Value of systolic right ventricular function parameters in children with pulmonary arterial hypertension associated with congenital heart diseases

2013 ◽  
Vol 166 (4) ◽  
pp. e33
Author(s):  
Martin Koestenberger ◽  
William Ravekes
2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Seyeon Oh ◽  
Albert Y. Jang ◽  
Sehyun Chae ◽  
Seungbum Choi ◽  
Jeongsik Moon ◽  
...  

AbstractDespite the advancement of targeted therapy for pulmonary arterial hypertension (PAH), poor prognosis remains a reality. Mesenchymal stem cells (MSCs) are one of the most clinically feasible alternative treatment options. We compared the treatment effects of adipose tissue (AD)-, bone marrow (BD)-, and umbilical cord blood (UCB)-derived MSCs in the rat monocrotaline-induced pulmonary hypertension (PH) model. The greatest improvement in the right ventricular function was observed in the UCB-MSCs treated group. The UCB-MSCs treated group also exhibited the greatest improvement in terms of the largest decrease in the medial wall thickness, perivascular fibrosis, and vascular cell proliferation, as well as the lowest levels of recruitment of innate and adaptive immune cells and associated inflammatory cytokines. Gene expression profiling of lung tissue confirmed that the UCB-MSCs treated group had the most notably attenuated immune and inflammatory profiles. Network analysis further revealed that the UCB-MSCs group had the greatest therapeutic effect in terms of the normalization of all three classical PAH pathways. The intravenous injection of the UCB-MSCs, compared with those of other MSCs, showed superior therapeutic effects in the PH model for the (1) right ventricular function, (2) vascular remodeling, (3) immune/inflammatory profiles, and (4) classical PAH pathways.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Cristiano Miotti ◽  
Roberto Badagliacca ◽  
Carmine Dario Vizza

Abstract Aims Right ventricular function adaptation to afterload as assessed by the ratio of tricuspid annular plane excursion (TAPSE) to systolic pulmonary artery pressure (PASP) has been shown to be of prognostic relevance in pulmonary arterial hypertension (PAH). To test the hypothesis that improvement in TAPSE/PASP under targeted therapies is associated with the likelihood to achieve a better prognostic low-risk status in PAH. Methods and results The study retrospectively enrolled 677 PAH patients (55% idiopathic) with follow-up clinical, right heart catheterization, and echocardiographic evaluations within 12 months [interquartile range (IQR): 180–344 days] after initiation of targeted therapies from 2005 to 2017 in 11 Italian centres. European guidelines-derived and United States Registry to Evaluate Early and Long-Term PAH Disease Management registry REVEAL 2.0 risk scores were assessed at baseline and follow-up. The patients improved their functional class and 6-min walk distance, but a minority of them achieved or maintained the low risk status as assessed either with the European or the REVEAL 2.0 score (30% and 27%, respectively). The TAPSE/PASP ratio increased curvilinearly in proportion to decreased pulmonary vascular resistance (PVR) by more than 50%. Patients at low risk were, respectively, 4.93 and 3.37 times more likely to have TAPSE/PASP ≥0.35 mm/mmHg than those at intermediate or high risk, according to the ESC/ERS [odds ratio: (OR) 4.93, confidence interval (CI): 3.40–7.14; P = 0.0001] and the REVEAL 2.0 score (OR: 3.37, CI: 2.32–4.90; P = 0.0001). Conclusions Improvement of TAPSE/PASP under targeted therapies in PAH is associated with a low risk status, marked reduction in PVR, and improved outcome.


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