Contraceptive Options for the Patient with Pulmonary Arterial Hypertension

2011 ◽  
Vol 10 (3) ◽  
pp. 167-172 ◽  
Author(s):  
Patricia Santiago-Munoz
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Maternal Mortality ◽  
Pregnancy Outcomes ◽  
High Rate ◽  
World Health ◽  
Main Concern ◽  
Care Providers ◽  
Unintended Pregnancies ◽  
Pulmonary Arterial

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.

scholarly journals EXPRESS: Genetic basis of pulmonary arterial hypertension: A prospective study from a highly inbred population

2021 ◽  
pp. 204589402110320
Author(s):  
Abdullah Aldalaan ◽  
Khushnooda Ramzan ◽  
Sarfraz Saleemi ◽  
Ihab Weheba ◽  
Laila Alquait ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Genetic Basis ◽  
Genetic Diagnosis ◽  
Saudi Arabian ◽  
High Rate ◽  
Risk Genes ◽  
Entire Cohort ◽  
Pulmonary Arterial ◽  
A Prospective Study

Pulmonary arterial hypertension (PAH), whether idiopathic PAH (IPAH), heritable PAH, or associated with other conditions, is a rare and potentially lethal disease characterized by progressive vascular changes. To date, there is limited data on the genetic basis of PAH in the Arab region, and none from Saudi Arabian patients. This study aims to identify genetic variations and to evaluate the frequency of risk genes associated to PAH, in Saudi Arabian patients. Adult PAH patients, diagnosed with IPAH and pulmonary veno-occlusive disease (PVOD), of Saudi Arabian origin, were enrolled in this study. Forty-eight patients were subjected to whole exome sequencing (WES), with screening of 26 genes suggested to be associated with the disease. The median age at diagnosis was 29.5 years of age, with females accounting for 89.5% of our cohort population. Overall, we identified variations in 9 genes previously associated with PAH, in 16 patients. Fourteen of these variants have not been described before. Plausible deleterious variants in risk genes were identified in 33.3% (n=16/48) of our entire cohort and 25% of these cases carried variants in BMPR2 (n=4/16). Our results highlight the genetic etiology of PAH in Saudi Arabia patients and provides new insights for the genetic diagnosis of familial and IPAH, as well as for the identification of the biological pathways of the disease. This will enable the development of new target therapeutic strategies, for a disease with a high rate of morbidity and mortality.

Reliability and responsiveness of incremental shuttle walk test to estimate exercise capacity in patients with pulmonary arterial hypertension

2020 ◽  
Vol 16 (3) ◽  
pp. 179-185
Author(s):  
R. Ishrat ◽  
A. Mujaddadi ◽  
M.S. Ali ◽  
D. Talwar ◽  
M.E. Hussain
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Intraclass Correlation ◽  
World Health ◽  
Minimal Detectable Change ◽  
Walk Test ◽  
Cross Sectional ◽  
Suitable Alternative ◽  
Pulmonary Arterial

The purpose of the present study was to evaluate the reliability and responsiveness of the incremental shuttle walk test (ISWT) to estimate exercise capacity in patients with pulmonary arterial hypertension (PAH). This was a cross-sectional longitudinal study performed on stable PAH patients (n=30, mean age ± standard deviation, 60±13.6 years) with World Health Organization functional class II & III. Reliability was assessed by comparing the distance covered between ISWT-1 (initial) and ISWT-2 (retest). Responsiveness was determined by comparing the distance covered in the ISWT-3 post pulmonary rehabilitation (PR) to the ISWT-1. The distance covered between ISWT-1 (177±87.85 m) and ISWT-2 (191.67±96.39 m) was not statistically significant. The result of the relative reliability has shown to be excellent with an intraclass correlation coefficient (ICC2,1 = 0.98, 95%CI = 0.95-0.99). Absolute reliability was evaluated through the standard error of the measurement and minimal detectable change at a 95% confidence interval (MDC95) were 12.29 and 33.9 m, respectively. Bland Altman plot showed good agreement between the two ISWTs. Following PR, the effect size (ES=0.78) and standardised response mean (SRM=1.50) were moderate and large respectively. ISWT is considered to be a reliable and responsive measure to estimate exercise capacity in patients with PAH. The ISWT may be considered a suitable alternative tool over a 6-min walk test and in the absence of equipment availability or expertise for conducting cardiopulmonary exercise test for the assessment of exercise capacity in these patients.

Recent Updates in Both the Diagnosis and Treatment Options that Impact the Clinical Treatment of Pulmonary Arterial Hypertension

2010 ◽  
Vol 2 ◽  
pp. CMT.S4192
Author(s):  
Charles D. Burger
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Plan ◽  
Treatment Options ◽  
Pharmaceutical Formulations ◽  
World Health ◽  
New Agents ◽  
Pulmonary Arterial ◽  
New Treatment ◽  
Health Organization

The evaluation and management of pulmonary arterial hypertension (PAH) is a rapidly evolving area of subspecialty medicine requiring regular clinical updates. Most notably are changes in the World Health Organization diagnostic scheme whereby the clinician categorizes the correct type of pulmonary hypertension in order direct the most specific evaluation and treatment plan. In addition, there have been several changes in both the FDA-approved pharmaceutical formulations and new agents for the treatment of PAH. This review will provide an update in these areas and more importantly, guidance to the clinician on the most appropriate utilization of these new treatment options.

scholarly journals Oral treprostinil in transition or as add-on therapy in pediatric pulmonary arterial hypertension

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401985647 ◽  
Author(s):  
D. Dunbar Ivy ◽  
Jeffrey A. Feinstein ◽  
Delphine Yung ◽  
Mary P. Mullen ◽  
Edward C. Kirkpatrick ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Cardiopulmonary Exercise Testing ◽  
Functional Class ◽  
World Health ◽  
Walk Distance ◽  
Pulmonary Arterial ◽  
Health Organization

Treprostinil, a prostacyclin analogue, is approved for the treatment of pulmonary arterial hypertension (PAH) in adults. Transition from parenteral to oral treprostinil has been successfully accomplished in adults with PAH but not in children. In this multicenter study, pediatric patients treated with parenteral (Cohort 1) or inhaled (Cohort 2) treprostinil were transitioned to oral treprostinil. Prostacyclin-naïve individuals on background oral PAH therapy received oral treprostinil as add-on therapy (Cohort 3). Successful transition was oral treprostinil dose maintenance through week 24. Patients were monitored for adverse events (AEs), 6-min walk distance (6MWD), PAH symptoms, World Health Organization (WHO) Functional Class (FC), cardiac magnetic resonance imaging (cMRI), cardiopulmonary exercise testing (CPET), and quality of life through 24 weeks. A total of 32 patients were enrolled in the study; 23 (72%) were girls (mean age = 12.2 years). All patients were on background oral PAH therapy. Overall, patients (96.9%) maintained transition to oral treprostinil; one patient (Cohort 1) transitioned to oral treprostinil, then back to parenteral after experiencing syncope and WHO FC change from II to III. Cohorts 1, 2, and 3 received a final mean oral treprostinil dose of 5.6, 3.3, and 4.5 mg t.i.d., respectively. All cohorts had variable changes in 6MWD, cMRI, and CPET. Overall, 12 serious AEs were reported. All patients had drug-related AEs including headache (81%), diarrhea (69%), nausea (66%), vomiting (66%), and flushing (56%). Pediatric patients maintained transition to oral treprostinil with preservation of exercise capacity and WHO FC. Prostanoid-related AEs were most common and similar to those reported in adults.

scholarly journals Safety and efficacy of transitioning from the combination of bosentan and sildenafil to alternative therapy in patients with pulmonary arterial hypertension

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402094552
Author(s):  
Nathan J. Verlinden ◽  
Raymond L. Benza ◽  
Amresh Raina
Keyword(s):  
Drug Interaction ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Alternative Therapy ◽  
Functional Class ◽  
World Health ◽  
Risk Scores ◽  
Safety And Efficacy ◽  
Significant Drug Interaction ◽  
Pulmonary Arterial

The combination of bosentan and sildenafil is commonly used to treat patients with pulmonary arterial hypertension (PAH); however, there is evidence of a significant drug interaction between these two medications. We sought to evaluate the safety and efficacy of transitioning patients with PAH from the combination of bosentan and sildenafil to alternative therapy. A retrospective database review was performed on 16 patients with PAH who were treated with the combination of bosentan and sildenafil and transitioned to alternative treatment at our center. Invasive and non-invasive patient parameters were collected at baseline and after transition. 56.3% of patients were in World Health Organization functional class (WHO FC) III and a majority of patients (68.7%) were on background prostacyclin therapy. The most common reason for transition was concern for a drug interaction in seven patients (43.8%). The most common transition was bosentan to macitentan in eight patients (50%). Fifteen patients (93.8%) tolerated the transition after a median follow-up of 6.5 months with minor adverse events occurring in four patients (25%). In 11 patients, 6-min walk distance (6MWD) was unchanged comparing baseline to post transition measurements with a median change of +8 m (range: −50 to + 70; P = 0.39). Nine patients (81.8%) had stable (within 15% margin) or significant improvement (increase by ≥15%) in 6MWD after transition. All patients demonstrated stable or improved WHO FC after transition. There were no significant changes after transition in hemodynamics, N-terminal pro-brain natriuretic peptide (NT-proBNP) values, or Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk scores. In our study, transitioning patients from bosentan and sildenafil to alternative therapy was safe and resulted in clinical stability.

Single-center prognostic validation of the risk assessment of the 2015 ESC/ERS guidelines in patients with pulmonary arterial hypertension in Japan

2020 ◽  
Vol 98 (9) ◽  
pp. 653-658 ◽  
Author(s):  
Ryo Imai ◽  
Shiro Adachi ◽  
Masahiro Yoshida ◽  
Shigetake Shimokata ◽  
Yoshihisa Nakano ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Risk Group ◽  
Low Risk ◽  
World Health ◽  
Right Atrial ◽  
Walking Distance ◽  
Pulmonary Arterial

The 2015 European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension include a multidimensional risk assessment for patients with pulmonary arterial hypertension (PAH). However, prognostic validations of this risk assessment are limited, especially outside Europe. Here, we validated the risk assessment strategy in PAH patients in our institution in Japan. Eighty consecutive PAH patients who underwent right heart catheterization between November 2006 and December 2018 were analyzed. Patients were classified as low, intermediate, or high risk by using a simplified version of the risk assessment that included seven variables: World Health Organization functional class, 6-min walking distance, peak oxygen consumption, brain natriuretic peptide, right atrial pressure, mixed venous oxygen saturation, and cardiac index. The high-risk group showed significantly higher mortality than the low- or intermediate-risk group at baseline (P < 0.001 for both comparisons), and the mortalities in the intermediate- and low-risk groups were both low (P = 0.989). At follow-up, patients who improved to or maintained a low-risk status showed better survival than those who did not (P = 0.041). Our data suggest that this risk assessment can predict higher mortality risk and long-term survival in PAH patients in Japan.

scholarly journals Pulmonary arterial hypertension associated with protein kinase inhibitors: a pharmacovigilance–pharmacodynamic study

2019 ◽  
Vol 53 (5) ◽  
pp. 1802472 ◽  
Author(s):  
Lucie Cornet ◽  
Charles Khouri ◽  
Matthieu Roustit ◽  
Christophe Guignabert ◽  
Marie-Camille Chaumais ◽  
...  
Keyword(s):  
Cluster Analysis ◽  
Pulmonary Arterial Hypertension ◽  
Protein Kinase ◽  
Arterial Hypertension ◽  
Kinase Inhibitors ◽  
Protein Kinase Inhibitors ◽  
World Health ◽  
Protein Kinase Family ◽  
Kinase Family ◽  
Pulmonary Arterial

The pathophysiology of pulmonary arterial hypertension (PAH) induced by protein kinase inhibitors (PKIs) remains unclear. To gain knowledge into this rare and severe pathology we performed a study combining a pharmacovigilance approach and the pharmacodynamic properties of PKIs.A disproportionality analysis on the World Health Organization pharmacovigilance database VigiBase using the reporting odds ratio (ROR) and 95% confidence interval was first performed. Then, we identified the most relevant cellular targets of interest through a systematic literature review and correlated the pharmacovigilance signals with the affinity for the different PKIs. We further performed a hierarchical cluster analysis to assess patterns of binding affinity.A positive disproportionality signal was found for dasatinib, bosutinib, ponatinib, ruxolitinib and nilotinib. Five non-receptor protein kinases significantly correlate with disproportionality signals: c-Src (r=0.79, p=0.00027), c-Yes (r=0.82, p=0.00015), Lck (r=0.81, p=0.00046) and Lyn (r=0.80, p=0.00036), all belonging to the Src protein kinase family, and TEC (r=0.85, p=0.00006). Kinases of the bone morphogenetic protein signalling pathway also seem to play a role in the pathophysiology of PKI-induced PAH. Interestingly, the dasatinib affinity profile seems to be different from that of other PKIs in the cluster analysis.The study highlights the potential role of the Src protein kinase family and TEC in PAH induced by PKIs. This approach combining pharmacovigilance and pharmacodynamics data allowed us to generate some hypotheses about the pathophysiology of the disease; however, the results have to be confirmed by further studies.

scholarly journals Assessment of the REPLACE study composite endpoint in riociguat-treated patients in the PATENT study

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402097312
Author(s):  
Gérald Simonneau ◽  
Hossein-Ardeschir Ghofrani ◽  
Paul A. Corris ◽  
Stephan Rosenkranz ◽  
Ekkehard Grünig ◽  
...  
Keyword(s):  
Relative Risk ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Clinical Improvement ◽  
Composite Endpoint ◽  
World Health ◽  
Walking Distance ◽  
Pulmonary Arterial ◽  
Clinical Worsening

The goal of treatment in patients with pulmonary arterial hypertension is to achieve a low risk status, indicating a favorable long-term outcome. The REPLACE study investigated the efficacy of switching to riociguat in patients with pulmonary arterial hypertension and an insufficient response to phosphodiesterase-5 inhibitors. In this post hoc analysis, we applied the REPLACE composite endpoint of clinical improvement to the placebo-controlled PATENT-1 study of riociguat in pulmonary arterial hypertension and its long-term extension, PATENT-2. Clinical improvement was defined as ≥2 of the following in patients who completed the study without clinical worsening: ≥10% or ≥30 m improvement in 6-minute walking distance; World Health Organization functional class I or II; ≥30% decrease in N-terminal prohormone of brain natriuretic peptide. At PATENT-1 Week 12, patients treated with riociguat were more likely to achieve the composite endpoint vs. placebo ( P < 0.0001), with similar results in pretreated ( P = 0.0189) and treatment-naïve ( P < 0.0001) patients. Achievement of the composite endpoint at Week 12 was associated with a 45% reduction in relative risk of death and a 19% reduction in relative risk of clinical worsening in PATENT-2. Overall, these data suggest that use of the REPLACE composite endpoint in patients with pulmonary arterial hypertension is a valid assessment of response to treatment.

scholarly journals Epidemiology and initial management of pulmonary arterial hypertension: real-world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE)

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401987715 ◽  
Author(s):  
Alexandra Arvanitaki ◽  
Maria Boutsikou ◽  
Anastasia Anthi ◽  
Sotiria Apostolopoulou ◽  
Aikaterini Avgeropoulou ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
World Health ◽  
Real World Data ◽  
Treatment Data ◽  
Risk Patients ◽  
Pulmonary Arterial ◽  
Health Organization

Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.

scholarly journals The Modified Borg Dyspnea Scale does not predict hospitalization in pulmonary arterial hypertension

2017 ◽  
Vol 7 (2) ◽  
pp. 384-390 ◽  
Author(s):  
Debasree Banerjee ◽  
Jane Kamuren ◽  
Grayson L. Baird ◽  
Amy Palmisciano ◽  
Ipsita Krishnan ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
World Health ◽  
Pulmonary Vascular Disease ◽  
Common Symptom ◽  
Class Iii ◽  
Patient Reported ◽  
Pulmonary Arterial ◽  
Minute Walk

Background Breathlessness is the most common symptom reported by patients with pulmonary arterial hypertension (PAH). The Modified Borg Dyspnea Scale (MBS) is routinely obtained during the six-minute walk test in the assessment of PAH patients, but it is not known whether the MBS predicts clinical outcomes such as hospitalizations in PAH. Methods We performed a retrospective study of World Health Organization (WHO) Group 1 PAH patients followed at our center. The dates of the first three MBS and hospitalizations that occurred within three months of a documented MBS were collected. Marginal Cox hazard regression modeling was used to assess for a relationship between MBS and all-cause as well as PAH-related hospitalization. Results A total of 50 patients were included; most (92%) were functional class III/IV, 44% and 65% were treatment-naïve prior to their first MBS and hospitalization, respectively. The first recorded MBS was inversely correlated with the first recorded six-minute walk distance (6MWD) (r = –0.41, P < 0.01) but did not track with WHO functional class (r = 0.07, P = 0.63). MBS did not predict all-cause (hazard ratio [HR], 0.91; 95% confidence interval [CI], 0.76–1.08; P = 0.28) or PAH-related hospitalization (HR, 1.04; 95% CI, 0.89–1.23; P = 0.61), though there was a strong relationship between 6MWD and PAH-related hospitalization ( P = 0.01). These findings persisted after multivariable adjustment. Conclusions Breathlessness as assessed by MBS does not predict all-cause or PAH-related hospitalization. Robust and validated patient-reported outcomes are needed in pulmonary vascular disease.

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