scholarly journals A Retrospective Study on the Efficacy of PAH-targeted Therapy in Patients with Severe Pulmonary Hypertension in Chronic Lung Disease

2020 ◽  
Author(s):  
li jieying ◽  
sun zhang ◽  
haiming Chen ◽  
wenliang Guo ◽  
chunli Liu ◽  
...  

Abstract Background Using PAH-targeted therapy in severe pulmonary hypertension (PH) patients with chronic lung disease (CLD) is controversial as the pulmonary vasodilation could worsen hypoxemia. Objective To investigate the efficacy of patients with severe PH-CLD to PAH targeted therapy. Methods We retrospectively enrolled patients with severe PH-CLD in our study. Each patient underwent laboratory indicators and transthoracic echocardiography at baseline and after the therapy, and survival after therapy were also evaluated. Results After PAH targeted therapy, NT - proBNP significantly decreased (1043.10(236.75-2697.50) pg/ml vs 287.10(21.00-555.90) pg/ml, p=0.035), whereas PaO2 and the oxygenation index in blood gas analysis significantly increased(59.18± 30.12 mmHg vs 74.35(61.63-163.95)mmHg, p=0.010 and 177.21± 84.38 mmHg vs 286.22± 165.13 mmHg, p=0.035, respectively). But the difference at 1-,3- years survival rates between the two groups was not statistically significant (82.82% and 66.26% vs 75.87% and 20.23%, respectively, p=0.094). Multivariate regression analysis identified baseline mean right atrial pressure values and cardiac index as significant predictors of survival of patients with severe PH-CLD. Conclusions PAH targeted therapy may have benefit in patients with severe PH-CLD and didn’t worsen their hypoxemia. Right ventricular function might have an important impact on the survival of patients.

2018 ◽  
Vol 4 (2) ◽  
pp. 00124-2017 ◽  
Author(s):  
Simon Bax ◽  
Charlene Bredy ◽  
Aleksander Kempny ◽  
Konstantinos Dimopoulos ◽  
Anand Devaraj ◽  
...  

European Respiratory Society (ERS) guidelines recommend the assessment of patients with interstitial lung disease (ILD) and severe pulmonary hypertension (PH), as defined by a mean pulmonary artery pressure (mPAP) ≥35 mmHg at right heart catheterisation (RHC). We developed and validated a stepwise echocardiographic score to detect severe PH using the tricuspid regurgitant velocity and right atrial pressure (right ventricular systolic pressure (RVSP)) and additional echocardiographic signs.Consecutive ILD patients with suspected PH underwent RHC between 2005 and 2015. Receiver operating curve analysis tested the ability of components of the score to predict mPAP ≥35 mmHg, and a score devised using a stepwise approach. The score was tested in a contemporaneous validation cohort. The score used “additional PH signs” where RVSP was unavailable, using a bootstrapping technique.Within the derivation cohort (n=210), a score ≥7 predicted severe PH with 89% sensitivity, 71% specificity, positive predictive value 68% and negative predictive value 90%, with similar performance in the validation cohort (n=61) (area under the curve (AUC) 84.8% versus 83.1%, p=0.8). Although RVSP could be estimated in 92% of studies, reducing this to 60% maintained a fair accuracy (AUC 74.4%).This simple stepwise echocardiographic PH score can predict severe PH in patients with ILD.


Author(s):  
Vania Cecilia Prudencio Ribera ◽  
Agueda Aurtenetxe Pérez ◽  
María Lorena Coronel ◽  
Virginia Luz Pérez González ◽  
Francisco Javier Mazo Etxaniz ◽  
...  

2018 ◽  
Vol 46 (6) ◽  
pp. 657-663 ◽  
Author(s):  
Satoshi Suzuki ◽  
Hajime Togari ◽  
Jim L. Potenziano ◽  
Michael D. Schreiber

AbstractObjective:To analyze data from a registry of Japanese neonates with hypoxic respiratory failure associated with pulmonary hypertension (PH) to compare the effectiveness of inhaled nitric oxide (iNO) in neonates born <34 weeks vs. ≥34 weeks gestational age (GA).Materials and methods:iNO was administered according to approved Japanese product labeling. Study data were collected before iNO administration and at predefined intervals until discontinuation.Results:A total of 1,114 neonates were included (n=431, <34 weeks GA; n=675, ≥34 weeks GA; n=8, missing age data). Mean decrease from baseline oxygenation index (OI) was similar in both age groups. OI reduction was more pronounced in the <34 weeks subgroups with baseline OI ≥25. Survival rates were similar in the <34 weeks GA and ≥34 weeks GA groups stratified by baseline OI (OI<15, 89% vs. 93%; 15≤OI<25, 85% vs. 91%; 25≤OI≤40, 73% vs. 79%; OI>40, 64% vs. 66%).Conclusion:iNO improved oxygenation in preterm neonates as effectively as in late preterm and term neonates, without negative impact on survival. If clinically significant PH is present, as measured by pulse oximetry or echocardiography, a therapeutic trial of iNO might be indicated for preterm neonates.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Jeong-Hoon Kim ◽  
Duk-Hyun Kang ◽  
Jong-Young Lee ◽  
Jong-Min Song ◽  
Tae-Jin Yun ◽  
...  

The benefits of surgical closure has been unclear in adult atrial septal defect (ASD) with severe pulmonary hypertension (PHT), and we tried to evaluate improvement of PHT and long-term survival after surgical closure compared to medical follow-up. Methods: From 1996 to 2006, we included a total of 71 adult ASD patients (age; 43±15 years) with severe PHT documented by echocardiography. The inclusion criteria were defined as ASD diameter > 15 mm, enlarged right ventricle, and the baseline peak velocity of tricuspid regurgitation (TR) ≥ 4.0 m/sec. We excluded 5 patients with Eisenmenger syndrome documented by cardiac catheterization. Surgical closure was performed on 55 patients (OP group) and the remaining 16 patients were followed up medically (MED group). The improvement of PHT was defined as TR velocity ≤ 3.5 m/sec on follow-up echo. Results: Baseline characteristics and clinical results were compared between the two groups in table . There were no significant differences in terms of gender, ASD diameter, cardiac rhythm, and TR velocity, but the MED group was significantly older. During follow-up of 46±33 months, there were 5 deaths in the MED group and no operative or late death in the OP group, and the 5-year actuarial survival rate of the OP group was significantly higher than the MED group (58±15%, p<0.05). On subgroup analysis according to age, the OP group showed significantly better survival rates than the MED group (p<0.05) in elderly patients (age > 50). In the OP group, TR velocity was significantly decreased from 4.5±0.4 to 3.0±0.7 m/sec on follow-up echo, and improvement of PHT was observed in 47 (85%) patients. On multivariate analysis, female gender and lower baseline TR velocity were the significant independent predictors of improved PHT after surgery. Conclusions: In adult ASD with severe PHT, surgical closure can be safely performed and improve PHT effectively. Especially in elderly patients, ASD closure is significantly related with the better survival rates.


PEDIATRICS ◽  
2014 ◽  
Vol 134 (1) ◽  
pp. e274-e278 ◽  
Author(s):  
D. J. Ferdman ◽  
E. B. Rosenzweig ◽  
W. A. Zuckerman ◽  
U. Krishnan

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