Pulmonary arterial wall thickness as a promising echocardiographic follow-up parameter in severe pediatric pulmonary hypertension

2015 ◽  
Vol 50 (12) ◽  
pp. 1176-1177
Author(s):  
Martin Koestenberger ◽  
Sabrina Schweintzger
2015 ◽  
Vol 50 (12) ◽  
pp. 1253-1261 ◽  
Author(s):  
Nazmi Narin ◽  
Abdullah Ozyurt ◽  
Suleyman Sunkak ◽  
Ali Baykan ◽  
Mustafa Argun ◽  
...  

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 1146-1146
Author(s):  
Liping Ma ◽  
Jianxing Chang ◽  
Hui Wu ◽  
Yabing Chen

Abstract Abstract 1146 Pulmonary arterial hypertension (PAH) is often as a result of COPD and contributes to morbidity and mortality of chronic cor pulmonale. Hypoxemia is its main clinical features. The toll-like receptors are a group of type I transmembrane proteins that play a central role in specific recognition of pathagen-associated molecular patterns and are critical for the induction of innate immunity and inflammation. Expression of these innate immune receptors, especially TLR4 in healthy and pathological arteries has implicated its role in the homeostasis of vasculature. Chronic hypoxia-induced PAH is mediated by increased production of reactive oxygen species (ROS). ROS have been linked to activation of toll-like receptor 4 (TLR4) signaling. The present studies investigated the role of TLR4 in the pathogenesis PAH and the underlying mechanisms. In vitro, pulmonary arterial smooth muscle cells (PASMC) derived from TLR4−/− mice exhibited increased production of intracellular reactive oxygen species (ROS). Furthermore, under hypoxia, the expression of TLR4 in PASMC from WT mice was decreased, ROS production in PASMC from WT mice were increased, suggesting down-regulation of TLR4 may contribute to hypoxia-induced PAH. Using a hypoxia-induced PAH model, we found that hypoxia induced PAH and increased pulmonary arterial wall thickness in wild type (WT) mice. In contrast, mice deficient in TLR4 spontaneously developed PAH and increased pulmonary arterial wall thickness, which were not further enhanced by hypoxia. Consistent with the development of PAH in TLR4−/− mice, echocardiography confirmed right ventricular hypertrophy and decreased pulmonary arterial acceleration time compared to WT mice. Above results suggest that TLR4 plays an important role in maintaining normal pulmonary vasculature, and that hypoxia induces PAH via TLR4. In short, these studies demonstrate an important role of TLR4 in maintaining normal pulmonary vasculature and in the development of hypoxia-induced PAH. Genetic ablation of TLR4 and inhibition of TLR4 expression by hypoxia induces PASMC proliferation and vascular remodeling, in association with redox signaling in the pathogenesis of PAH. Disclosures: No relevant conflicts of interest to declare.


2012 ◽  
Vol 15 (2) ◽  
pp. 111 ◽  
Author(s):  
Yang Hyun Cho ◽  
Tae-Gook Jun ◽  
Ji-Hyuk Yang ◽  
Pyo Won Park ◽  
June Huh ◽  
...  

The aim of the study was to review our experience with atrial septal defect (ASD) closure with a fenestrated patch in patients with severe pulmonary hypertension. Between July 2004 and February 2009, 16 patients with isolated ASD underwent closure with a fenestrated patch. All patients had a secundum type ASD and severe pulmonary hypertension. Patients ranged in age from 6 to 57 years (mean � SD, 34.9 � 13.5 years). The follow-up period was 9 to 59 months (mean, 34.5 � 13.1 months). The ranges of preoperative systolic and pulmonary arterial pressures were 63 to 119 mm Hg (mean, 83.8 � 13.9 mm Hg) and 37 to 77 mm Hg (mean, 51.1 � 10.1 mm Hg). The ranges of preoperative values for the ratio of the pulmonary flow to the systemic flow and for pulmonary arterial resistance were 1.1 to 2.7 (mean, 1.95 � 0.5) and 3.9 to 16.7 Wood units (mean, 9.8 � 2.9 Wood units), respectively. There was no early or late mortality. Tricuspid annuloplasty was performed in 14 patients (87.5%). The peak tricuspid regurgitation gradient and the ratio of the systolic pulmonary artery pressure to the systemic arterial pressure were decreased in all patients. The New York Heart Association class and the grade of tricuspid regurgitation were improved in 13 patients (81.2%) and 15 patients (93.7%), respectively. ASD closure in patients with severe pulmonary hypertension can be performed safely if we create fenestration. Tricuspid annuloplasty and a Cox maze procedure may improve the clinical result. Close observation and follow-up will be needed to validate the long-term benefits.


2011 ◽  
Vol 301 (5) ◽  
pp. H1810-H1818 ◽  
Author(s):  
Lian Tian ◽  
Steven R. Lammers ◽  
Philip H. Kao ◽  
Mark Reusser ◽  
Kurt R. Stenmark ◽  
...  

Understanding how arterial remodeling changes the mechanical behavior of pulmonary arteries (PAs) is important to the evaluation of pulmonary vascular function. Early and current efforts have focused on the arteries' histological changes, their mechanical properties under in vitro mechanical testing, and their zero-stress and no-load states. However, the linkage between the histology and mechanical behavior is still not well understood. To explore this linkage, we investigated the geometry, residual stretch, and histology of proximal PAs in both adult rat and neonatal calf hypoxic models of pulmonary hypertension (PH), compared their changes due to chronic hypoxia across species, and proposed a two-layer mechanical model of artery to relate the opening angle to the stiffness ratio of the PA outer to inner layer. We found that the proximal PA remodeling in calves was quite different from that in rats. In rats, the arterial wall thickness, inner diameter, and outer layer thickness fraction all increased dramatically in PH and the opening angle decreased significantly, whereas in calves, only the arterial wall thickness increased in PH. The proposed model predicted that the stiffness ratio of the calf proximal PAs changed very little from control to hypertensive group, while the decrease of opening angle in rat proximal PAs in response to chronic hypoxia was approximately linear to the increase of the stiffness ratio. We conclude that the arterial remodeling in rat and calf proximal PAs is different and the change of opening angle can be linked to the change of the arterial histological structure and mechanics.


1995 ◽  
Vol 6 (1) ◽  
pp. 57-64 ◽  
Author(s):  
Agostino Gnasso ◽  
Arturo Pujia ◽  
Concetta Irace ◽  
Pier Luigi Mattioli

Stroke ◽  
1997 ◽  
Vol 28 (10) ◽  
pp. 1972-1980 ◽  
Author(s):  
Eva Stensland-Bugge ◽  
Kaare H. Bønaa ◽  
Oddmund Joakimsen

ESC CardioMed ◽  
2018 ◽  
pp. 2507-2511 ◽  
Author(s):  
Daniela Calderaro ◽  
Luis Felipe Prada ◽  
Rogério Souza

The diagnosis of pulmonary hypertension (PH) relies on the haemodynamic criterion of mean pulmonary arterial pressure greater than or equal to 25 mmHg, assessed by right heart catheterization. The scope of this chapter is to discuss the key elements of clinical assessment of PH patients and the decision process to indicate right heart catheterization. Investigation must get through all the possible causes of PH according to their probability and frequency in the population. Echocardiography is the most important non-invasive test as an indicator for further diagnostic evaluation. Patients who are eligible for right heart catheterization should always be referred to PH centres, where technical skills and standardized procedures will enable maximal reliability of haemodynamic measurement. In the reference centre, a multidisciplinary team will discuss clinical and haemodynamic data, to propose the best therapeutic and follow-up schedule.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Gargani ◽  
V Codullo ◽  
P Argiento ◽  
A Moreo ◽  
F Pieri ◽  
...  

Abstract Background Patients with systemic sclerosis (SSc) are at risk of developing pulmonary arterial hypertension (PAH), which is often diagnosed late when the benefits of vasoactive therapies are limited. The concept of exercise pulmonary hypertension as a possible transitional phase anticipating resting PAH has been assessed in several pathologies, but has not been endorsed by the latest European Guidelines, because not supported by sufficient data. Purpose To evaluate whether PASP values at rest and at peak exercise, estimated at echocardiography, could be predictors of further development of PAH. Methods Four hundred and twenty-nine SSc patients without a previous diagnosis of PAH, enrolled at 4 referral Centres, underwent standard exercise Doppler echocardiography with PASP estimation at rest and at peak stress. Patients were then followed-up to assess the development of PAH, as diagnosed by a complete diagnostic work-up including right heart catheterization. PAH was defined by pre-capillary pulmonary hypertension (mean pulmonary artery pressure ≥25 mmHg with pulmonary arterial wedge pressure ≤15 mmHg), without significant interstitial lung disease and/or left heart disease. Results During the median follow-up of 75 months (IQR 29–114), 16 patients developed PAH. A combined cut-off of ≥24 mmHg as resting PASP and ≥40 as peak PASP was identified as the best predictor of further development of PAH (see Figure). Both resting PASP and peak PASP were predictors of PAH at univariate analysis (resting PASP OR 1.13, 95% C.I. 1.07–1.19, p<0.0001; peak PASP OR 1.13, 95% C.I. 1.07–1.18, p<0.0001). At multivariate analysis, only peak PASP was independently associated to PAH development (OR 1.13, 95% C.I. 1.04–1.18, p<0.001). Only one patient among those with resting PASP <24 mmHg and peak PASP <40 mmHg (34.7% of the total population) developed PAH during the follow-up (after 10 years from normal exercise Doppler echocardiography). Kaplan-Meier curves Conclusions Exercise increase in PASP is an independent predictor of later development of PAH in SSc. An increase in exercise PASP is frequent and is not necessarily associated with a later development of PAH, whereas the very high negative predictive value of a normal PASP both at rest and at peak exercise can be used in the clinical practice to confidently rule out about one third of patients. Acknowledgement/Funding Italian Ministry of Health (Ricerca Finalizzata 2011-2012)


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