRQoL. Further targeted qualitative and quantitative studies are needed to better characterize and improve SHRQoL in patients with PAH.

Insights from the Menstrual Cycle in Pulmonary Arterial Hypertension.

Rationale: Sex hormones play a role in pulmonary arterial hypertension (PAH), but the menstrual cycle has never been studied.Objectives: We conducted a prospective observational study of eight women with stable PAH and 20 healthy controls over one cycle.Methods: Participants completed four study visits 1 week apart starting on the first day of menstruation. Relationships between sex hormones, hormone metabolites, and extracellular vesicle microRNA (miRNA) expression and clinical markers were compared with generalized linear mixed modeling.Results: Women with PAH had higher but less variable estradiol (E2) levels (P < 0.001) that tracked with 6-minute walk distance (P < 0.001), N-terminal prohormone of brain natriuretic peptide (P = 0.03) levels, and tricuspid annular plane systolic excursion (P < 0.01); the direction of these associations depended on menstrual phase. Dehydroepiandrosterone sulfate (DHEA-S) levels were lower in women with PAH (all visits, P < 0.001). In PAH, each 100-µg/dl increase in DHEA-S was associated with a 127-m increase in 6-minute walk distance (P < 0.001) and was moderated by the cardioprotective E2 metabolite 2-methoxyestrone (P < 0.001). As DHEA-S increased, N-terminal prohormone of brain natriuretic peptide levels decreased (P = 0.001). Expression of extracellular vesicle miRNAs-21, -29c, and -376a was higher in PAH, moderated by E2 and DHEA-S levels, and tracked with hormone-associated changes in clinical measures.Conclusions: Women with PAH have fluctuations in cardiopulmonary function during menstruation driven by E2 and DHEA-S. These hormones in turn influence transcription of extracellular vesicle miRNAs implicated in the pathobiology of pulmonary vascular disease and cancer.

Sexual health and health-related quality of life among women with pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is characterized by progressive limitations in physical activity and health-related quality of life (HRQoL). HRQoL deficits may extend beyond the traditional domains of physical activity, psychological health, and emotional wellbeing to sexual health and function. Sexual HRQoL has not been studied in PAH, nor has the impact of PAH therapies themselves on sexual health and intimacy. In this initial investigation, we sought to explore HRQoL among women diagnosed with PAH and to determine if PAH treatment type (intravenous or subcutaneous prostanoids versus oral medications) was associated with levels of self-reported HRQoL assessed by validated measures for PAH-specific, general, and sexual HRQoL. We administered the emPHasis-10, Short Form (SF)-36, Female Sexual Dysfunction Scale-Revised (FSDS-R), and the Arizona Sexual Experience Scale (ASEX) to 35 women with self-reported World Health Organization Group 1 PAH at the 2016 Pulmonary Hypertension Association International Conference and Scientific Sessions. HRQoL instruments demonstrated excellent internal reliability. Women with PAH had high levels of sexual distress captured with the FSDS-R scale. The FSDS-R (but not ASEX) was significantly correlated to emPHasis-10 ( r = 0.64, p < 0.01) and most SF-36 domains ( r = - 0.36 to - 0.64, p < 0.05). Participants treated with intravenous or subcutaneous prostanoids had higher (worse) FSDS-R scores than those on oral therapies while ASEX, emPHasis-10, and SF-36 scores were similar across treatment types. Sexual HRQoL may impact overall quality of life in PAH and specific assessment of sexual health and functioning within intimate relationships may detect deficits in wellbeing not addressed by established HRQoL metrics. Further study to address all aspects of HRQoL in PAH is required.