Search

Current guidelines recommend oxygen (O2) supplementation in patients with pulmonary hypertension (PH), despite scarce data on long-term O2 therapy (LTOT). The aim of this prospective, randomized, controlled trial was to investigate the effect of LTOT in patients with precapillary PH on exercise capacity, clinical parameters and hemodynamics. Patients with precapillary PH under stable therapy and O2 desaturations at rest and/or during exercise were randomized to receive LTOT (≥ 16 h/day) or no O2 (control group) for 12 weeks. The control group was offered LTOT after 12 weeks. The primary endpoint changes of 6-minute walking distance (6MWD) from baseline to 12 weeks was hierarchically tested: (1) pre-post primary and secondary intervention (2) intervention vs. control group. Secondary endpoints included changes in clinical parameters. Twenty patients were randomized (women n = 14, age 67 ± 11.4 years, mean pulmonary arterial pressure 39.7 ± 12.5 mmHg, 70% functional class III). 6MWD significantly improved by 42.2 ± 34.20 m (p = 0.003) within 12 weeks LTOT. The intervention group significantly improved in 6MWD (38.9 ± 33.87 m) compared to the control group (− 12.3 ± 21.83 m, p = 0.015). No consistent between-group differences in other parameters were found. LTOT was well tolerated and led to significant improvement of 6MWD. The effect of LTOT should be investigated in larger controlled-trials. [ABSTRACT FROM AUTHOR]

The PEGASUS study is the first multicentric and prospective assessment of the safety of air travel flying in pulmonary hypertension (PH) (NCT03051763). Data of air travel from 60 patients with PH was available. No severe adverse events occurred. Nine patients self‐reported mild adverse events during flight (13%), while after landing, 12 patients reported events (20%). Solely one patient (2%) had an adverse event leading to medical consultation. In patients with PH and World Health Organization functional classes II and III, air travel was safe. [ABSTRACT FROM AUTHOR]

Objective: The evidence‐based DETECT pulmonary arterial hypertension (PAH) algorithm is frequently used in patients with systemic sclerosis (SSc) to help clinicians screen for PAH by using noninvasive data to recommend patient referral to echocardiography and, if applicable, for a diagnostic right‐sided heart catheterization. However, the hemodynamic definition of PAH was recently updated in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines. The performance of DETECT PAH in identifying patients with a high risk of PAH according to this new definition was assessed. Methods: In this post hoc analysis of DETECT, which comprised 466 patients with SSc, the performance of the DETECT PAH algorithm in identifying patients with a high risk of PAH as defined in the 2022 ESC/ERS guidelines (mean pulmonary arterial pressure [mPAP] >20 mm Hg, pulmonary capillary wedge pressure [PCWP] ≤15 mm Hg, and pulmonary vascular resistance >2 Wood units) was assessed using summary statistics and was descriptively compared to the known performance of DETECT PAH as defined in 2014, when it was developed (mPAP ≥25 mm Hg and PCWP ≤15 mm Hg). Results: The sensitivity of DETECT PAH in identifying patients with a high risk of PAH according to the 2022 ESC/ERS definition was lower (88.2%) compared to the 2014 definition (95.8%). Specificity improved from 47.8% to 50.8%. Conclusion: The performance of the DETECT algorithm to screen for PAH in patients with SSc is maintained when PAH is defined according to the 2022 ESC/ERS hemodynamic definition, indicating that DETECT remains applicable to screen for PAH in patients with SSc. [ABSTRACT FROM AUTHOR]

Objectives Pulmonary arterial hypertension (PAH) occurs in various connective tissue diseases (CTDs). We sought to assess contemporary treatment patterns and survival of patients with various forms of CTD-PAH. Methods We analysed data from COMPERA, a European pulmonary hypertension registry, to describe treatment strategies and survival in patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types of CTD. All-cause mortality was analysed according to the underlying CTD. For patients with SSc-PAH, we also assessed survival according to initial therapy with endothelin receptor antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a combination of these two drug classes. Results This analysis included 607 patients with CTD-PAH. Survival estimates at 1, 3 and 5 years for SSc-PAH (n  = 390) were 85%, 59% and 42%; for SLE-PAH (n  = 34) they were 97%, 77% and 61%; for MCTD-PAH (n  = 33) they were 97%, 70% and 59%; for UCTD-PAH (n  = 60) they were 88%, 67% and 52%; and for other CTD-PAH (n  = 90) they were 92%, 69% and 55%, respectively. After multivariable adjustment, the survival of patients with SSc-PAH was significantly worse compared with the other conditions (P  = 0.001). In these patients, the survival estimates were significantly better with initial ERA–PDE5i combination therapy than with initial ERA or PDE5i monotherapy (P  = 0.016 and P  = 0.012, respectively). Conclusions Mortality remains high in patients with CTD-PAH, especially for patients with SSc-PAH. However, for patients with SSc-PAH, our results suggest that long-term survival may be improved with initial ERA–PDE5i combination therapy compared with initial monotherapy. [ABSTRACT FROM AUTHOR]

The nitric oxide (NO)–soluble guanylate cyclase (sGC)–cyclic guanosine monophosphate (cGMP) pathway plays a key role in the pathogenesis of pulmonary hypertension (PH). Targeted treatments include phosphodiesterase type 5 inhibitors (PDE5i) and sGC stimulators. The sGC stimulator riociguat is approved for the treatment of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). sGC stimulators have a dual mechanism of action, enhancing the sGC response to endogenous NO and directly stimulating sGC, independent of NO. This increase in cGMP production via a dual mechanism differs from PDE5i, which protects cGMP from degradation by PDE5, rather than increasing its production. sGC stimulators may therefore have the potential to increase cGMP levels under conditions of NO depletion that could limit the effectiveness of PDE5i. Such differences in mode of action between sGC stimulators and PDE5i could lead to differences in treatment efficacy between the classes. In addition to vascular effects, sGC stimulators have the potential to reduce inflammation, angiogenesis, fibrosis and right ventricular hypertrophy and remodelling. In this review we describe the evolution of treatments targeting the NO–sGC–cGMP pathway, with a focus on PH. [ABSTRACT FROM AUTHOR]

The number of adults with congenital heart defects (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary hypertension (PH), which may develop early in untreated CHD. Despite timely treatment of CHD, PH not infrequently persists or recurs in older age and is associated with significant morbidity and mortality.The revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart disease" is addressed only relatively superficial in these guidelines. Therefore, in the present article, this topic is commented in detail from the perspective of congenital cardiology., Competing Interests: Harald Kaemmerer: Unabhängig von diesem Manuskript Sponsorship and honoraria Janssen/Johnson & Johnson; Bristol Myers Squibb. Steering Board COMPERA International.Gerhard Paul Diller: Advisory consultancy work for Janssen/Johnson & Johnson.Ingo Dähnert: Proctortätigkeit für Occlutech und Medtronic, Studien für Janssen und Novartis, Advisory Board für Actelion/Janssen.Christina A. Eichstaedt: C. A. E. ist Miterfinderin des europäischen Patents (EP3507380) “Gene panel specific for pulmonary hypertension and its uses”. CAE hat von msd Vortragshonorare erhalten, unabhängig von dieser Arbeit.Andreas Eicken: Keine Interessenkonflikte.Annika Freiberger: Keine Interessenkonflikte.Sebastian Freilinger: Keine Interessenkonflikte.Ralf Geiger: Keine Interessenkonflikte.Matthias Gorenflo: Advisory Board für Janssen.Ekkehard Grünig: E. G. hat Honorare für Vorträge/Konsultationen von Bayer/MSD, Ferrer, GEBRO, GSK, Janssen und OMT erhalten. Forschungsförderung für klinische Studien wurde von Acceleron, Actelion, BayerHealthCare, MSD, Bellerophon, GossamerBio, Janssen, Novartis, OMT, Pfizer, REATE und United Therapeutics erhalten.Alfred Hager: Unabhängig von diesem Manuskript erhielt A. H. Reisekostenerstattungen von Actelion, Pfizer, GlaxoSmithKline, Lilly und OMT; Rednergelder von Encysive, Pfizer, Actelion, Medtronic, Schiller, GlaxoSmithKline, OMT, AOP Orphan und Janssen; Autorenvergütungen von Actelion; Beraterhonorare von Actelion, Bayer, Ethypharm und GlaxoSmithKline; er hält Aktien von Merck Inc., Merck KGaA, Johnson & Johnson, Pfizer und Abbott.Michael Huntgeburth: Honorare Advisory-Board Janssen-Cilag, Johnson & Johnson.Ann-Sophie Kaemmerer-Suleiman: Keine Interessenkonflikte.Rainer Kozlik-Feldmann: Teaching-Kurse für Occlutech, Proktortätigkeit für Abbott (Amplatzer).Astrid E. Lammers: CEC chair and advisory consultancy work for Janssen/Johnson & Johnson.Nicole Nagdyman: Keine Interessenkonflikte.Sebastian Michel: Keine Interessenkonflikte.Kai Helge Schmidt: Vortragshonorare von Janssen, MSD und Abbott.Anselm Uebing: Keine Interessenkonflikte.Fabian von Scheidt: Keine Interessenkonflikte.Christian Apitz: Honorare für Vortragstätigkeit und Advisory Board für Janssen., (Thieme. All rights reserved.)