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Purpose During the COVID-19 pandemic, most of the health care systems suspended their non-urgent activities. This included the cancellation of consultations for patients with rare diseases, such as severe pulmonary hypertension (PH), accompanied by potential medication shortage and loss of follow-up. Thus, the aim of the study was to evaluate PH patient health status evolution, access to health care and mental health experience during the early phase of the pandemic. Methods We conducted an internet patient survey, available in 16 languages, between 22/05/2020 and 28/06/2020. The survey included questions corresponding to demographic, COVID-19 and PH related information. Results 1073 patients (or relatives, 27%) from 52 countries all over the world participated in the survey. Seventy-seven percent (77%) of responders reported a diagnosis of pulmonary arterial hypertension (PAH) and 15% of chronic thromboembolic PH (CTEPH). The COVID-19 related events were few: only 1% of all responders reported a diagnosis of COVID-19. However, the PH related events during the early phase of the pandemic seemed far more frequent with 8% of patients reporting health deterioration possibly related to PH, and 4% requiring hospitalization for PH care. 11% of the patients reported difficulties to have access to their PH expert center, and the reported rate of interruption of treatment due to shortage of medication was 3%. Both general practitioners and specialized PH centers were contacted by equal proportions of patients to seek COVID-19 related information. 67% of the participants reported anxiety or depression. Conclusion Although COVID-19 incidence in PH patients was low during the early phase of the pandemic, PH related problems occurred frequently as the pandemic progressed with difficulties to have access to specialized care. Primary health care as well as to information on the impact of COVID-19 on people with PH. In addition to specialized care, primary health care was considered to be of importance by PH patients. Further studies are needed to evaluate the long-term consequences of COVID-related PH care disruption. The evaluation of long-term consequences of COVID related care disruption in PH will require further studies.

Combined diffusion capacity of the lung for carbon monoxide (DLco) and nitric oxide (DLno) measurements allow for the estimation of pulmonary capillary blood volume (Vc) and alveolar membrane diffusion (Dm). The clinical usefulness of these measurements in pulmonary hypertension (PH) is unclear.Combined DLco and DLno were measured in 290 consecutive patients with precapillary PH (pulmonary arterial hypertension (PAH), n = 153; pulmonary veno-occlusive disease (PVOD), n = 33; and chronic thromboembolic pulmonary hypertension (CTEPH), n = 104). Clinical correlates of Vc and Dm were assessed in a sub-group of PAH patients without comorbidities.PVOD patients compared with PAH and CTEPH patients displayed the lowest values of Vc (29.4 ± 16.8 ml vs 56.3 ± 26.5 ml vs 56.9 ± 26.2 ml, p0.01, respectively) and Dm (27.7 ± 11.6 ml/mm Hg/min vs 43.4 ± 14.8 ml/mm Hg/min vs 44.7 ± 17.7 ml/mm Hg/min, p0.01, respectively). The DLno/DLco ratio was highest in the PVOD group (5.82 ± 2.04 vs 4.95 ± 1.31 vs 5.16 ± 1.58, p0.05). In a sub-set of 69 PAH patients without comorbidities, Vc and Dm correlated significantly with functional capacity (6-minute walking distance, oxygen consumption) and New York Heart Association Functional Classification but not with invasive hemodynamics. Only Dm was significantly associated with survival. On receiver operating characteristic curve analysis, Vc, Dm, and DLno/DLco were not superior to DLco for discriminating PVOD from PAH or CTEPH.PVOD patients display higher values of the DLno/DLco ratio compared with PAH and CTEPH, suggesting proportionally greater reduction in Vc relative to Dm. However, partitioning of diffusion failed to be more clinically relevant than conventional DLco for detection of PVOD.

Purpose Antibody mediated rejection (AMR) is increasingly recognized as a damaging process conducing lung transplant (LTX) recipients to chronic lung allograft dysfunction and severely impacting the prognostic of the patients. Until now, few treatments are effective for AMR. The aim of the study was to assess retrospectively the effect of a treatment combining plasma exchange therapy (PFX), intravenous immunoglobulin (IVIg) and rituximab (RTX) on graft function in patients with AMR. Methods AMR was diagnosed according to combination of criteria including allograft dysfunction, pathological findings (complement deposition, AFOP or DAD) and positive serology for anti-HLA donor specific antibodies. Statistical analysis were performed using paired t test or ANOVA. Results Between 1/1/2013 and 31/12/2017, 40 lung transplant recipients were treated for AMR (94/2/2/2% double LTX, single LTX, heart-LTX, liver-LTX; 42% COPD, 21% CF, 25% ILD, 6% PAH, 6% other) in UZ Leuven, Belgium. Median duration between LTX and AMR was 1205 days (13-6086). A mean of 6±1 plasma exchanges was performed per patient. At follow up, 6 (15%) patients were re-transplanted and 18 (45%) died. Lost of graft (death plus redo LTX) was 62% at 1 year after ending PFX. FEV1 declined the 6 months before AMR treatment but stopped to decline significantly during the 6 months after combination therapy (627±537ml and 53±434ml, p Conclusion Survival after AMR is poor. PFX, IVIg and RTX may stabilize lung function in good responders.