scholarly journals Pregnancy in pulmonary arterial hypertension

2016 ◽  
Vol 25 (142) ◽  
pp. 431-437 ◽  
Author(s):  
Karen M. Olsson ◽  
Richard Channick
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Post Partum ◽  
Early Termination ◽  
European Respiratory Society ◽  
Advanced Therapies ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology ◽  
In Pregnancy

Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH) remains high in pregnancy and is especially high during the post-partum period. However, recent data indicates that morbidity and mortality during pregnancy and after birth have improved for PAH patients. The current European Society of Cardiology/European Respiratory Society guidelines recommend that women with PAH should not become pregnant. Therefore, the risks associated with pregnancy must be emphasised and counselling offered to women at the time of PAH diagnosis and to women with PAH who become pregnant. Early termination should be discussed. Women who choose to continue with their pregnancy should be treated at specialised pulmonary hypertension centres with experience in managing PAH during and after pregnancy.

scholarly journals Idiopathic Pulmonary Arterial Hypertension Unmasked by Pregnancy

2020 ◽  
Vol 19 (4) ◽  
pp. 240-243
Author(s):  
Adam Maxwell ◽  
◽  
Thomas Holman ◽  
Timea Novak ◽  
◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Post Partum ◽  
Right Heart Failure ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Shortness Of Breath ◽  
Acute Medical Unit ◽  
Pulmonary Angiogram ◽  
Pulmonary Arterial

A 31-year old woman presented to the acute medical unit 9 days post-partum with shortness of breath and peripheral oedema. Initially suspected to have either a pulmonary embolism or post-partum cardiomyopathy, she proceeded to have imaging including a CT Pulmonary angiogram and echocardiogram, which were suggestive of pulmonary hypertension and severe right heart failure. Her history and other investigations did not reveal any obvious cause for this. She was transferred to a specialist centre where she was diagnosed with Idiopathic Pulmonary Arterial Hypertension (IPAH), previously known as primary pulmonary hypertension. Shortness of breath during pregnancy and in the postpartum period is a relatively common acute medical presentation. Whilst IPAH is a rare diagnosis, it carries a high mortality rate, particularly in pregnancy, and requires prompt specialist investigation, diagnosis and management.

scholarly journals Dart to the target: an alternative bull’s eye parametric display for European Society of Cardiology / European Respiratory Society goal-orientated risk reduction strategy in pulmonary arterial hypertension

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401878052
Author(s):  
Cihangir Kaymaz ◽  
Ozgur Yasar Akbal ◽  
Aykun Hakgor ◽  
Hacer Ceren Tokgoz ◽  
Seda Tanyeri
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Risk Reduction ◽  
European Society ◽  
European Respiratory Society ◽  
Reduction Strategy ◽  
Risk Reduction Strategy ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology ◽  
Bull's Eye

Despite the significant mortality and mobidity benefits being obtained with the targeted therapies in patients with pulmonary arterial hypertension (PAH), mid- to long-term survival of patients with this disease has remained unsatisfactory. For earlier and reliable risk stratification in PAH and tailoring the dynamic management strategies, various risk assessment models have been developed. Currently available risk reduction strategy recommended by the European Society of Cardiology (ESC)/European Respiratory Society (ERS) 2015 Pulmonary Hypertension Guidelines has been utilized in three recent registries. In this review, we evaluated the risk prediction models and management algorithms in this setting and propose an alternative parametric display, a bull’s eye, dart table scheme for ESC/ERS goal-orientated risk reduction strategy in patients with PAH.

scholarly journals Advanced risk stratification of intermediate risk group in pulmonary arterial hypertension

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402096173
Author(s):  
Athiththan Yogeswaran ◽  
Manuel J. Richter ◽  
Natascha Sommer ◽  
Hossein A. Ghofrani ◽  
Werner Seeger ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pressure Ratio ◽  
Systolic Pulmonary Artery Pressure ◽  
Intermediate Risk ◽  
European Respiratory Society ◽  
Treatment Naïve ◽  
Risk Patients ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

In 301 treatment-naïve patients with pulmonary arterial hypertension stratified by the European Society of Cardiology/European Respiratory Society risk score, further stratification of intermediate-risk patients based on six-minute walk distance and the tricuspid annular plane systolic excursion/systolic pulmonary artery pressure ratio identified a subset with mortality rates comparable to low-risk patients.

scholarly journals Strategy of medical treatment of pulmonary arterial hypertension in the current international recommendations

2016 ◽  
Vol 13 (2) ◽  
pp. 46-64
Author(s):  
T V Martyniuk ◽  
I E Chazova
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Optimal Choice ◽  
New Drugs ◽  
Reverse Remodeling ◽  
Significant Progress ◽  
European Respiratory Society ◽  
Specific Therapy ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

Significant progress in the treatment of pulmonary arterial hypertension (PAH), in recent years, is associated with the introduction into clinical practice of a number of drugs pathogenetic action, can cause vasodilation and reverse remodeling of pulmonary vessels. Recently PAH-specific therapy was replenished with new drugs. This review is created as a result of the analysis of modern American recommendations CHEST and recommendations of the European society of cardiology and the European respiratory society (ESC/ERS) in order to provide all professionals involved in the maintenance of the PAH, data on the main approaches to the pharmacotherapy and the optimal choice of pharmacological treatment methods.

Pulmonary arterial hypertension in systemic sclerosis: Diagnosis and treatment according to the European Society of Cardiology and European Respiratory Society 2015 guidelines

2018 ◽  
Vol 4 (1) ◽  
pp. 35-42 ◽  
Author(s):  
Nicola Giordano ◽  
Claudio Corallo ◽  
Chiara Chirico ◽  
Angelica Brazzi ◽  
Adriana Marinetti ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Endothelial Dysfunction ◽  
Arterial Hypertension ◽  
Correct Diagnosis ◽  
European Respiratory Society ◽  
Long Term Outcome ◽  
Screening Programs ◽  
Pulmonary Arterial

Scleroderma (systemic sclerosis) is an autoimmune connective tissue disease which presents endothelial dysfunction and fibroblast dysregulation, resulting in vascular and fibrotic disorders. Pulmonary hypertension is frequent in patients with systemic sclerosis: the natural evolution of the disease can induce the development of different forms of pulmonary hypertension, representing one of the main causes of death. Among the different forms of pulmonary hypertension in systemic sclerosis, pulmonary arterial hypertension is the most frequent one (rate of occurrence is estimated between 7% and 12%). This pulmonary vascular complication should be treated with a combination of drugs that is able to counteract endothelial dysfunction, antagonizing the endothelin-1 system and replacing prostaglandin I2 and nitric oxide activity. A correct diagnosis is mandatory, because it is possible only for pulmonary arterial hypertension to use specific drugs that are able to control the symptomatic condition and the evolution of the disease. According to the most recent guidelines, for the patients with systemic sclerosis, also without pulmonary hypertension symptoms, echocardiography screening for the detection of pulmonary hypertension is recommended. Pulmonary arterial hypertension screening programs in systemic sclerosis patients is able to identify milder forms of the disease, allowing earlier management and better long-term outcome.

scholarly journals Advanced pulmonary arterial hypertension: mechanical support and lung transplantation

2017 ◽  
Vol 26 (146) ◽  
pp. 170089 ◽  
Author(s):  
Sonja Bartolome ◽  
Marius M. Hoeper ◽  
Walter Klepetko
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Therapeutic Option ◽  
Survival Rates ◽  
Mechanical Support ◽  
European Respiratory Society ◽  
Term Survival ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

The development of targeted therapies has transformed the outlook for patients with pulmonary arterial hypertension (PAH); however, some patients fail to achieve an adequate clinical response despite receiving maximal treatment. For these patients, lung transplantation remains an important therapeutic option, and recommendations for transplantation are included in the current European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension. Although lung transplantation is not without risk, overall long-term survival rates are good and substantial improvements in quality of life have been reported for lung transplant recipients. In this review, we describe the important considerations prior to, during and after transplantation, including the role of mechanical support, in patients with advanced PAH.

scholarly journals EXPRESS: Pulmonary arterial hypertension in pregnancy – a systematic review of outcomes in the modern era

2021 ◽  
pp. 204589402110136
Author(s):  
Ting Ting Low ◽  
Nita Guron ◽  
Robin Ducas ◽  
Kenichiro Yamamura ◽  
Pradeepkumar Charla ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Mortality Rate ◽  
Arterial Hypertension ◽  
Maternal Mortality ◽  
Post Partum ◽  
Systolic Pressure ◽  
Pulmonary Artery Systolic Pressure ◽  
Maternal Mortality Rate ◽  
Pulmonary Arterial ◽  
In Pregnancy

Background: Pregnancy is hazardous with pulmonary arterial hypertension (PAH), but the risks may have improved in recent years. We sought to systematically evaluate PAH and pregnancy-related outcomes in the last decade. Methods: We searched for articles describing outcomes in pregnancy cohorts published between 2008-2018. 3658 titles were screened and 13 studies included for analysis. Pooled incidences and percentages of maternal and perinatal outcomes were calculated.  Results: Out of 272 pregnancies, 214 pregnancies advanced beyond 20 gestational weeks. The mean maternal age was 28±2 years, mean pulmonary artery systolic pressure on echocardiogram was 76±19mmHg. Aetiologies include idiopathic PAH 22%, congenital heart disease 64%, and others 15%. Majority (74%) had good functional class I/II. Only 48% of women received PAH-specific therapy. Premature deliveries occur in 58% of pregnancies at mean of 34±1 weeks, most (76%) had caesarean section. Maternal mortality rate was 12% overall (n=26); even higher for idiopathic PAH aetiology alone (20%). Reported causes of death included right heart failure, cardiac arrest, PAH crises, pre-eclampsia and sepsis. 61% of maternal deaths occur at 0-4 days post-partum. Stillbirths rate was 3% and neonatal mortality rate 1%. Conclusions: PAH in pregnancy continues to be perilous with high maternal mortality rate. Continued prospective studies are needed.

scholarly journals Heritable pulmonary hypertension: from bench to bedside

2017 ◽  
Vol 26 (145) ◽  
pp. 170037 ◽  
Author(s):  
Barbara Girerd ◽  
Jason Weatherald ◽  
David Montani ◽  
Marc Humbert
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Genetic Diagnosis ◽  
Predictive Testing ◽  
Incomplete Penetrance ◽  
European Respiratory Society ◽  
Dominant Disease ◽  
Pulmonary Arterial ◽  
Heritable Pulmonary Arterial Hypertension

Mutations in the BMPR2 gene, and more rarely in ACVRL1, endoglin, caveolin-1, KCNK3 and TBX4 genes predispose to heritable pulmonary arterial hypertension, an autosomal dominant disease with incomplete penetrance. Bi-allelic mutations in the EIF2AK4 gene predispose to heritable pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis, an autosomal recessive disease with an unknown penetrance.In France, the national pulmonary hypertension referral centre offers genetic counselling and testing to adults and children. Predictive testing is also proposed to adult relatives at risk of carrying a predisposing mutation. In that context, we offer all asymptomatic BMPR2 mutation carriers a programme to detect pulmonary arterial hypertension at an early phase, as recommended by the 2015 European Society Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines. Finally, pre-implantation genetic diagnosis has been conducted on five embryos from two couples in which the fathers were carriers of a pathogenic BMPR2 mutation.

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