scholarly journals EXPRESS: Global Trends in Chronic Thromboembolic Pulmonary Hypertension Clinical Trials and Dissemination of Results

2021 ◽  
pp. 204589402110599
Author(s):  
Micheal McInnis ◽  
Clement T Chow ◽  
Alexandre Boutet ◽  
Sebastian Mafeld ◽  
John Granton ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
World Health ◽  
Thromboembolic Pulmonary Hypertension ◽  
National Affiliation ◽  
Group 3 ◽  
Group 2 ◽  
Clinical Trials Registry ◽  
Group 1

Treatment options for chronic thromboembolic pulmonary hypertension (CTEPH) are rapidly expanding. The purpose of this study is to identify trends in CTEPH clinical trials and the publication of results. We performed a worldwide review of completed and ongoing clinical trials through searching the ClinicalTrials.gov database and the World Health Organization International Clinical Trials Registry Platform for “CTEPH” and related terms. Entries were classified as pharmaceutical/procedural interventions (Group 1), all other clinical trials (Group 2) and patient registries (Group 3). Trial characteristics and national affiliation were recorded. PubMed was searched for related publications. There were 117 clinical trials registry entries after removing duplicates and non-target records. Group 1 comprised 29 pharmaceutical, 15 procedural, and 4 combined interventions starting in 2005, 2010 and 2016, respectively. Riociguat and balloon pulmonary angioplasty (BPA) were the most frequent pharmaceutical and procedural interventions, respectively. The proportion of procedural trials increased over time from 0% of those in 2005-2009, to 29% in 2010-2014 and 54% in 2015-2020. There were 56 entries in Group 2 and 13 in Group 3. Japan was the most frequent national affiliation and the most frequent participating country, present in 28% of all trials. The proportion of entries with published results was highest with Group 3 (62%) and lowest with Group 1 (27%). 30% of all publications occurred in 2020. In conclusion, CTEPH clinical trials are increasingly procedural based with growth largely attributable to Japan and BPA. Most trials have not published but results from BPA clinical trials are anticipated soon.

scholarly journals Non-Group 1 Pulmonary Hypertension Associated With Systemic Sclerosis: An Under-studied Patient Population

2017 ◽  
Vol 16 (2) ◽  
pp. 68-75
Author(s):  
Zafia Anklesaria ◽  
Rajeev Saggar ◽  
Ariss Derhovanessian ◽  
Rajan Saggar
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
World Health ◽  
Response To Treatment ◽  
Specific Therapy ◽  
Pulmonary Manifestations ◽  
Group 3 ◽  
Group 2 ◽  
The Impact ◽  
Group 1

Background: Systemic sclerosis (SSc) is a heterogeneous disorder that results in multiorgan dysfunction. The most common pulmonary manifestations are pulmonary hypertension (PH) and interstitial lung disease (ILD). Systemic sclerosis may be complicated by World Health Organization (WHO) Group 1 PH (SSc-PAH), which is the most well-studied subtype. The PH associated with SSc may also be secondary to underlying left heart disease (SSc-PH-LHD) or ILD (SSc-PH-ILD), and these subgroups are classified as WHO Group 2 and Group 3 PH, respectively. These non-WHO Group 1 PH subsets are notoriously under-studied. Available data suggest that the impact of PH-specific therapy in SSc-PH-LHD and SSc-PH-ILD is limited and survival is poor despite attempted treatment. Implication for clinicians: Most research and clinical trials surrounding PH in SSc have thus far focused on WHO Group 1 SSc-PAH. There are limited data surrounding therapeutic options for WHO Group 2 (SSc-PH-LHD) and Group 3 PH (SSc-PH-ILD) phenotypes. This review aims to summarize and consolidate the data surrounding these 2 distinct clinical phenotypes and to emphasize the available prognostic and treatment considerations. Conclusions: Given the unique pathophysiology, prognostic implications, and poor response to treatment of WHO Group 2 and 3 SSc-PH phenotypes, there is an overwhelming need for more data to best understand optimal management strategies. The focus should be individual patient-level prognostication, how and when to initiate and manage PH-specific therapy, and appropriate triage with regard to the timing of lung (or heart-lung) transplantation.

scholarly journals Predictors of Chronic Thromboembolic Pulmonary Hypertension

Kardiologiia ◽  
2018 ◽  
Vol 58 (12) ◽  
pp. 60-65
Author(s):  
M. R. Kuznetsov ◽  
I. V. Reshetov ◽  
B. B. Orlov ◽  
A. A. Khotinsky ◽  
A. A. Atayan ◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Anticoagulant Therapy ◽  
Vena Cava ◽  
Significant Risk ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Thromboembolic Pulmonary Hypertension ◽  
Group 2 ◽  
Group 1

Purpose:to elucidate predictors of development of chronic thromboembolic pulmonary hypertension (CTEPH) after acute pulmonary artery thromboembolism (PTE).Material and methods. We included in this study 210 patients hospitalized with diagnosis of submassive and massive PTE from 2013 to 2017. In 1 to 3 years after initial hospitalization these patients were invited for control examination. According to results of this examination patients were divided into two groups: with (group 1, n=45) and without (group 2, n=165) signs of CTEPH. Severity of pulmonary artery vascular bed involvement was assessed by multislice computed tomography (MSCT) angiography and lung scintigraphy. For detection of thrombosis in the inferior vena cava system we used ultrasound angioscanning.   Examination also included echocardiography.Results.In the process of mathematical analysis, the following risk factors for the development of CTEPH embolism were determined: duration of thrombotic history (group 1 – 13.70±2.05 days, group 2– 16.16±1.13 days, p=0.015), localization of venous thrombosis in the lower extremities (the most favorable – shin veins, popliteal, and common femoral veins, unfavorable – superficial femoral vein). The choice of the drug for thrombolytic and anticoagulant therapy: streptokinase and urokinase were significantly more effective than alteplase, rivaroxaban was superior to the combination of unfractionated or low molecular weight heparins with warfarin. Also, risk factors for the development of CTEPH were the initial degree of pulmonary hypertension and tricuspid insufficiency, as well as the positive dynamics of these indicators at the background of thrombolytic or anticoagulant therapy. Of concomitant diseases, significant risk factors for development of CTEPH were grade 3 hypertensive disease, diabetes mellitus, post­infarction cardiosclerosis. On the other hand, age, gender, degree of severity at the time of admission, presence of infarction pneumonia, surgical prevention of recurrent pulmonary embolism, number of pregnancies and deliveries, history of trauma and malignancies, cardiac arrhythmias produced no significant impact on the development of CTEPH.

P4674Implementation of the new chronic thromboembolic pulmonary hypertension treatment algorithm: an ongoing effort from a portuguese referral centre

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Alegria ◽  
M J Loureiro ◽  
F Ferreira ◽  
R Cale ◽  
M Cabral ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Treatment Algorithm ◽  
Treatment Strategies ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
All Cause Mortality ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Background To date, the first-line treatment for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA), although a significant number of patients will have inoperable disease or residual pulmonary hypertension (PH). Balloon pulmonary angioplasty (BPA) has provided a new therapeutic option for these patients. In addition, medical therapy (MT) also plays an important role. Purpose Characterization of a population of patients with CTEPH or chronic thromboembolic disease (CTED) and comparison of the different treatment strategies according to the updated treatment algorithm. Methods Retrospective analysis of patients with CTEPH/CTED followed in a referral centre for the treatment of PH submitted to different treatment strategies: PEA plus MT and BPA in patients with residual PH (group 1), MT plus BPA (group 2), and MT only (group 3). Cox regression was used to identify predictors of all-cause mortality. Results 58 patients were included (median age 63.5 years, 74% female); 17% had CTED, and the remaining had CTEPH 50% (n=29) were submitted to PEA (group 1), of which 58% had residual PH (21% underwent BPA, n=3). Among the remaining patients, 31% (n=9) underwent MT plus BPA (group 2), and 69% (n=20) were treated with MT only (group 3). Overall, 55% were under pulmonary vasodilator therapy, including 38% with riociguat. Most of the patients (67%) were in functional class III or IV, the distance in the 6MWT was 328±147 meters, the median NT-proBNP was 538pg/ml, 40% had RV systolic dysfunction, the mean mPAP was 42±13 mmHg, and the mean PVR was 11±6 WU. Comparing the different treatment strategies, during follow-up (median 945 days) the following differences were found (comparison between group 1 vs group 2 vs group 3): improvement in functional class (class III-IV: 0% vs 0% vs 58%); distance in 6MWT (438±83 vs 390±79 vs 281±105 meters); evolution of NT-proBNP (−984±1736 vs −198±205 vs +1177±2342); normalization of RV dimensions (89% vs 50% vs 20%); resolution of pericardial effusion (100% vs 100% vs 0%); normalization of mPAP (73% vs 71% vs 0%); PVR (median 3.4 vs 2.7 vs 10.6 WU); all-cause mortality (7% vs 0% vs 35%) (p<0.02 in all). In the overall population, the most relevant predictors of all-cause mortality were the absence of functional class improvement, baseline and follow-up NT-proBNP, baseline and follow-up SPAP by echocardiogram, and maintenance of treatment with prostanoids (p<0.05 in all). Conclusion Our results confirm that, in patients with CTEPH/CTED, PEA is associated with functional, and hemodynamic improvement and increased survival, although BPA is an alternative in patients with inoperable disease or residual PH, with similar results on short-term follow-up. Patients who are not submitted to surgical or percutaneous intervention have a poor prognosis, both in terms of morbidity and mortality.

scholarly journals How is juvenile rheumatic heart disease different: baseline results from a prospective north Indian registry

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K Mahajan ◽  
D.R Prakash Chand Negi
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Funding Source ◽  
Thromboembolic Events ◽  
Group 3 ◽  
Group 2 ◽  
Rheumatic Heart ◽  
Group 1

Abstract Introduction Juvenile rheumatic heart disease (RHD) refers to RHD in patients &lt;20 years of age. There are no contemporary data highlighting the differences between juvenile and older RHD patients. Purpose We aim to report the age related differences in the pattern, and consequencies of valvular dysfunction in patients of RHD. Methods The 2475 consecutive patients of RHD diagnosed using clinical and echocardiographic criteria were registered prospectively from 2011 till December 2019. Patients were divided into 3 groups according to their age: Group 1 (Juvenile RHD), Group 2 (21–50 years), and Group 2 (&gt;51 years).The data concerning the socio-demographic and clinical profile were recorded systematically, and the nature and severity of valvular dysfunction was assessed by echocardiography. The data were analyzed using the Epi-InfoTM Software. Results Out of 2475 RHD patients, Juvenile RHD comprised of 211 (8.5%) patients. Group 2 and 3 comprised of 1691 (68.3%) and 573 (23.2%) patients respectively. Overall, 1767 (71.4%) patients were females, however this female predilection was less pronounced in juvenile RHD (55.5% females vs 44.5% males) as compared to older groups. Past history of acute rheumatic fever was more commonly recorded in Juvenile RHD group (37.9% vs 18.8% in group 2 and 10% in group 3, p=0.0001). At the time of registration, the presence of advanced heart failure symptoms (dyspnea class III and IV) (11.4% group 1 vs 13.9% group 2 vs 20.6% group 3, p&lt;0.0001), right heart failure symptoms (0.9% group 1 vs 2.5% group 2 vs 7.3% group 3, p&lt;0.01), thromboembolic events (0% group 1 vs 4.1% group 2 vs 3.3% group 3, p&lt;0.01), atrial fibrillation (2.8% group 1 vs 24.5% group 2 vs 45.9% group 3, p&lt;0.0001), and pulmonary hypertension (27.1% group 1 vs 40.3% group 2 vs 51.9% group 3, p&lt;0.01), were all more commonly recorded in non-juvenile older RHD groups. Multivalvular involvement was also less common in juvenile RHD (34.6% vs 42.4% and 44.5%, p=0.04). Mitral regurgitation was the most common lesion in Juvenile RHD followed by aortic regurgitation (68.7% and 40.2% respectively). Stenotic lesions (both mitral and aortic) were present more commonly in older age groups. Conclusion RHD is predominantly a disease of females, however the predilection is less common in juvenile patients. Juvenile RHD predominantly affects the mitral valve and mainly leads to regurgitant lesions. As the age advances, the complications of RHD, mainly heart failure symptoms, thromboembolic events, pulmonary hypertension, and atrial fibrillation, become more common. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Self sponsored registry

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 ).

1409Use of phenomapping to determine response of treatment by sacubitril/valsartan in patients with heart failure with reduced ejection fraction

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Godet ◽  
O Raitiere ◽  
H Chopra ◽  
P Guignant ◽  
C Fauvel ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Ejection Fraction ◽  
Left Ventricular ◽  
Heat Map ◽  
Reduced Ejection Fraction ◽  
Patients With Heart Failure ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Background Treatment by sacubitril/valsartan decreases mortality, improves KCCQ score and ejection fraction in patients with heart failure with reduced ejection fraction (HF REF), but there is currently no data to predict response to treatment. Purpose The purpose of our work was to assess whether unbiased clustering analysis, using dense phenotypic data, could identify phenotypically distinct HF-REF subtypes with good or no response after 6 months of sacubitril/valsartan administration. Methods A total of 78 patients in NYHA functional class 2–3 and treated by ACE inhibitor or AAR2, were prospectively assigned to equimolar sacubitril/valsartan replacement. We collected demographic, clinical, biological and imaging continuous variables. Phenotypic domains were imputed with 5 eigenvectors for missing value, then filtered if the Pearson correlation coefficient was >0.6 and standardized to mean±SD of 0±1. Thereafter, we used agglomerative hierarchical clustering for grouping phenotypic variables and patients, then generate a heat map (figure 1). Subsequently, participants were categorized using Penalized Model-Based Clustering. P<0,05 was considered significant. Results Mean age was 60.4±13.4 yo and 79.0% patients were males. Mean ejection fraction was 29.3±7.0%. Overall, 16 phenotypic domains were isolated (figure 1) and 3 phenogroups were identified (Table 1). Phenogroup 1 was remarkable by isolated left ventricular involvement (LVTDD 64.3±5.9mm vs 73.9±8.7 in group 2 and 63.8±5.7 in group3, p<0.001) with moderate diastolic dysfunction (DD), no mitral regurgitation (MR) and no pulmonary hypertension (PH). Phenogroups 2 and 3 corresponded to patients with severe PH (TRMV: 2.93±0.47m/s in group 2 and 3.15±0.61m/s in groupe 3 vs 2.16±0.32m/s in group 1), related to severe DD (phenogroup 2) or MR (phenogroup 3). In both phenogroups, the left atrium was significantly enlarged and the right ventricle was remodeled, compared with phenogroup 1. Despite more severe remodeling and more compromised hemodynamic in phenogroups 2 and 3, the echocardiographic response to sacubitril/valsartan was comparable in all groups with similar improvement of EF and reduction of cardiac chambers dimensions (response of treatment, defined by improvement of FE +15% and/or decreased of indexed left ventricule diastolic volume −15% = group 2: 22 (76%); group 3: 18 (60%); group 1: 9 (50%); p=0.17; OR group 2 vs 1: OR=3.14; IC95% [0.9–11.03]; p=0.074; OR group 3 vs 1: OR=1.5; IC95% [0.46–4.87]; p=0.5)). The clinical response was even better in phenogroups 2 and 3 (Group 2: 19 (66%); group 3: 21 (78%) vs group 1: 9 (50%); p=0.05). Heat map Conclusion HF-REF patients with severe diastolic dysfunction, significant mitral regurgitation and elevated pulmonary hypertension by echocardiographic had similar reverse remodeling but better clinical improvement than patients with isolated left ventricular systolic dysfunction.

scholarly journals Respiratory rate modulation improves symptoms in patients with pulmonary hypertension

2021 ◽  
Vol 9 ◽  
pp. 205031212110539
Author(s):  
Barbro Kjellström ◽  
Bodil Ivarsson ◽  
Lise-Lotte Landenfelt Gestré ◽  
Henrik Ryftenius ◽  
Magnus Nisell
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Respiratory Rate ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
World Health ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Respiratory Modulation ◽  
Health Organization

Background: Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are chronic diseases with a severe symptom burden. Common symptoms are dyspnoea at light activity and general fatigue that limits daily activities. Respiratory modulation by device-guided breathing decreased symptoms in patients with heart failure. The aim of this pilot study was to investigate if respiratory modulation could improve symptoms of dyspnoea in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Method: Adult patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension with symptoms of dyspnoea at rest or light activity performed home-based respiratory modulation by device-guided breathing 20 min a day for 3 months. Patients were on stable disease-specific treatment ⩾3 months and willing to undergo all study procedures. Dyspnoea score, World Health Organization class, physical status, N-terminal pro b-type natriuretic peptide, quality of life, respiratory rate and 6-min walk distance were assessed before and after 3 months with respiratory modulation. Results: Nine patients with pulmonary arterial hypertension and five with chronic thromboembolic pulmonary hypertension completed the study protocol. Mean age was 71 ± 14 years, and 11 were women. After 3 months of respiratory modulation, dyspnoea score (−0.6, p = 0.014), respiratory rate at rest (−3 breaths/min, p = 0.013), World Health Organization class (−0.3, p = 0.040), quality of life (EuroQol Visual Analogue Scale +5 points, not significant) and decreased N-terminal pro b-type natriuretic peptide (−163 ng/L, p = 0.043) had improved. The fatigue and respiratory rate after the 6-min walk decreased while the 6-min walk distance remained unchanged. Conclusion: Patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension that used device-guided breathing for 3 months improved symptoms of dyspnoea and lowered the respiratory rate at rest and after exercise.

scholarly journals Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry

2020 ◽  
Vol 6 (2) ◽  
pp. 00218-2019
Author(s):  
Abdullah M. Aldalaan ◽  
Sarfraz A. Saleemi ◽  
Ihab Weheba ◽  
Abeer Abdelsayed ◽  
Pekka Hämmäinen ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Disease Severity ◽  
Tertiary Care ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
World Health ◽  
Cumulative Probability ◽  
Probability Of Survival ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive vascular disease with poor prognosis if left untreated. This study aims to assess the patient characteristics, treatment approach and clinical and survival outcomes for CTEPH patients enrolled in the Systematic Prospective Follow Up for Better Understanding of Clinical Characteristics of Patients with Pulmonary Hypertension Disease (SAUDIPH) registry.MethodsThis study presents a subanalysis of CTEPH patients enrolled in the SAUDIPH registry. This registry enrolled patients with pulmonary hypertension, established through right heart catheterisation, under clinical management at a specialised tertiary care centre. Patients received standard care during the period of the registry.ResultsAt the time of this analysis, 64 CTEPH patients were enrolled in the registry. Mean age at diagnosis was 39.7 years and there was a female predominance (67.6%). At baseline, most patients were in World Health Organization functional classes III or IV (70.1%). At the last follow-up visit, most patients (63.2%) had undergone endarterectomy, showing significant improvement in disease severity from baseline. Patients who underwent endarterectomy showed numerically higher (p=0.126) probability of survival at 1 year (97.5%) versus those who did not undergo endarterectomy (94.4%).ConclusionPatients were diagnosed at relatively young age, but still showed high disease severity, suggesting delay in diagnosis. Patients who underwent surgical treatment showed substantial improvements in clinical and haemodynamic parameters, while the remaining patients tended to show disease progression. The 96.6% 1-year cumulative probability of survival was high compared to previous studies.

“Portrait” of patients with idiopathic pulmonary hypertension and chronic thromboembolic pulmonary hypertension depending on comorbid status: current and prognosis features

2020 ◽  
Vol 30 (4) ◽  
pp. 427-436
Author(s):  
I. N. Taran ◽  
A. A. Belevskaya ◽  
Z. S. Valieva ◽  
M. A. Saidova ◽  
T. V. Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Step Test ◽  
3 Dimensional ◽  
Thromboembolic Pulmonary Hypertension ◽  
Idiopathic Pulmonary Hypertension ◽  
Group 5 ◽  
Group 2 ◽  
The Moment ◽  
The Right

Changing the "portrait" of patients with idiopathic pulmonary hypertension (IPH) and chronic thromboembolic pulmonary hypertension (CTPH) over the past decade suggests the need to develop models of phenotypes in patients with this cohort.The purpose of the study was to estimate the nature of the disease course and prognosis of patients with IPH and inoperable CTPH based on the formation of phenotypic groups depending on the comorbid status.Materials and Methods. Patients with IPH (n = 88) and inoperable CTPH (n = 38) aged 38.5 [28.5; 51] and 53.5 [41; 58] years respectively were enrolled in the study. 6-minute step test (6-MST) and spiroveloergometry, transthoracic echocardiography (EchoCG), catheterization of the right side of the heart was performed at the moment of diagnosis verification and in 13 [12; 20] months. All patients were divided into 5 groups according to comorbid status: Group 1 (n = 29) – patients with IPH/CTPH without comorbid pathology; Group 2 (n = 23) with body mass index (BMI) ≥ 25 kg/m2 ; Group 3 (n = 27) – with BMI ≥ 25 kg/m2 and dyslipidemia; Group 4 (n = 30) with hypertension, BMI ≥ 25 kg/m2 and dyslipidemia; Group 5 (n = 17) with the listed comorbid pathologies and carbohydrate metabolic disorders.Results. Initially more severe functional status was observed in patients of the 4th and 5th groups as compared to those in the 1st and 3rd groups. According to the data of the 3-dimensional EchoCG, in patients of the 4th and 5th groups a reliably more expressed increase in the volume of the right ventricle was revealed in comparison with such values in the 1st and 3rd groups. In IPH groups 1 and 3, the positive results of the vasoreactivity test (34.5% and 48.2%) were significantly more frequent in comparison with groups 4 (6.7%) and 5 (5.9%). The period from the diagnosis verification to adding the 2nd specific drug was significantly shorter in patients with IPH/CTPH of the 5th group compared to patients of the 3rd group. a high risk of lethal outcome after 13 [12; 30] months of treatment was significantly less frequently observed with IPH in Groups 1 and 3 as compared to Group 5.Conclusion. The combination of BMI ≥ 25 kg/m2, dyslipidemia, hypertension, as well as the additional presence of carbohydrate metabolism disorders is associated with a less favorable course of the disease in patients with IPH and inoperable CTPH.

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