scholarly journals Sex Differences, Estrogen Metabolism and Signaling in the Development of Pulmonary Arterial Hypertension

2021 ◽  
Vol 8 ◽  
Author(s):  
Yanan Sun ◽  
Shreya Sangam ◽  
Qiang Guo ◽  
Jian Wang ◽  
Haiyang Tang ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Animal Models ◽  
Arterial Hypertension ◽  
Heart Function ◽  
Female Rats ◽  
Estrogen Signaling ◽  
Estrogen Metabolism ◽  
Right Heart ◽  
Right Heart Function ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a complex and devastating disease with a poor long-term prognosis. While women are at increased risk for developing PAH, they exhibit superior right heart function and higher survival rates than men. Susceptibility to disease risk in PAH has been attributed, in part, to estrogen signaling. In contrast to potential pathological influences of estrogen in patients, studies of animal models reveal estrogen demonstrates protective effects in PAH. Consistent with this latter observation, an ovariectomy in female rats appears to aggravate the condition. This discrepancy between observations from patients and animal models is often called the “estrogen paradox.” Further, the tissue-specific interactions between estrogen, its metabolites and receptors in PAH and right heart function remain complex; nonetheless, these relationships are essential to characterize to better understand PAH pathophysiology and to potentially develop novel therapeutic and curative targets. In this review, we explore estrogen-mediated mechanisms that may further explain this paradox by summarizing published literature related to: (1) the synthesis and catabolism of estrogen; (2) activity and functions of the various estrogen receptors; (3) the multiple modalities of estrogen signaling in cells; and (4) the role of estrogen and its diverse metabolites on the susceptibility to, and progression of, PAH as well as their impact on right heart function.

scholarly journals Noninvasive Assessment of Right Heart Function and Hemodynamics During Exercise in Patients With Pulmonary Arterial Hypertension

2013 ◽  
Vol 106 (2) ◽  
pp. 141-146 ◽  
Author(s):  
Michelle L. Freeman ◽  
Carolyn Landolfo ◽  
Robert E. Safford ◽  
Cesar A. Keller ◽  
Michael G. Heckman ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Heart Function ◽  
Right Heart ◽  
Right Heart Function ◽  
Pulmonary Arterial ◽  
Noninvasive Assessment

scholarly journals Right heart function during simulated altitude in patients with pulmonary arterial hypertension

Open Heart ◽  
2017 ◽  
Vol 4 (1) ◽  
pp. e000532 ◽  
Author(s):  
Leigh M Seccombe ◽  
Vincent Chow ◽  
Wei Zhao ◽  
Edmund M T Lau ◽  
Peter G Rogers ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Heart Function ◽  
Simulated Altitude ◽  
Right Heart ◽  
Right Heart Function ◽  
Pulmonary Arterial

scholarly journals Favorable Pregnancy Outcomes in Women With Well-Controlled Pulmonary Arterial Hypertension

2021 ◽  
Vol 8 ◽  
Author(s):  
Nadine Corbach ◽  
Charlotte Berlier ◽  
Mona Lichtblau ◽  
Esther I. Schwarz ◽  
Fiorenza Gautschi ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pregnancy Outcomes ◽  
Multidisciplinary Team ◽  
Heart Function ◽  
Case Series ◽  
Functional Class ◽  
Tertiary Referral Center ◽  
Right Heart Function ◽  
Pulmonary Arterial

Introduction: Since pregnancy in women with pulmonary arterial hypertension (PAH) is associated with a high risk of morbidity and mortality, it is recommended that pregnancy should be avoided in PAH. However, some women with mild PAH may consider this recommendation as unsuitable. Unfortunately knowledge on pregnancy outcomes and best management of PAH during pregnancy is limited.Methods: Data from all women with PAH who were followed during pregnancy by a multidisciplinary team at a tertiary referral center for PAH and who delivered between 2004 and 2020 were retrospectively analyzed in a case series. PAH risk factor profiles including WHO functional class (WHO-FC), NT-pro-BNP, echocardiographic pulmonary arterial pressure (PAP) and right heart function were analyzed prior to, during and following pregnancy.Results: In seven pregnancies of five women with PAH (median age 29 (27; 31) years), there were no abortions or terminations. Five pregnancies were planned (all in WHO-FC I-II), two incidental (WHO-FC II, III). During pregnancy none of the women had complications or clinical worsening of PAH. After a median pregnancy duration of 37 1/7 weeks all gave birth to healthy babies by cesarean section in spinal anesthesia. During pregnancy, PAP tended to increase, whilst the course of WHO-FC and NT-pro-BNP were variable and no trend could be detected.Conclusion: Women with PAH with a low risk profile closely followed by a multidisciplinary team had a favorable course during and after pregnancy, resulting in successful deliveries of healthy newborns.

scholarly journals Elevated serum circ_0068481 levels as a potential diagnostic and prognostic indicator in idiopathic pulmonary arterial hypertension

2019 ◽  
Vol 9 (4) ◽  
pp. 204589401988841 ◽  
Author(s):  
Ying Zhang ◽  
Yongbin Chen ◽  
Hua Yao ◽  
Zhenbang Lie ◽  
Guo Chen ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Heart Function ◽  
Univariate Analysis ◽  
Right Heart Failure ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Circular Rnas ◽  
Right Heart ◽  
Pulmonary Arterial

Circular RNAs have continuous, stable, and covalently closed circular structures and are not easily degraded by nucleases, thus they are ideal serum biomarkers for detecting diseases. However, research is still lacking on circular RNAs as diagnostic and prognostic markers for idiopathic pulmonary arterial hypertension. This study investigated the potential role of serum circ_0068481 levels in idiopathic pulmonary arterial hypertension diagnosis and prognosis. This prospective cohort study enrolled 82 patients with idiopathic pulmonary arterial hypertension between January 2016 and July 2018 at Guangdong Provincial People’s Hospital. Serum circ_0068481 levels were measured using quantitative reverse transcription-polymerase chain reaction. Baseline data, including clinical background, hemodynamic variables, and biochemical variables, were collected. Receiver operating characteristic curves were used to investigate diagnostic effect, the Kaplan–Meier method was used to estimate survival rates, and univariate analysis of prognostic factors was performed with a Cox proportional hazard model. We found that serum circ_0068481 expression levels were significantly higher in patients with idiopathic pulmonary arterial hypertension and had higher sensitivity and specificity for predicting idiopathic pulmonary arterial hypertension. Additionally, we found that circ_0068481 expression correlated significantly with heart function, 6-min walk distance, serum N-terminal pro-B-type natriuretic peptide, serum H2S, the 6th World Symposium on Pulmonary Hypertension risk stratification, right heart failure, and patient death. Moreover, serum circ_0068481 levels were elevated in patients with idiopathic pulmonary arterial hypertension and right heart failure and were able to predict right heart failure. Serum circ_0068481 levels were also elevated in patients who died with idiopathic pulmonary arterial hypertension and were able to predict poorer clinical outcomes. Circ_0068481 is a novel and noninvasive biomarker for diagnosing idiopathic pulmonary arterial hypertension and predicting poor clinical outcome in patients with idiopathic pulmonary arterial hypertension.

scholarly journals Reversible Pulmonary Arterial Hypertension Induced by Dasatinib in a Patient With Chronic Myeloid Leukemia

2017 ◽  
Vol 33 (4) ◽  
pp. 284-289 ◽  
Author(s):  
Atai Watanabe ◽  
Kazuaki Yokoyama ◽  
Nobuhiro Ohno ◽  
Kaoru Uchimaru ◽  
Naohide Yamashita ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Chronic Myeloid Leukemia ◽  
Arterial Hypertension ◽  
Transthoracic Echocardiography ◽  
Myeloid Leukemia ◽  
Kinase Inhibitor ◽  
Heart Function ◽  
Drug Discontinuation ◽  
Right Heart ◽  
Pulmonary Arterial

A case study is provided of dasatinib-induced pulmonary arterial hypertension (PAH) in a patient with chronic myeloid leukemia. This condition resolved completely within 2 months of drug discontinuation. Transthoracic echocardiography (TTE) data were obtained throughout the recovery process. After 30 months of dasatinib treatment, a woman in her 30s developed orthopnea and signs of right heart failure (leg edema, hepatomegaly, and weight gain). Transthoracic echocardiography indicated elevated mean pulmonary artery pressure, severely impaired systolic and diastolic right ventricular functions, and dilation of the right ventricle and atrium. Once dasatinib was discontinued, clinical symptoms improved rapidly, and follow-up TTE 2 months later showed normal right heart function. Treatment with an alternative tyrosine kinase inhibitor was initiated and has continued without recurrence of PAH. This case suggests that dasatinib, which inhibits a broad spectrum of tyrosine kinases, could cause reversible PAH; therefore, careful cardiopulmonary evaluation by TTE is necessary.

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