Increased native T1-values at the interventricular insertion regions in precapillary pulmonary hypertension

Abstract Cardiac magnetic resonance imaging of the pressure overloaded right ventricle (RV) of precapillary pulmonary hypertension (PH) patients, exhibits late gadolinium enhancement at the interventricular insertion regions, a phenomenon which has been linked to focal fibrosis. Native T1-mapping is an alternative technique to characterize myocardium and has the advantage of not requiring the use of contrast agents. The aim of this study was to characterize the myocardium of idiopathic pulmonary arterial hypertension (IPAH), systemic scleroderma related PH (PAH-Ssc) and chronic thromboembolic PH (CTEPH) patients using native T1-mapping and to see whether native T1-values were related to disease severity. Furthermore, we compared native T1-values between the different precapillary PH categories. Native T1-mapping was performed in 46 IPAH, 14 PAH-SSc and 10 CTEPH patients and 10 control subjects. Native T1-values were assessed using regions of interest at the RV and LV free wall, interventricular septum and interventricular insertion regions. In PH patients, native T1-values of the interventricular insertion regions were significantly higher than the native T1-values of the RV free wall, LV free wall and interventricular septum. Native T1-values at the insertion regions were significantly related to disease severity. Native T1-values were not different between IPAH, PAH-Ssc and CTEPH patients. Native T1-values of the interventricular insertion regions are significa...
Source: The International Journal of Cardiovascular Imaging - Category: Radiology Source Type: research

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ConclusionThe absence of PSGL ‐1 induces a reduction in Treg cells, NO production, and ERα expression and causes an increase in Ang II in the lungs of female mice, favoring the development of PAH.
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: Original Article Source Type: research
CONCLUSIONS: In our cohort, the prevalence of serodiscordant SSc patients was low. They differed from their counterparts in some clinical manifestations. The management of patients with SSc should be guided by both serology and cutaneous subtype. PMID: 31969223 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
We report the case of a 72-year-old woman with limited systemic sclerosis, who was initially diagnosed with systemic sclerosis-related pulmonary arterial hypertension. However, after introduction of sildenafil and bosentan, the patient developed acute pulmonary oedema, and findings from complementary exams were suggestive of PVOD.
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
ConclusionThe combination of low peak VO2, high VE/VCO2 slope, low PetCO2, and high respiratory reserve suggest that patients with SSc without overt cardiac or respiratory disease present with a cardiovascular limitation to exercise. The latter may be related to latent cardiac dysfunction or pulmonary vascular disease.
Source: Archives of Cardiovascular Diseases Supplements - Category: Cardiology Source Type: research
Objective: To assess the role of BAL fluid IL-6 and IL-7 levels in disease activity assessment in Systemic sclerosis-associated interstitial lung disease (SSc-ILD)Methods: Twenty cases of SSc-ILD were enrolled after excluding overlap syndrome, smokers, lung infection, and pulmonary hypertension. They were evaluated with six-minute walk test, spirometry, HRCT-chest with semi-quantitative scoring and bronchoalveolar lavage. Re-evaluation was done at 6 months after six cyclophosphamide pulse (CYP) therapy. BAL fluid IL-6 and IL-7 levels were estimated using ELISA based kits. Twenty patients of carcinoma esophagus undergoing p...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
Conclusions: Psychological evaluation and social support become mandatory because their prognosis depends on these issues.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Background: Patients with systemic sclerosis (SSc) are at high risk of developing pulmonary hypertension (PH) which crucially affects patients’ symptoms, quality of life and prognosis. The aim of this study was to analyse the impact of the new hemodynamic definition of precapillary PH as proposed at the 2018 World Symposium on PH in SSc-patients.Methods: SSc-patients were screened for PH using clinical assessments as WHO-functional class, 6 minute walking distance (6MWD) and right heart catheterisation. Patients were divided into hemodynamic subgroups according to their mean pulmonary arterial pressure (mPAP) values ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
In this study, we aimed to define whether change influences on survival in patients with PAH-SSc.Methods: We performed a cohort study of PAH-SSc patients (n=76; mean age 53±11 years, 92% female) evaluated at the Institute. The Kaplan-Meier method and Cox regression analysis were used to assess survival and predictors of mortality. The clinical characteristics and survival were compared in patients categorized by PAH severity based on the World Health Organization classification and 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.Results: We found that survival to be dependent on the...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Conclusions:Cardiopulmonary complications are common in patients with scleroderma, one of which may be sleep-disordered breathing. In our cohort, approximately one-third of individuals with scleroderma had evidence of sleep-disordered breathing. Moreover, the rate of sleep-disordered breathing in our population of scleroderma patients was twice the rate of pulmonary hypertension and was approximately the same as the rate of interstitial lung disease. Future prospective studies are needed to further assess the role of sleep-disordered breathing in scleroderma clinical outcomes.Citation:Nokes BT, Raza HA, Cartin-Ceba R, Lyng...
Source: Journal of Clinical Sleep Medicine : JCSM - Category: Sleep Medicine Source Type: research
CONCLUSION: In this high-risk cohort of patients with SSc, a positive EE may predict the future development of resting PH. In addition, a majority of patients may have a persistently positive EE for years without progression to resting PH. Finally, a consistently negative EE may identify patients at low risk for future PH. PMID: 31732551 [PubMed - as supplied by publisher]
Source: J Rheumatol - Category: Rheumatology Authors: Tags: J Rheumatol Source Type: research
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