Sex-specific differences and survival in patients with idiopathic pulmonary arterial hypertension 2008–2016

Kjellström, Barbro; Nisell, Margret; Kylhammar, David; Bartfay, Sven‐Erik; Ivarsson, Bodil; Rådegran, Göran; Hjalmarsson, Clara

doi:10.1183/23120541.00075-2019

Cited by 21 publications

(12 citation statements)

References 29 publications

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“…The study population showed a near-equal distribution between women and men. Interestingly, our cohort is quite comparable to that of the Swedish Pulmonary Arterial Hypertension Registry, which has been published recently (17). Absence of female predominance in the present study may be related to sample size and yet-unclear evolution of referral biases (17).…”

Section: Discussionsupporting

confidence: 75%

“…Interestingly, our cohort is quite comparable to that of the Swedish Pulmonary Arterial Hypertension Registry, which has been published recently (17). Absence of female predominance in the present study may be related to sample size and yet-unclear evolution of referral biases (17). Diastolic echocardiographic indexes of ventricular function were not assessed and need further evaluation since echocardiography is more accessible than CMR in most PH centers.…”

Section: Discussionsupporting

confidence: 62%

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Right ventricular function correlates of right atrial strain in pulmonary hypertension: a combined cardiac magnetic resonance and conductance catheter study

Tello

Dalmer

Vanderpool

et al. 2020

American Journal of Physiology-Heart and Circulatory Physiology

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The functional relevance of right atrial (RA) function in pulmonary hypertension (PH) remains incompletely understood. The purpose of this study was to explore the correlation of cardiac magnetic resonance (CMR) feature tracking-derived RA phasic function with invasively measured pressure-volume (P-V) loop-derived right ventricular (RV) end-diastolic elastance ( Eed) and RV-arterial coupling [ratio of end-systolic elastance to arterial elastance ( Ees/ Ea)]. In 54 patients with severe PH, CMR was performed within 24 h of diagnostic right heart catheterization and P-V measurements. RA phasic function was assessed by CMR imaging of RA reservoir, passive, and active strain. The association of RA phasic function with indexes of RV function was evaluated by Spearman’s rank correlation and linear regression analyses. Median [interquartile range] RA reservoir strain, passive strain, and active strain were 19.5% [11.0–24.5], 7.0% [4.0–12.0], and 13.0% [7.0–18.5], respectively. Ees/ Ea was 0.73 [0.48–1.08], and Eed was 0.14 mmHg/mL [0.05–0.22]. RV diastolic impairment [RV end-diastolic pressure (EDP) and Eed] was correlated with RA phasic function, but Ea and Ees were not. In addition, RA phasic function was correlated with inferior vena cava diameter. In multivariate linear regression analysis, adjusting for key P-V loop indexes, Eed and EDP remained significantly associated with RA phasic function. We conclude that RA phasic function is altered in relation to impaired diastolic function of the chronically overloaded right ventricle and contributes to backward venous flow and systemic congestion. These results call for more attention to RA function in the management of patients with PH. NEW & NOTEWORTHY There is growing awareness of the importance of the right atrial (RA)-right ventricular (RV) axis in pulmonary hypertension (PH). Our results uncover alterations in RA phasic function that are related to depressed RV lusitropic function and contribute to backward venous return and systemic congestion in chronic RV overload. Assessment of RA function should be part of the management and follow-up of patients with PH.

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Section: Discussionsupporting

confidence: 75%

Section: Discussionsupporting

confidence: 62%

Right ventricular function correlates of right atrial strain in pulmonary hypertension: a combined cardiac magnetic resonance and conductance catheter study

Tello

Dalmer

Vanderpool

et al. 2020

American Journal of Physiology-Heart and Circulatory Physiology

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“…A greater proportion of female PAH patients has been noted in several registries, ranging from 56% to 86%, implicating female sex as a risk factor for PAH [18,[20][21][22][23][24][25][26]. As seen in the COMPERA registry, this higher female to male ratio 2.3:1 in younger patients (median 54 years) seems to decrease as age at diagnosis rises (1.2:1 in 75-year-old patients) along with the onset of menopause [18].…”

Section: Epidemiologymentioning

confidence: 99%

Sex and gender in pulmonary arterial hypertension

et al. 2021

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Pulmonary arterial hypertension (PAH) is a rare disease characterised by pulmonary vascular remodelling and elevated pulmonary pressure, which eventually leads to right heart failure and death. Registries worldwide have noted a female predominance of the disease, spurring particular interest in hormonal involvement in the disease pathobiology. Several experimental models have shown both protective and deleterious effects of oestrogens, suggesting that complex mechanisms participate in PAH pathogenesis. In fact, oestrogen metabolites as well as receptors and enzymes implicated in oestrogen signalling pathways and associated conditions such as BMPR2 mutation contribute to PAH penetrance more specifically in women. Conversely, females have better right ventricular function, translating to a better prognosis. Along with right ventricular adaptation, women tend to respond to PAH treatment differently from men. As some young women suffer from PAH, contraception is of particular importance, considering that pregnancy in patients with PAH is strongly discouraged due to high risk of death. When contraception measures fail, pregnant women need a multidisciplinary team-based approach. This article aims to review epidemiology, mechanisms underlying the higher female predominance, but better prognosis and the intricacies in management of women affected by PAH.

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“…Due to the female predominance in many forms of pulmonary arterial hypertension [17], additional comparisons were implemented between female and male patients, as well as pre-vs. post-iEPO effects for female patients.…”

Section: Data Collectionmentioning

confidence: 99%

The Clinical Impact of Flow Titration on Epoprostenol Delivery via High Flow Nasal Cannula for ICU Patients with Pulmonary Hypertension or Right Ventricular Dysfunction: A Retrospective Cohort Comparison Study

Gurnani

Roberts

et al. 2020

JCM

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1) Background: inhaled epoprostenol (iEPO) delivered via high-flow nasal cannula (HFNC) has been reported to be effective for pulmonary hypertension and right ventricular dysfunction. In vitro studies have identified HFNC gas flow as a key factor in trans-nasal aerosol delivery efficiency; however, little evidence is available on the clinical impact of flow titration on trans-nasal aerosol delivery. At our institution, iEPO via HFNC was initiated in 2015 and the concept of flow titration during iEPO via HFNC has been gradually accepted and carried out by clinicians in the recent years.(2) Methods: a retrospective review of the electronic medical records for all adult patients who received iEPO via HFNC in a tertiary teaching hospital. Pre-and post-iEPO responses were reported for patients whose HFNC flow was titrated or maintained constant during iEPO delivery. Positive response to iEPO was defined as the reduction of mean pulmonary arterial pressure (mPAP) > 10% for pulmonary hypertension patients or the improvement of oxygenation [pulse oximetry (SpO 2 )/fraction of inhaled oxygen (F I O 2 )] > 20%. The number of responders to iEPO was compared between groups with titrated vs constant flow. (3) Results: 51 patients who used iEPO to treat pulmonary hypertension and/or right ventricular dysfunction were reviewed. Following iEPO administration via HFNC, mPAP decreased (43.6 ± 11.7 vs. 36.3 ± 9.7 mmHg, p < 0.001). Among the 51 patients, 24 had concomitant refractory hypoxemia, their oxygenation (SpO 2 /F I O 2 ) improved after iEPO delivery (127.8 ± 45.7 vs. 157.6 ± 62.2, p < 0.001). During iEPO initiation, gas flow was titrated in 25 patients and the remaining 26 patients used constant flow. The percentage of patients in the flow titration group who met the criteria for a positive response was higher compared to the group with constant flow (85.7% vs. 50%, p = 0.035). Pre-vs post-iEPO responses were significant in the flow titration group included improvement in cardiac output (p = 0.050), cardiac index (p = 0.021) and F I O 2 reduction (p = 0.016). These improvements in hemodynamics and F I O 2 were not observed in the constant flow group. (4) Conclusion: in patients with pulmonary hypertension and/or right ventricular dysfunction, trans-nasal iEPO decreased pulmonary arterial pressure. It also improved oxygenation in patients with combined refractory hypoxemia. These improvements were more evident in patients whose gas flow was titrated during iEPO initiation than those patients using constant flow.

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Sex-specific differences and survival in patients with idiopathic pulmonary arterial hypertension 2008–2016

Cited by 21 publications

References 29 publications

Right ventricular function correlates of right atrial strain in pulmonary hypertension: a combined cardiac magnetic resonance and conductance catheter study

Right ventricular function correlates of right atrial strain in pulmonary hypertension: a combined cardiac magnetic resonance and conductance catheter study

Sex and gender in pulmonary arterial hypertension

The Clinical Impact of Flow Titration on Epoprostenol Delivery via High Flow Nasal Cannula for ICU Patients with Pulmonary Hypertension or Right Ventricular Dysfunction: A Retrospective Cohort Comparison Study

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