INTRODUCTION:ESC/ERS guidelines recommend risk stratification of prevalent patients with pulmonary arterial hypertension (PAH) using noninvasive parameters, whereas right heart haemodynamic parameters are left to the clinician's discretion if deemed necessary. The study aimed to define the possible contribution of invasive haemodynamic parameters in predicting both the risk of death from all causes and the risk of clinical worsening (CW) in patients with PAH categorized at follow-up by the noninvasive ESC/ERS 4-strata risk stratification model. METHODS:We evaluated incident patients with PAH enrolled in 11 Italian centres between 2005 and 2021 who had a first follow-up right heart catheterization within 6-12 months of diagnosis. In each noninvasive risk category, patients were subsequently stratified in a subgroup with a good haemodynamic profile if stroke volume index was ⩾38 mL/m2 and right atrial pressure was <8 mmHg and a subgroup with a poor haemodynamic profile if stroke volume index <38 ml/m2 and/or right atrial pressure ⩾8 mmHg. Median follow-up was 3.7 years (interquartile range 1.2-6.8) months. RESULTS:Among low-risk patients (n = 162) survival was similar, but the CW rate was better in the good haemodynamic compared with the poor haemodynamic subgroup (P = .033). Among patients at intermediate-low risk (n = 240), both survival and CW rates were significantly better in the good haemodynamic subgroup compared with the poor haemodynamic subgroup (P = .028 and P = .011, respectively). Among patients at intermediate-high risk (n = 339), the CW rate was similar but survival was significantly better in the good haemodynamic than in the poor haemodynamic subgroup (P = .015). In the high-risk group, only 1 out of 28 patients had a good haemodynamic profile. CONCLUSION:In prevalent patients with PAH, a good haemodynamic profile predicts better survival in intermediate-risk patients and, importantly, a lower CW rate in low-risk patients.

BACKGROUND:The demographic shift of pulmonary arterial hypertension (PAH) patients, the expanding therapeutic armamentarium with new emerging drugs and the heterogenous experience in handling more complex therapies are making the management of PAH increasingly challenging. This project was developed to standardize PAH management across Italian centres and ensure timely access to the optimal therapeutic strategies. METHODS:A consensus document containing 10 statements, mainly focused on the use of parenteral prostanoids, was developed by eight experts in PAH during in-person and web-based meetings. Forty-six physicians were invited online to rate each statement, indicating their agreement, neutrality or disagreement. RESULTS:Forty physicians participated to the survey. There was strong agreement that age and comorbidities significantly influence the choice of PAH-targeted therapies. Agreement was also reached on the use of parenteral prostanoids in the following clinical scenarios: a) treatment-naive patients at high risk; b) newly diagnosed patients classified as intermediate-risk, with severe right ventricular dysfunction assessed invasively and c) prevalent high-risk patients, as defined by the 4-strata risk score. The use of parenteral prostanoids in selected newly diagnosed intermediate-risk patients with severe right ventricular dysfunction assessed by imaging achieved a mild agreement. Three statements did not meet the predefined threshold for consensus. CONCLUSIONS:These consensus statements are intended to support physicians in starting parenteral prostanoids, minimizing delays that could negatively affect patient prognosis.

Background and Aims The aim of this study was to evaluate whether echocardiography-derived phenotypes describing different degrees of right ventricular (RV) remodelling and dysfunction add prognostic information to that of current risk stratification tools in patients with pulmonary arterial hypertension (PAH) at first follow-up.Methods In 11 centres of the Italian Pulmonary Hypertension NETwork (IPHNET), data were prospectively collected from patients with PAH who underwent re-evaluation between 6 and 12 months after diagnosis. Echocardiographic variables were combined a priori to define four phenotypes representing different degrees of RV dilatation and right ventricular-pulmonary arterial (RV-PA) coupling: a mildly dilated right ventricle with preserved RV-PA coupling defined phenotype-1; a mildly dilated right ventricle with poor RV-PA coupling defined phenotype-2; a severely dilated right ventricle with preserved RV-PA coupling defined phenotype-3; a severely dilated right ventricle with poor RV-PA coupling, either with or without tricuspid regurgitation of moderate degree or more, defined phenotype-4. Patients were followed up for all-cause death for a median of 3.7 years.Results These echocardiographic phenotypes were present in all European Society of Cardiology/European Respiratory Society or REVEAL 2.0 risk groups except for the high-risk groups, which included only phenotype-3 and phenotype-4. In each risk group, RV phenotype-4 identified patients with a poorer prognosis; RV phenotype-1 identified patients with better survival in intermediate risk groups.Conclusions Echocardiography-derived phenotypes describing different degrees of RV remodelling and dysfunction provide prognostic information which is independent of and additional to the clinically defined risk in PAH patients at first follow-up.

Background: Idiopathic pulmonary fibrosis (IPF) is an incurable disease with limited therapeutic options and a poor prognosis. Current standard therapies are characterized by drugs or surgical strategies with limited effects, as they are either not curative or their use is restricted to a specific subset of the population. The aim of this scoping review is to evaluate the drugs currently under investigation in Phase II and Phase III trials and provide an overview of the mechanisms of new therapeutic strategies for IPF. Methods: The search strategy was conducted in accordance with PRISMA guidelines and included studies conducted on adults with IPF retrieved from the registered ClinicalTrials.gov database up to 31 December 2025. Results: Nineteen studies were included. The clinical trials investigate key signaling pathways and molecular targets, including MAPK, RhoA/ROCK, PDE4B/cAMP, Wnt/β-catenin, Hedgehog/SMO, IL-11/STAT3, and LPA/autotaxin, as well as extracellular receptors and mediators such as CSF1R, TBXA2R, and WISP1. Conclusions: Ongoing clinical research in IPF reflects a broad diversification of molecular targets; however, translational success remains limited. Current evidence suggests that biological complexity, pathway redundancy, and systemic constraints significantly restrict the clinical impact of single-target strategies. Future progress will likely depend on improved patient stratification, combination approaches, and biomarker-guided trial design rather than isolated pathway modulation.

BACKGROUND:Right ventricular functional adaptation to afterload is a major determinant of outcome in pulmonary arterial hypertension (PAH). We aimed to investigate if right ventricular-pulmonary artery (PA) coupling evaluated by the ratio of tricuspid annular plane systolic excursion (TAPSE) to systolic pulmonary artery pressure (sPAP) improves risk assessment scores for survival prediction. METHODS:A total of 677 consecutive patients with PAH (55% idiopathic) were prospectively enrolled with follow-up clinical, right heart catheterization, and echocardiographic evaluations within 12 months (interquartile range, 180-344 days) after initiation of targeted therapies in 11 Italian centers. European Society of Cardiology/European Respiratory Society guidelines-derived risk scores and REVEAL 2.0 (US Registry to Evaluate Early and Long-Term PAH Disease Management 2.0) risk scores were collected at baseline and follow-up. 254 consecutive patients with PAH retrospectively enrolled in a German reference center served as a validation cohort. RESULTS:A low-risk status at a median of 3.7 years (interquartile range, 1.2-6.8) follow-up was significantly associated with each unit (0.1 mm/mm Hg) increase in TAPSE/sPAP under targeted therapies (European Society of Cardiology/European Respiratory Society score: odds ratio, 1.78; P≤0.001; REVEAL 2.0 score: odds ratio, 1.43; P≤0.001). At follow-up, the TAPSE/sPAP ratio increased the prognostic information of each risk stratum of the European Society of Cardiology/European Respiratory Society risk score, except the highest risk stratum, with 0.5 mm/mm Hg, 0.35 mm/mm Hg, and 0.30 mm/mm Hg, from the lowest to the intermediate-high risk score, identified as the best cutoff value. TAPSE/sPAP ratio increased the prognostic information of the REVEAL 2.0 score at follow-up, with 0.35 mm/mm Hg identified as the best cutoff value to discriminate within a score of 5 to 8, with no added value for scores <5 and >8. These results were confirmed in the validation cohort. CONCLUSIONS:Assessment of right ventricular-PA coupling by the TAPSE/sPAP ratio in PAH improves risk assessment scores except in the lowest or most advanced stage of the disease.

Compared to immunocompetent individuals, solid organ transplant recipients (SOTRs) develop a weaker immune response to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and vaccination. Although anti-SARS-CoV-2 vaccines can prevent symptomatic and severe disease, the SOTR population remains at risk as long as SARS-CoV-2 continues to circulate. To protect transplanted patients against severe COVID-19, two primary preventive strategies have been proposed: anti-SARS-CoV-2 vaccination and pre-exposure prophylaxis (PrEP) with monoclonal antibodies that possess neutralizing activity against SARS-CoV-2. The effectiveness of vaccination varies depending on the type of organ transplanted and the immunosuppressive therapy used, whereas the effectiveness of PrEP does not depend on these factors. The timing of vaccination and PrEP administration is also crucial. A stronger immune response is observed when vaccination is conducted during the nadir of immunosuppressive therapy. However, when PrEP is administered concomitantly with the vaccine, the efficacy of the vaccination could be reduced, both in terms of antibody production and cell-mediated immunity. Therefore, PrEP should be administered at least 15 days after vaccine administration. In addition to the availability of various preventive measures against COVID-19 for the most vulnerable transplant patients, the scientific community strongly recommends adhering to protective measures, such as wearing masks, practicing hand hygiene, and maintaining social distancing. These expert recommendations offer crucial guidance on preventing SARS-CoV-2 infection in solid organ transplant patients and are applicable to everyday clinical practice.

Razionale. L’ipertensione arteriosa polmonare (IAP) è una patologia rara, che negli ultimi anni ha visto una crescente attenzione nei suoi riguardi e che ad oggi dispone di un ampio armamentario di farmaci specifici. L’approccio terapeutico è in continua evoluzione, ma persistono differenze tra i vari paesi. Materiali e metodi. Al fine di cogliere le differenze esistenti tra le realtà italiane che si occupano di IAP, tra dicembre 2024 e febbraio 2025 è stata condotta una survey mediante un questionario online composto da 20 domande.Risultati. La survey ha ricevuto una buona adesione, vedendo la partecipazione di 30 Centri italiani appartenenti a 15 regioni. I risultati hanno mostrato che tutti i Centri partecipanti eseguono il cateterismo cardiaco destro alla diagnosi di IAP (100%), mentre il test genetico è disponibile nel 73% dei casi. I Centri con un elevato volume di pazienti hanno il 10.4% dei pazienti in monoterapia orale, mentre i Centri con un basso volume di pazienti hanno una percentuale bassa di pazienti in triplice terapia orale (15%) o prostanoidi parenterali (11.3%). Molti Centri (70%) utilizzano prostanoidi parenterali nell’approccio iniziale nei pazienti con IAP ad alto rischio. Nove Centri (30%) raggiungono il profilo di basso rischio in oltre il 60% della popolazione seguita. In linea con la letteratura, una strategia iniziale con prostanoidi parenterali è risultata associata ad un’elevata probabilità di raggiungere un profilo di basso rischio. Il 70% dei Centri ha pazienti in lista d’attesa per trapianto polmonare.Conclusioni. Molti passi in avanti devono essere ancora compiuti per migliorare la gestione dei pazienti con IAP in Italia. Tuttavia, la consapevolezza della potenza terapeutica dei prostanoidi parenterali, soprattutto quando iniziati precocemente, e lo spirito di collaborazione tra i vari Centri esperti sono alla base del buon traguardo finora ottenuto nella realtà italiana.

Progressive fibrosing interstitial lung diseases (PF-ILDs) are characterized by persistent progression and have limited treatment options. The identification of reliable biomarkers to monitor fibrosis and therapeutic response remains a clinical challenge. This study investigated circulating plasma biomarkers associated with PF-ILDs and their potential role in monitoring disease evolution during nintedanib treatment. From 127 putative fibrosis biomarkers, seven candidates were identified with high diagnostic value (area under the curve [AUC] > 0.7), of which five (IGFBP2, PTX3, LGALS1, LGALS9, and MMP2) showed significant dynamic changes (assessed by longitudinal plasma proteomic analysis) in PF-ILD patients treated with 12-months nintedanib, correlating with improvements in forced vital capacity and diffusing capacity of the lung for carbon monoxide. Principal component analysis identified a shift in molecular profiles over time, suggesting nintedanib-induced modulation of these biomarkers. Receiver operating characteristic analysis demonstrated that while LGALS9 maintained a stable predictive value during nintedanib treatment, LGALS1, IGFBP2, PTX3, and MMP2 exhibited increasing AUC scores, indicating their potential role in monitoring fibrosis progression. We also identified optimal biomarker cut-off values at 12 months, which may provide reliable thresholds for fibrosis assessment. In conclusion, our exploratory analysis identified five biomarkers whose plasma concentrations changed during antifibrotic treatment, highlighting their potential prognostic value. Further validation in larger cohorts is needed to confirm their clinical utility.

Background. Pulmonary arterial hypertension (PAH) is a rare syndrome characterized by remodeling of the small pulmonary arterial vessels and increased pulmonary vascular resistance, which in the later stages can lead to right-sided heart failure and death. The therapeutic approach is evolving, but differences remain between countries. Methods. An Italian survey was designed to evaluate differences in the diagnosis, management and treatment of PAH patients across Italian centers. The survey was administered to 32 PAH Centers in Italy via an online questionnaire. A panel of 9 PAH experts analyzed and discussed the results. Results. Thirty Centers from 15 regions responded, thus representing the entire Italian reality. The results showed that all participating Centers perform right heart catheterization at PAH diagnosis (100%), while genetic testing is available in 73% of cases. Centers with a high patient volume have 10.4% of patients on oral monotherapy, while Centers with a low patient volume have a low percentage of patients on triple oral therapy (15%) or parenteral prostanoids (11.3%). Many Centers (70%) use parenteral prostanoids in up-front approach in incident high-risk PAH patients. Nine Centers (30%) achieve the low risk profile in more than 60% of the population followed. In line with the literature, an upfront strategy including parenteral prostanoids was associated with a high likelihood of achieving a low-risk profile. Overall, 70% of Centers have patients on the list for lung transplant. Conclusions. Italian PAH Centers have excellent adherence to the diagnostic standards recommended by European guidelines and good uniformity in therapeutic management, with some divergences related to differences in the phenotype of PAH patients. The survey also revealed a good awareness of the efficacy of parenteral prostanoids, especially if started early. Collaboration and referral of selected PAH patients to Centers with greater experience in the management of complex infusion therapies is the key to success.

BACKGROUND:Our understanding of pulmonary arterial hypertension (PAH) pathophysiology and treatment has significantly improved over time. Within this scenario we established the Italian Network on Pulmonary Arterial Hypertension (IPHNET), promoting a national prospective registry (ASPYRE-1) to collect data on contemporary PAH patients and to define the baseline features of Italian PAH patients. The aim of our study is to show the clinical characteristics of the Italian PAH patients. This cohort of patients will be followed in the ongoing registry to provide important insight into risk stratification and mode of death. METHODS:Twenty-six PAH centers were initially involved in the registry. Data were collected on anthropometric measurements, medical history, vital signs, clinical signs of heart failure, physical examination, laboratory tests (hematology and clinical chemistry), WHO functional class evaluation, non-encouraged 6-min walk test (6MWT), echocardiographic imaging and right heart catheterization (RHC) of outpatients and inpatients with PAH (Group 1) diagnosis. All the centers shared a common database for the prospective follow-up of the patients. RESULTS:between May 2014 and January 2023, a total of 500 prevalent patients with clinical diagnosis of PAH were enrolled: idiopathic (40.6 %), heritable (4.6 %), associated with drug or toxins (0.8 %), associated with connective tissue disease (26.4 %), associated with HIV infection (4.8 %), associated with portal hypertension (3.8 %), associated with congenital heart disease (16,4 %), with features of venous/capillary involvement (1.4 %), others (1.2 %) were enrolled. According to the ERS/ESC guidelines risk assessment, 207 (41.4 %) patients were at low risk, and 286 (57.2 %) were at intermediate risk. According to the REVEAL 2.0 score, 352 (70.4 %) patients were at low risk (score < 7), 93 (18.6 %) were at intermediate risk (score 7-8), and 55 (11 %) were at high risk (score > 8), Of the total patient population, 177 (35.4 %) received monotherapy, 222 (44.4 %) received double combination therapy, and 101 (20.2 %) received triple combination therapy. CONCLUSION:Built as a collaborative registry of centers treating PAH patients in Italy, it holds significant promise for addressing several critical needs within the field, including patients' clinical trajectory and risk stratification.

BACKGROUND:Pulmonary arterial hypertension (PAH) is a rare syndrome characterized by remodeling of the small pulmonary arterial vessels and increased pulmonary vascular resistance, which in the later stages can lead to right-sided heart failure and death. The therapeutic approach is evolving, but differences remain between countries. METHODS:An Italian survey was designed to evaluate differences in the diagnosis, management and treatment of PAH patients across Italian centers. The survey was administered to 32 PAH Centers in Italy via an online questionnaire. A panel of 9 PAH experts analyzed and discussed the results. RESULTS:Thirty Centers from 15 regions responded, thus representing the entire Italian reality. The results showed that all participating Centers perform right heart catheterization at PAH diagnosis (100%), while genetic testing is available in 73% of cases. Centers with a high patient volume have 10.4% of patients on oral monotherapy, while Centers with a low patient volume have a low percentage of patients on triple oral therapy (15%) or parenteral prostanoids (11.3%). Many Centers (70%) use parenteral prostanoids in up-front approach in incident high-risk PAH patients. Nine Centers (30%) achieve the low risk profile in more than 60% of the population followed. In line with the literature, an upfront strategy including parenteral prostanoids was associated with a high likelihood of achieving a low-risk profile. Overall, 70% of Centers have patients on the list for lung transplant. CONCLUSIONS:Italian PAH Centers have excellent adherence to the diagnostic standards recommended by European guidelines and good uniformity in therapeutic management, with some divergences related to differences in the phenotype of PAH patients. The survey also revealed a good awareness of the efficacy of parenteral prostanoids, especially if started early. Collaboration and referral of selected PAH patients to Centers with greater experience in the management of complex infusion therapies is the key to success.

Background:Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, life-threatening lung disease with a global incidence of 0.09-1.30 per 10,000 individuals. Pirfenidone and nintedanib are the approved treatments for IPF. Objectives:This study evaluated the real-world safety and tolerability profiles of pirfenidone and nintedanib in IPF patients treated at the Mediterranean Institute for Transplantation and Advanced Specialized Therapies (IRCCS ISMETT). A comparative analysis was conducted based on the number, types, and severity of adverse drug reactions (ADRs) and to identify potential predictors of treatment discontinuation or ADR onset based on patient characteristics. Design:A retrospective observational study was conducted on 531 IPF patients treated at IRCCS ISMETT with either pirfenidone or nintedanib. Methods:Eligible patients were selected based on the logged monthly dispensations provided by the pharmacy service for both therapies. Covariates were extracted from electronic medical records (age, sex, body mass index, smoking history, comorbidities, forced vital capacity (FVC) %, diffusing capacity of the lung for carbon monoxide (DLCO) %, 6-minute walk test (6-MWT), polytherapy, oxygen therapy, drug switch, etc.). ADRs were categorized by severity and follow-up status, and further classified according to the Medical Dictionary for Regulatory Activities, specifying the Preferred Terms and the related System Organ Classes. Chi-square or Fisher's exact test was used for categorical variables, and univariate and multiple logistic regression identified potential risk factors for ADR onset. Backward Stepwise logistic regression (BSLR) was used to determine independent variables associated with ADR occurrence. Results:The nintedanib group had more frequent ADRs related to gastrointestinal and hepatobiliary disorders, with nausea, diarrhea, anorexia, and weight loss as the most common. The pirfenidone group had more ADRs related to skin, nervous system, and vascular disorders, such as rash, nausea, dizziness, and blood pressure imbalances. Significant baseline differences between groups included age, smoking status, FVC (%), DLCO (%), and 6-MWT, with the nintedanib cohort showing worse baseline characteristics. A total of 450 ADRs were reported: 59.6% for nintedanib and 40.4% for pirfenidone. Independent variables that significantly increased the likelihood of experiencing ADR were drug change, treatment type, gender, and age. Conclusion:Identifying ADR predictors is essential for personalizing treatment strategies. Both pirfenidone and nintedanib are crucial in managing IPF, highlighting the need for further research to optimize personalized therapies and patient outcomes.

BACKGROUND:Right ventricular (RV) imaging has not a definite role in risk stratification of pulmonary arterial hypertension (PAH) patients. We tested the hypothesis that echocardiography-derived phenotypes, depicting different degrees of RV remodeling and dysfunction, may provide additional prognostic information to current risk stratification tools. METHODS:Consecutive incident PAH patients aged ≥18 years, diagnosed between January 2005 and December 2021, underwent clinical assessment, right heart catheterization, standard echocardiography. Simple echocardiographic variables were combined in order to define a priori four phenotypes representing different degrees of RV dilatation and RV-pulmonary arterial (PA) coupling: Phenotype 1 with mildy dilated right ventricle and preserved RV-PA coupling (n = 152 patients); phenotype 2 with mildly dilated right ventricle and poor RV-PA coupling (n = 143 patients); phenotype 3 with severely dilated right ventricle and preserved RV-PA coupling (n = 201 patients); phenotype 4 with severely dilated right ventricle and poor RV-PA coupling, with or without severe tricuspid regurgitation (n = 519 patients). Risk stratification was based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) 3-strata model and Registry to Evaluate Early and Long-Term PAH disease Management (REVEAL) 2.0 score. RESULTS:These phenotypes were present in all risk groups. Notably, regardless of the ESC/ERS risk stratum assigned to the patient, phenotype 4 was associated with a 2-fold increase of the odds of death (HR 2.1, 95% CI 1.6-2.8, p < 0.001), while phenotype 1 was associated with a 71% reduction in the odds of dying (HR 0.29, 95% CI 0.18-0.47, p < 0.001). CONCLUSIONS:Echocardiography-derived phenotypes describing RV remodeling and dysfunction may provide prognostic information which is independent of and additional to the clinically defined risk in incident PAH patients.

Pulmonary arterial hypertension is characterized by elevated blood pressure and pathological changes in the pulmonary arterioles, leading to the development of right-heart failure and potentially fatal outcomes if left untreated. This review aims to provide an overview of novel drugs or formulations and new drug indications for pulmonary arterial hypertension that are currently in phases II–III of randomized controlled trials, and describe the rationale for the use of these targeted therapies, as well as their efficacy, safety profile, and impact on quality of life and survival. The literature research was conducted using data from ClinicalTrials.gov for the period between 1 January 2016 up to 31 December 2022. The population of interest includes individuals aged ≥ 18 years who have been diagnosed with pulmonary arterial hypertension. The review selection criteria included trials with recruiting, enrolling by invitation, active, terminated or completed status in 2022 and 2023. A total of 24 studies were selected for evaluation based on the inclusion and exclusion criteria. This review summarizes the updated information from randomized clinical trials involving novel therapies for pulmonary arterial hypertension. However, larger clinical trials are required to validate their clinical safety and effects. In the future, clinicians should choose therapies based on the patient's individual situation and requirements when developing treatment strategies.

Kaposi sarcoma (KS) herpesvirus/human herpesvirus-8 (HHV-8) neoplastic and nonneoplastic disease in solid organ transplant recipients can be life-threatening. We evaluated the seroprevalence of HHV-8 infection among donors (D) and recipients (R), the incidence of HHV-8 transmission/reactivation, and the clinical characteristics, management, and outcomes of HHV-8-related diseases, including KS herpesvirus-associated inflammatory cytokine syndrome (KICS), in consecutive SOT patients from 2011 to 2023. HHV-8 seroprevalence was 3.3% in 1349 donors and 8.4% in 1856 recipients screened (P < .0001). In the D+/R-group (n-49), 13 patients developed HHV-8-related diseases: 7 liver recipients had KICS, and 1 lung recipient had KS with subsequent KICS. Four KICS patients treated with rituximab survived, whereas the 3 patients not treated with rituximab died. Within the D-/R-group, of 5 (0.3%) patients with non-donor-derived primary HHV-8 infection, 3 liver recipients developed KICS. Of the R+ patients (n-155), 3 developed KS. In our cohort, 25/944 (2.6%) liver transplant recipients had a primary HHV-8 infection, and 10 of them (40%) developed KICS; 40% (4/10) of HHV-8 seropositive heart transplant recipients developed reactivation, and 2 of them (50%) had fatal KS. Serologic screening and molecular surveillance of D+/R-patient groups facilitate early recognition and effective therapy of KICS.

Lung transplant recipients (LTRs) respond poorly to vaccination. SARS-CoV-2 pre-exposure prophylaxis (PrEP) with tixagevimab/cilgavimab (TIX/CIL) reduces the incidence of infection and the evolution to severe COVID-19. In vitro data show decreased activity against Omicron variants. We evaluated the clinical efficacy and safety of TIX/CIL in LTRs during the Omicron wave. A prospective observational cohort study was conducted at ISMETT in Palermo (Italy). In June 2022, SARS-CoV-2 PrEP with TIX/CIL 150/150 mg was offered to LTRs. LTRs who received TIX/CIL were compared to LTRs who did not. Logistic regression analysis (adjusted for prior COVID-19, SARS-CoV-2 vaccination, age, years from transplant, and rejection) was performed. The objective of this study was to compare the following among the two populations: prevalence of SARS-CoV-2, length of SARS-CoV-2 positivity, and COVID-19 disease severity. Among 110 eligible LTRs, 79 (72%) received TIX/CIL and 31 (28%) did not. SARS-CoV-2 infections occurred in 6% (n = 5) of patients who received TIX/CIL and 29% (n = 9) of patients who did not (p < 0.001). In both groups, infections were mild/asymptomatic, and no one was hospitalized or died. At multivariate analysis, TIX/CIL was associated with a lower risk of infection (aOR 0.22; 95%CI 0.06–0.78; p = 0.02). TIX/CIL was safe and effective in reducing the risk of SARS-CoV-2 in LTRs during the Omicron wave.

L’ipertensione polmonare (IP) è una patologia frequente in tutto il mondo, nella maggior parte dei casi secondaria a malattie cardiovascolari e respiratorie, con una prognosi infausta. L’ipertensione arteriosa polmonare (IAP) è una forma rara che colpisce il sistema vascolare arterioso polmonare. IP e IAP sono caratterizzate da sintomi aspecifici e da un progressivo aumento delle resistenze vascolari polmonari che determina disfunzione ventricolare destra, talvolta irreversibile. Negli ultimi anni, il crescente impegno dei sanitari e delle istituzioni ha consentito diagnosi più accurate in tempi più brevi. Tuttavia, il divario tra le linee guida e la pratica clinica rimane una sfida per tutti gli specialisti coinvolti nella gestione della malattia. Considerando la necessità di condividere e descrivere percorsi diagnostici e terapeutici, di valutare i risultati ottenuti e di affrontare i costi e le problematiche organizzative prodotte da questa malattia, tutte le figure coinvolte dovrebbero collaborare per migliorarne l’impatto prognostico e la spesa sanitaria. In questo documento di consenso, gli esperti dell’Associazione Nazionale Medici Cardiologi Ospedalieri (ANMCO) e della Società Italiana di Cardiologia (SIC) sull’IP affrontano 1) definizione, classificazione e bisogni insoddisfatti dell’IP e IAP; 2) classificazione e caratteristiche dei centri coinvolti nella diagnosi e cura della malattia; 3) proposta di organizzazione di un percorso diagnostico-terapeutico, basato su robuste e recenti evidenze scientifiche.

Pulmonary hypertension (PH) is a frequent pathological condition worldwide, mainly secondary to cardiovascular and respiratory diseases, with a poor prognosis. Pulmonary arterial hypertension (PAH) is a rare form that affects the arterial pulmonary vasculature. PH and PAH are characterized by non-specific symptoms and a progressive increase of pulmonary vascular resistance that results in progressive, sometimes irreversible, right ventricular dysfunction. In recent years, a growing medical and social commitment on this disease allowed more accurate diagnosis in shorter times. However, the gap between guidelines and clinical practice remains a challenge for all medical doctors involved in the disease management. Considering the needs to share and describe diagnostic and therapeutic pathways, to measure the results obtained and to address the economical and organizational problems of this disease, all involved figures should collaborate to improve its prognostic impact and health expenses. In this consensus document, the PH experts of the Italian Association of Hospital Cardiologists (ANMCO) together with those of the Italian Society of Cardiology (SIC), address 1) definition, classification and unmet needs of PH and PAH; 2) classification and characteristics of centers involved in the diagnosis and treatment of the disease; 3) proposal of organization of a diagnostic-therapeutic pathway, based on robust and recent scientific evidence.

Pulmonary hypertension (PH) is a frequent pathological condition worldwide, mainly secondary to cardiovascular and respiratory diseases, with a poor prognosis. Pulmonary arterial hypertension (PAH) is a rare form that affects the arterial pulmonary vasculature. PH and PAH are characterized by non-specific symptoms and a progressive increase of pulmonary vascular resistance that results in progressive, sometimes irreversible, right ventricular dysfunction. In recent years, a growing medical and social commitment on this disease allowed more accurate diagnosis in shorter times. However, the gap between guidelines and clinical practice remains a challenge for all medical doctors involved in the disease management. Considering the needs to share and describe diagnostic and therapeutic pathways, to measure the results obtained and to address the economical and organizational problems of this disease, all involved figures should collaborate to improve its prognostic impact and health expenses. In this consensus document, the PH experts of the Italian Association of Hospital Cardiologists (ANMCO) together with those of the Italian Society of Cardiology (SIC), address 1) definition, classification and unmet needs of PH and PAH; 2) classification and characteristics of centers involved in the diagnosis and treatment of the disease; 3) proposal of organization of a diagnostic-therapeutic pathway, based on robust and recent scientific evidence.

The pre- and postoperative management of lung transplant patients is one of the most challenging in solid organ transplantation. In order to maintain a successful lung transplant program over time, an adequate clinical team should be available because the primary transplant team will mostly be involved in the care of this complex population in the short and long term. Most patients will require a lifelong assistance exclusively from the transplant hospital. High-quality continuous education of the staff, adequate resources, integration between medical and surgical specialties, and constantly updated protocols and policies are all required to establish and maintain a viable lung transplant program. The role of the transplant pulmonologist far exceeds a comprehensive knowledge of lung disease and requires advanced skills in immunology, infectious diseases, and interventional bronchology. A thorough understanding of all expected complications after lung transplantation (LTX) is required to provide excellent short- and long-term outcomes.