Impact of targeted pulmonary arterial hypertension therapies in severe pulmonary hypertension in chronic lung diseases.

Research questions: Patients with severe pulmonary hypertension associated with chronic lung disease have a poor prognosis. Targeted pulmonary arterial hypertension therapies might improve exercise capacity and outcome, but there are no guidelines on treatments which are not recommended because of an unproven benefit, with discordant results from few studies in this context. The aim of our study was to evaluate targeted pulmonary arterial hypertension therapies for severe group 3 pulmonary hypertension patients. Study design and methods: We conducted an observational retrospective monocentre study on patients with severe group 3 pulmonary hypertension diagnosed on right heart catheterisation treated with targeted therapies. Primary outcome was an improvement of the distance on 6-min walk test of ≥30 m. Secondary end-points included changes in haemodynamics (pulmonary vascular resistance (PVR) and mean pulmonary arterial pressure (mPAP)) and identification of potential predictive factors of therapeutic response. Results: 139 patients were enrolled. Most patients had monotherapy with phosphodiesterase 5 inhibitors (n=128; 92%). Mean change in 6-min walk distance was +1.5 m after treatment (p=0.59). Forced expiratory volume in 1 s and forced vital capacity were not predictive factors for response. We found a significant improvement of PVR and mPAP of -1.0 Wood Units (p<0.001) and -4 mmHg (p<0.001), respectively, under treatment. 18% of patients had to withdraw treatment for intolerance. Treatment duration <3 months was associated with poor survival (hazard ratio 2.75, p=0.0005). Conclusion: Oral targeted pulmonary arterial hypertension therapies do not improve exercise capacity in patients with severe pulmonary hypertension associated with chronic lung disease, but could improve haemodynamic parameters.

Chest computed tomography improvement in patients with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: Early report.

RATIONALE AND OBJECTIVES: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have revolutionised the treatment of cystic fibrosis (CF). Chest computed tomography (CT) is key in the diagnosis and follow-up of anatomical damage to the lungs. Our study aimed to evaluate changes on lung CT scans of patients with CF after receiving elexacaftor-tezacaftor-ivacaftor (ETI) therapy for one year. MATERIALS AND METHODS: We conducted a retrospective, observational, single-centre study between 2018 and 2021 on adult patients with CF administered ETI. We reviewed chest CT scans before and at least one year after starting ETI. The Brody-II score (BSII) was measured by two experienced radiologists who were blinded to the treatment. Paranasal sinus CT scans and clinical and functional data were also compared. Wilcoxon tests were used to compare differences, and Spearman's correlation coefficient was used to evaluate changes in forced expiratory volume in one second (FEV1) and total BSII. RESULTS: In the period, 63 patients were given ETI, and 12 met the criteria for analysis. The inter-observer reproducibility of BSII was satisfactory (intraclass correlation coefficient = 0.83, 95% confidence interval 0.57-0.91). The BSII decreased after one year of treatment (-18 ±â€¯16, p = 0.002) due to lower mucous plugging (-7 ±â€¯4, p < 0.001) and peribronchial thickening (-9 ±â€¯10, p = 0.002) scores. Bronchial, parenchymal, and hyperinflation scores were unchanged. Clinical and functional parameters were significantly improved, except for total lung capacity. The correlation between ΔFEV1 and Δtotal BSII was strong (r = 0.88, p < 0.001). The paranasal sinus CT score significantly improved with ETI treatment. CONCLUSIONS: ETI decreased pulmonary and sinus morphological abnormalities after one year of treatment.

Pulmonary cement embolism complicating percutaneous kyphoplasty: A case report.

BACKGROUND: Vertebral cement augmentation procedures, as kyphoplasty (KP) or percutaneous vertebroplasty (PVP), are commonly used for the management of pain of the vertebral column usually due to fractures related to traumatic injury, osteoporosis or metastatic lesion. It is a useful and safe technique with few complications. Among them, symptomatic pulmonary cement embolism (PCE) can happened, even rarely described in the literature, leading to severe cardio-respiratory manifestations depending on the location and size of the cement emboli. CASE PRESENTATION: A 55 yo woman presented with atypical chest pain and presyncope three weeks after a motor vehicle accident resulting in an L1 compression fracture treated with kyphoplasty. She was hemodynamically stable. Blood tests showed D-Dimer 0.29 µg/mL, troponin <5ng/mL, Brain Natriuretic Protein 14 ng/mL and a PaO2 of 99 mmHg on arterial blood gas analysis. A computed tomography scan of chest showed linear hyperdense foreign bodies in two segmental pulmonary arteries at the level of middle lobe and right lower lobe compatible with pulmonary cement embolism. Anticoagulation with rivaroxaban for sixth months resulted in resolution of symptoms. CONCLUSIONS: In this case, the pulmonary cement embolism occurred after kyphoplasty, which is associated with less risk of PCE than vertebroplasty. The procedure was done under biplanar fluoroscopy and no leakage of cement was noted, which would raise suspicion for CPE. Repeat imagine after this procedure is not routinely done. This case demonstrates that systematic imaging post procedure should be considered.An anticoagulation with rivaroxaban seems to be effective in our patient.