Long-term improvement of pulmonary haemodynamics and exercise capacity in patients with pulmonary hypertension and hypoventilation following NIPPV and influence of NIPPV adherence

Author(s):  
Matthias Held ◽  
Katharina Schuh ◽  
Sabine Karl ◽  
Johanna Walthelm ◽  
Stefan Baron ◽  
...  
2017 ◽  
Vol 50 (4) ◽  
pp. 1700465 ◽  
Author(s):  
Athénaïs Boucly ◽  
Vincent Cottin ◽  
Hilario Nunes ◽  
Xavier Jaïs ◽  
Abdelatif Tazi ◽  
...  

Studies reporting the effects of modern strategies with pulmonary arterial hypertension (PAH)-targeted therapies in sarcoidosis-associated pulmonary hypertension (S-APH) are limited.Clinical and haemodynamic data from newly diagnosed patients with severe S-APH (mean pulmonary artery pressure (mPAP) >35 mmHg or mPAP 25–35 mmHg with cardiac index <2.5 L·min−1·m−2) were collected from the French Pulmonary Hypertension Registry between 2004 and 2015.Data from 126 patients with severe S-APH were analysed (mean±sd age 57.5±11.6 years, 74% radiological stage IV). 97 patients (77%) received PAH-targeted therapy and immunosuppressive therapy was initiated or escalated in 33 patients at the time of pulmonary hypertension diagnosis. Four months after PAH-targeted therapy initiation, mean±sd pulmonary vascular resistance decreased from 9.7±4.4 to 6.9±3.0 Wood units (p<0.001), without significant improvement in exercise capacity. Among the 11 patients treated only with immunosuppressive therapy, a haemodynamic improvement was observed in four patients, including two with compressive lymph nodes. After a median follow-up of 28 months, 39 patients needed PAH-targeted therapy escalation, nine underwent lung transplantation and 42 had died. Survival at 1, 3 and 5 years was 93%, 74% and 55%, respectively.PAH-targeted therapy improved short-term pulmonary haemodynamics in severe S-APH without change in exercise capacity. Immunosuppressive therapy improved haemodynamics in selected patients. Pulmonary hypertension in sarcoidosis remains associated with a poor prognosis.


2012 ◽  
Vol 2012 ◽  
pp. 1-16 ◽  
Author(s):  
Adil Shujaat ◽  
Abubakr A. Bajwa ◽  
James D. Cury

The development of pulmonary hypertension in COPD adversely affects survival and exercise capacity and is associated with an increased risk of severe acute exacerbations. Unfortunately not all patients with COPD who meet criteria for long term oxygen therapy benefit from it. Even in those who benefit from long term oxygen therapy, such therapy may reverse the elevated pulmonary artery pressure but cannot normalize it. Moreover, the recent discovery of the key roles of endothelial dysfunction and inflammation in the pathogenesis of PH provides the rationale for considering specific pulmonary vasodilators that also possess antiproliferative properties and statins.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Kimura ◽  
T Kohno ◽  
T Kawakami ◽  
T Shinya ◽  
T Hiraide ◽  
...  

Abstract Introduction There has been increasing evidence of the efficacy of balloon pulmonary angioplasty (BPA) in improving the hemodynamics, exercise capacity, and biomarkers of patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, there is no consensus on the adjustment of home oxygen therapy (HOT) and pulmonary hypertension (PH)-specific medications after BPA in patients with CTEPH. Purpose We aimed to examine the current status of the de-escalation/discontinuation of HOT and PH-specific medications post-BPA, and clarify its effect on subsequent hemodynamics, biomarkers, and long-term clinical outcomes. Methods and results From November 2012 to July 2019, 134 consecutive CTEPH patients who underwent BPA at a single university hospital were enrolled (age; 63.6±13.4 years, female; n=87 [64.9%], WHO functional class [WHO-FC] II/III/IV; 33/92/9). Hemodynamic data, functional capacity (6-minute walk distance and WHO-FC), biomarkers (brain natriuretic peptide [BNP] and high-sensitivity troponin T [hs-TropT]), and respiratory function were evaluated at baseline, immediately and 1 year post-BPA. Clinical outcomes (all-cause death and heart failure [HF] admission) were also assessed during the follow up period. The total number of sessions was 6.3±2.0, and the number of target vessels was 14.3±2.0. Mean pulmonary arterial pressure decreased from 37.8±11.2 to 20.4±5.1 mmHg 1-year after BPA (p&lt;0.01). The proportion of patients who required HOT (at rest or on exertion) and combination medical therapy (≥2 PH-specific medications) decreased 1 year post-BPA (from 59.0% to 7.5%, and from 41.8% to 10.4%, respectively; Figure). Among 79 patients who required HOT during daytime, 64 patients (81.0%) discontinued HOT just after BPA completion. Among 56 patients who required combination medical therapy, 29 (51.8%) discontinued combination therapy. Baseline factors influencing the continuation of HOT and combination medical therapy post-BPA were almost identical (i.e. lower exercise capacity and pulmonary diffusion capacity, and worse hemodynamics). Results showed that discontinuation of HOT and combination medical therapy did not affect the maintenance of improved hemodynamics and levels of BNP and hs-TropT, and no adverse clinical outcomes (all-cause death and HF hospitalization) were observed during 1 year post-BPA. Conclusions Most CTEPH patients discontinued HOT and PH-specific combination medical therapy after BPA, which was not associated with the deterioration of hemodynamics, functional capacity, or biomarkers. No adverse long-term outcomes were observed. De-escalation/discontinuation of HOT and PH-specific combination medical therapy after BPA is feasible and safe for patients with CTEPH. De-escalation of HOT and medical therapy Funding Acknowledgement Type of funding source: None


Sign in / Sign up

Export Citation Format

Share Document