scholarly journals Impact of routine screening on detection, severity and outcome of pulmonary arterial hypertension in systemic sclerosis

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
H Fayed ◽  
B E Schreiber ◽  
C P Denton ◽  
J G Coghlan ◽  
Keyword(s):  
Pulmonary Hypertension ◽  
P Value ◽  
Newly Diagnosed ◽  
Referral Centre ◽  
Screening Programs ◽  
Pulmonary Arterial ◽  
The Impact ◽  
Current Screening ◽  
Risk Categories ◽  
Group 1

Abstract Introduction It has been reported that up to 20% of systemic sclerosis (SSc) patients can be asymptomatic at the time of pulmonary arterial hypertension (PAH) diagnosis. The significant prevalence rate, lack of symptoms and high morbidity and mortality from SSc-PAH as well as the potential benefit from early intervention with the more widely available therapeutic options provide a strong rationale for active screening programs. The DETECT algorithm was developed in 2013 from a large prospective and multicentre study in SSc patients with higher risk of PAH. The objective of this study was to examine the impact of a screening program on the early detection of SSc-PAH. We looked at serial patients diagnosed with SSc associated PAH (SSC-PAH) in a large national pulmonary hypertension referral centre. Patients and methods All newly diagnosed adult patients with SSc-PAH prospectively enrolled in a large national pulmonary hypertension referral centre. The current study included newly diagnosed patients between January 2006 and January 2018. Results Three-hundred and five patients were diagnosed with SSc-PAH in our centre between 2006 and 2018. Of these, 164 patients were diagnosed before 2013 (January 2006 - December 2012) and 141 after 2013 (January 2013 - January 2018). Demographics were similar at presentation between the two groups. The non-invasive (WHO-FC, 6-MWD and NT-proBNP) and haemodynamic measurements (RAP, CI and SvO2) were used to calculate the ESC guidelines risk score. It was noted that higher proportion of patients in the post-2013 were in the higher risk categories than the pre 213 group (84.4% vs. 78%) but this was not found to be statistically significant (p value 0.356). There was no statistically significant difference in survival between the two groups (Post 2013 group, 1-, 3- year and 5-year survival was 87.9%, 60.4% and 52.1%, respectively and pre-2013 group 1-, 3- year and 5-year survival 89.6%, 65.2% and 49%, respectively) with a log rank p value of 0.869. Applying Cox regression analysis of proportional hazard and adjusting for ESC risk score at baseline, predicted survival was not found to be statistically different between the two groups. The ESC risk category at baseline was a highly significant predictor of survival as expected. Conclusion There remains a strong rationale for active screening for PAH in SSc patients which has a poor prognosis despite advances in therapeutic strategies. However, the current screening programme does not seem to have resulted in significantly earlier detection in this cohort. It would be important to analyse other patient populations in order to understand the impact of screening programmes. The current screening programs limitations may explain why we have not been able to detect more patients in the lower risk categories. Further development of these programs in order to overcome their shortfalls is direly needed. FUNDunding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): National Health ServiceRoyal Free NHS Trust Survival analysis

scholarly journals Sex Dimorphism in Pulmonary Hypertension: The Role of the Sex Chromosomes

Antioxidants ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 779
Author(s):  
Daria S. Kostyunina ◽  
Paul McLoughlin
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Sex Chromosomes ◽  
Bone Morphogenetic Protein 2 ◽  
Sex Dimorphism ◽  
Pulmonary Arterial ◽  
The Impact

Pulmonary hypertension (PH) is a condition characterised by an abnormal elevation of pulmonary artery pressure caused by an increased pulmonary vascular resistance, frequently leading to right ventricular failure and reduced survival. Marked sexual dimorphism is observed in patients with pulmonary arterial hypertension, a form of pulmonary hypertension with a particularly severe clinical course. The incidence in females is 2–4 times greater than in males, although the disease is less severe in females. We review the contribution of the sex chromosomes to this sex dimorphism highlighting the impact of proteins, microRNAs and long non-coding RNAs encoded on the X and Y chromosomes. These genes are centrally involved in the cellular pathways that cause increased pulmonary vascular resistance including the production of reactive oxygen species, altered metabolism, apoptosis, inflammation, vasoconstriction and vascular remodelling. The interaction with genetic mutations on autosomal genes that cause heritable pulmonary arterial hypertension such as bone morphogenetic protein 2 (BMPR2) are examined. The mechanisms that can lead to differences in the expression of genes located on the X chromosomes between females and males are also reviewed. A better understanding of the mechanisms of sex dimorphism in this disease will contribute to the development of more effective therapies for both women and men.

scholarly journals Symptoms, impacts, and suitability of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire in patients with chronic thromboembolic pulmonary hypertension (CTEPH): a qualitative interview study

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Brooke Currie ◽  
Evan Davies ◽  
Amélie Beaudet ◽  
Larissa Stassek ◽  
Leah Kleinman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Shortness Of Breath ◽  
Response Options ◽  
Qualitative Interview Study ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
The Impact

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension caused by blood clots and scar tissue in the blood vessels of the lungs. Health-related quality of life is often significantly impaired in patients with CTEPH. However, a better understanding of how CTEPH symptoms affect patients’ lives is needed to optimally assess the impact of the disease and treatment. Objectives This qualitative study aimed to better understand the symptoms of CTEPH and how they affect patients’ lives, as well as to determine the appropriateness of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire for use in this patient population. Methods Adults diagnosed with CTEPH, recruited from two clinical sites in the US, participated in one-to-one qualitative telephone interviews. They described their experience of CTEPH symptoms and the impact these symptoms have on their lives. They also provided feedback on the comprehensibility and relevance of the PAH-SYMPACT™‘s instructions, items, and response options. Results Participants (N = 12) had a mean age of 62.5 years. Two thirds were female and most (83%) had undergone pulmonary endarterectomy and/or balloon pulmonary angioplasty. The most frequently endorsed symptoms were shortness of breath (endorsed by all 12 participants), fatigue (11 participants), and lightheadedness (10 participants). All participants identified shortness of breath as an “extremely important” symptom, and seven participants rated fatigue as “extremely important.” The most frequent impacts of CTEPH were on ability to walk quickly (endorsed by all 12 participants), ability to walk up inclines or stairs (11 participants), and ability to carry things (11 participants). The PAH-SYMPACT™ items were relevant to most participants and reflected their experience of CTEPH. All participants indicated that no important CTEPH symptoms were missing from the PAH-SYMPACT™. Overall, the instructions, items, and response options of the PAH-SYMPACT™ were clear and easy to understand. Conclusions The symptoms and impacts experienced by patients with CTEPH align with items included in the PAH-SYMPACT™. The PAH-SYMPACT™ appears to be fit for purpose for assessing disease status in patients with CTEPH.

Abstract 16078: Pulmonary Vasculopathy Assessed By Intravascular Ultrasound in Persistent Pulmonary Hypertension After Mitral Valve Replacement, Comparation to Idiopathic Pulmonary Arterial Hypertension and Healthy Controls

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Juan Carlos J Grignola ◽  
Leticia L Fernandez-Lopez ◽  
Enric E Domingo-Ribas ◽  
Rio R Aguilar ◽  
Cristian Humberto C Arredondo ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Elastic Modulus ◽  
Mitral Valve ◽  
Valve Replacement ◽  
Mitral Valve Replacement ◽  
Persistent Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Purpose: The aim of the study is to assess pulmonary vasculopathy (wall fibrosis, pulmonary arterial pulsatility and elastic modulus ) in patients with persistent pulmonary hypertension (pulmonary systolic pressure by ECHO > 50 mmhg ) at least 1 year after mitral valve replacement with normal function of the valve. The evaluation was carried out by intravascular ultrasound (IVUS) in medium sized pulmonary arteries. We compared three groups: Group 1 ( persistent pulmonary hypertension after mitral valve replacement), Group 2 (pulmonary hypertension belonging to the group 1 of the Dana Point classification) and Group 3 (healthy controls). Methods: We studied 43 patients, 15 in Group 1 , 18 Group 2 and 10 in Group 3. Group 1: 13 females, the mean age of this group was 74+-7 years; Group 2: 14 females, 53+-14 years and Group 3: 6 females, 51+-5 years. All patients were submitted to left and right heart catheterization, and IVUS in medium sized elastic PA ( 2-3 mm diameter ) of the inferior lobes. Studied variables were: mean pulmonary artery pressure (PAP, mm Hg), pulmonary wedge pressure, aortic pressure, cardiac output (CO,l/min), pulmonary vascular resistance (PVR, Wood Units), IVUS pulsatility and elastic modulus (EM,mm Hg). Local pulsatility was estimated by IVUS: (systolic- diastolic lumen area/ diastolic lumen area) X 100. PA stiffness was assessed by the elastic modulus (EM= pulse pressure/ IVUSp). Results: In Group 3 all variables were statistically different from the other 2 groups (p<0.01). Variables are shown in table. Conclusions: Group 1, even with a lower mean PAP than Group 2 (p<0.05) showed a similar anatomical ( wall fibrosis ) and similar functional wall remodeling ( EM ).

P4689How is the impact of updated hemodynamic definitions on frequencies of overall pulmonary hypertension and pre-capillary pulmonary hypertension as compared to those with previous criteria

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Tanyeri ◽  
B Keskin ◽  
O Y Akbal ◽  
A Hakgor ◽  
A Karagoz ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Critical Role ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Mean Pressure ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology ◽  
The Impact ◽  
Wedge Pressure

Abstract Background and aim In this study we evaluated the impact of the updated pulmonary hypertension (PH) definitive criteria proposed in 6th World PH Symposium (WSPH) on numbers and frequencies of and pre- versus post-capillary PH as compared to those in European Society of Cardiology (ESC) 2015 PH Guidelines. Methods Study group comprised the retrospectively evaluated 1299 patients (pts) (age 53.1±18.8 years, female 807, 62.1%) who underwent right heart catheterisation (RHC) with different indications between 2006 and 2018. For ESC and WSPH PH definitions, pulmonary arterial mean pressure (PAMP) ≥25 mmHg (definition-A) and PAMP >20 mmHg (definition-B) RHC criteria were used, respectively. For pre-capillary PH definitions, pulmonary artery wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) ≥3 Wood units criteria were included in the both definitions. Results In RHC assessments, PAMP ≥25 mmHg and >20 mmHg were noted in 891 (68.6%) and 1051 (80.9%) of overall pts, respectively. Moreover, pre-capillary PH was diagnosed in 284 (21.8%) and 298 (22.9%) with definition-A and B, respectively. Although updated WSPH definition was associated with a net 12.3% and a relative 18% increase in the overall PH diagnosis, net and relative changes in the frequency of the pre-capillary PH were only 1% and 4.9%. Increase in the overall PH with updated WSPH criterias compared to previous ESC definitions was associated with increase in the number of pre-capillary PH (n=298, 22.9%) but not in the overall frequency of post-capillary PH (688, 52.9%). Because PVR was the product of the transpulmonary gradient (PAMP minus PAWP) divided by cardiac output, this measure was found to keep specificity for distinction between pre- versus post-capillary PH even after lowering thetreshold diagnostic for PAMP from 25 to 20 mmHg. Conclusions Although updated WSPH definition was associated with net 12.3% and relative 18% increase in the overall PH diagnosis, its impact on frequencies of pre- versus post-capillary PH within overall PH population was negligible.These seem to be due to critical role of PVR ensuring specificity in pre-capillary PH diagnosis even after lowering the definitive PAMP treshold to 20 mmHg.

scholarly journals P925 An analysis of the impact of etiology vs. impact of hemodynamic variables on the right ventricular free wall deformation in patients with pulmonary hypertension

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
N Ojrzynska ◽  
E Kowalik ◽  
M Kowalski ◽  
A Klisiewicz ◽  
M Demkow ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Ventricular Free Wall ◽  
P Value ◽  
Free Wall ◽  
Right Ventricular Free Wall ◽  
Mean Pulmonary Arterial Pressure ◽  
Hemodynamic Variables ◽  
Cardiac Catherization ◽  
The Impact

Abstract Purpose Pulmonary hypertension (PH) is a deadly disease leading to right ventricular (RV) failure. The aim of this study was to establish whether aetiology of PH has a greater impact on RV longitudinal deformation than cardiac catherization parameters. Methods We retrospectively analyzed echocardiographic studies and cardiac catherization parameters of 25 patients with pulmonary hypertension : 13 pts with precapillary PH associated with atrial/ventricular septum defect and 12 pts with postcapillary PH due to dilated cardiomyopathy. We measured regional strain and regional systolic and diastolic strain rates (SR) in right ventricular free wall in basal, mid and apical segments. Results Aside from TAPSE, echocardiographic parameters indicate no difference. Even though mean pulmonary arterial pressure and pulmonary vascular residence significantly higher in patients with precapillary PH, strain was significantly decreased in patients with postcapillary PH. Statistically significant differences were observed between pre- and postcapillary PH patients" strain in apical and mid myocardial segments. Early diastolic strain rate (SR) in apical RV segment in precapillary PH patients was significantly higher than in patients with postcapillary PH. In other myocardial segments there were no differences between systolic, early diastolic and late diastolic SR. Conclusion Our study suggests that RV free wall strain in patients with PH moderately correlate with aetiology of PH. We did not find correlation between higher RA pressure or PVR and worsening of RV function in PH of different aetiologies. Precapillary PH Postcapillary PH p value PA mean pressure (mmHG) 75,2 ± 17,7 42,0 ± 7,3 &lt;0,05 PWR (WU) 15,8 ± 6,7 4,7 ± 2,6 &lt;0,05 RV wall thickenss (mm) 11,0 ± 2,5 5,5 ± 1,0 NS RVIT (mm) 42,7 ± 10,4 42,8 ± 8,1 NS TAPSE (mm) 19,2 ± 4,2 15,6 ± 4,9 &lt;0,05 S" 10,8 ± 2,0 9,4 ± 2,1 NS basal segment strain -16 ± 7 -17 ± 5 NS mid segment strain -18 ± 8 -15 ± 4 &lt;0,05 apical segment strain -19 ± 8 -14 ± 4 &lt;0,05

The Impact of Distance to Treatment Center on the Outcome of AML

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4742-4742 ◽  
Author(s):  
Bruno C. Medeiros ◽  
Tamara J. Dunn ◽  
Holbrook E Kohrt ◽  
Steven Coutre ◽  
Jason Gotlib ◽  
...  
Keyword(s):  
Induction Chemotherapy ◽  
Conflicts Of Interest ◽  
Negative Impact ◽  
Adverse Outcomes ◽  
Treatment Center ◽  
P Value ◽  
Primary Therapy ◽  
Newly Diagnosed ◽  
Entire Cohort ◽  
The Impact

Abstract Abstract 4742 Introduction While the distance patients travel to a treatment center (DTC) adversely impacts survival of patients with trauma, cardiac, or neurological disorders, as well as certain solid tumors, less is known of its influence in acute myeloid leukemia (AML). Care for patients with AML involves frequent emergent and urgent management, often complicating primary therapy provided in distant tertiary referral centers. We therefore hypothesized that increased DTC has a negative impact on outcome. We tested this hypothesis by assessing the effect of DTC on survival of patients with AML receiving care at a single institution. Patients and Methods Within the Stanford Leukemia Database, we identified 884 consecutive adult patients between 1993 and 2009 meeting the following criteria: age >=18, newly diagnosed AML (excluding APL), clinical management at Stanford University Medical Center (SUMC), and verified residence location available for DTC determination. Of these, 571 were deemed fit by the admitting physician to receive myelosuppressive induction chemotherapy. DTC was calculated by straight-line journey distance between home address at the time of diagnosis and treatment center. Results The median age for the entire cohort is 55 years and 322 patients (36%) are older than 60 years of age. Median survival for the entire cohort was 14.0 months. DTC was not univariately associated with outcome as a continuous variable. When testing for a critical DTC threshold impacting outcomes across the entire cohort, we found a significant correlation between longer DTC and adverse outcomes, shorter DTC was associated with lower OS. Patients living within 20 miles of SUMC had a worse median overall survival (10.4 months versus 15.0 months, HR 1.23, corrected p-value 0.02). However, when adjusted for administration of induction chemotherapy (p<0.0001), age at presentation (p<0.0001) and karyotype at diagnosis (CBF vs other; p-value- 0.92), the negative impact of DTC was lost (p=0.08). Conclusion After accounting for confounding factors, DTC has no significant impact on the outcome of newly diagnosed AML patients receiving care at our institution. Unlike non-hematologic malignancies, distance to treatment center likely does not adversely influence outcomes for patients with AML. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Classification of Pulmonary Hypertension

2014 ◽  
Vol 13 (1) ◽  
pp. 17-20
Author(s):  
Rogerio Souza ◽  
Gerald Simonneau
Keyword(s):  
Pulmonary Hypertension ◽  
Task Force ◽  
Management Strategies ◽  
Pathological Findings ◽  
Clinical Classification ◽  
Current Classification ◽  
Pulmonary Arterial ◽  
Current Classification System ◽  
Group 1

Classification of pulmonary hypertension groups patients with similar pathological findings, hemodynamic profiles, and management strategies. Minor modifications have been made to the current classification system, particularly within Group 1 pulmonary arterial hypertension. This article summarizes the published conclusions of the Fifth World Symposium of Pulmonary Hypertension task force that addressed the updated clinical classification of pulmonary hypertension.

scholarly journals Intensity and quality of exertional dyspnoea in patients with stable pulmonary hypertension

2019 ◽  
Vol 55 (2) ◽  
pp. 1802108 ◽  
Author(s):  
Athénaïs Boucly ◽  
Capucine Morélot-Panzini ◽  
Gilles Garcia ◽  
Jason Weatherald ◽  
Xavier Jaïs ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Minute Ventilation ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Dynamic Hyperinflation ◽  
Exertional Dyspnoea ◽  
Work Effort ◽  
Inspiratory Capacity ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
The Impact

Dynamic hyperinflation is observed during exercise in 60% of patients with clinically stable pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), intensifying exertional dyspnoea. The impact of dynamic changes in respiratory mechanics during exercise on qualitative dimensions of dyspnoea in these patients has not been evaluated.26 patients (PAH n=17; CTEPH n=9) performed an incremental symptom-limited cycle exercise test. Minute ventilation (V′E), breathing pattern, operating lung volumes and dyspnoea intensity were assessed throughout exercise. Dyspnoea quality was serially assessed during exercise using a three-item questionnaire (dyspnoea descriptors). The inflection point of tidal volume (VT) relative to V′E was determined for each incremental test. Changes in inspiratory capacity during exercise defined two groups of patients: hyperinflators (65%) and non-hyperinflators (35%). Multidimensional characterisation of dyspnoea was performed after exercise using the Multidimensional Dyspnea Profile.In hyperinflators, inspiratory capacity decreased progressively throughout exercise by 0.36 L, while remaining stable in non-hyperinflators. The “work/effort” descriptor was most frequently selected throughout exercise in both types of patients (65% of all responses). At the VT/V′E inflection, work/effort plateaued while “unsatisfied inspiration” descriptors became selected predominantly only in hyperinflators (77% of all responses). In the affective domain, the emotion most frequently associated with dyspnoea was anxiety.In pulmonary hypertension patients who develop hyperinflation during exercise, dyspnoea descriptors referring to unsatisfied inspiration become predominant following the VT/V′E inflection. As these descriptors are generally associated with more negative emotional experiences, delaying or preventing the VT/V′E inflection may have important implications for symptom management in patients with pulmonary hypertension.

scholarly journals QOLP-31. ASSESSING THE IMPACT OF GLIOBLASTOMA ON WORK PRODUCTIVITY IN PATIENTS AND THEIR CAREGIVERS

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi204-vi204
Author(s):  
Terri Armstrong ◽  
Vinay Puduvalli ◽  
Tobias Walbert ◽  
Katherine Peters ◽  
Ashlee Loughan ◽  
...  
Keyword(s):  
Cognitive Function ◽  
Stable Disease ◽  
Work Productivity ◽  
Daily Activities ◽  
Newly Diagnosed ◽  
Activity Impairment ◽  
Eortc Qlq C30 ◽  
Eortc Qlq ◽  
The Impact ◽  
Group 1

Abstract Information is limited on the impact of glioblastoma (GBM) on patient employment and caregiver time commitments. This observational multisite study evaluated the impact of GBM on patients’ employment and on caregivers’ ability to work and perform daily activities. A total of 180 adults diagnosed with GBM and their caregivers were stratified across 4 groups representing specific stages in the disease course: Group 1=newly diagnosed; 2=postradiation; 3=stable disease; 4=recurrence. We assessed the impact of GBM on employment status and self-reported cognitive function in patients (EORTC QLQ-C30) and in caregivers (Work Productivity and Activity Impairment Questionnaire). Prior to diagnosis, 64% of all patients (74% < 65 years old) were employed, while only 22% (26% for those < 65 years old) were employed after diagnosis. Though not reaching significance, newly diagnosed patients and those with stable disease reported higher mean cognitive function scores (72.0 and 71.9) than other groups (61.1 and 61.3; P=0.227). At study enrollment, 56% of all caregivers were employed, while 41% had left their jobs to care for their patients. The impact on caregiver work productivity varied with a higher percentage of caregivers of newly diagnosed patients and those with recurrent disease missing work (45% and 24%, respectively) and experiencing work impairment (54% and 36%, respectively) than the other groups (P< 0.05). Forty-one percent of caregivers of newly diagnosed patients to 25% of patients with stable disease reported impairment of daily activities. GBM had a significant impact on patient and caregiver employment from the time of diagnosis. High cognitive burden among patients might have impacted their employment. Overall work and activity impairment for caregivers was evident in all groups with a notable impact at the time of first diagnosis and recurrence of disease. Future studies will explore the impact of these findings in a longitudinal cohort.

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