Sharing Diagnostic and Therapeutic Pathways in Pulmonary Arterial Hypertension: A Roadmap for the Triveneto Region

Davide Stolfo — 2025-05-19

Background: Pulmonary arterial hypertension (PAH) is a rare and progressive disease characterized by high mortality and a significant impact on quality of life. Despite therapeutic advances, considerable heterogeneity persists in Italy’s diagnostic and therapeutic pathways, leading to delays in diagnosis and disparities in access to care.
Objective: With this work we aim to define a roadmap for a shared diagnostic, therapeutic, and care pathway for PAH in the Triveneto region, aiming to reduce management variability, improve continuity of care, and optimize the use of available resources.
Methods: A multidisciplinary group of cardiologists, pulmonologists, and rheumatologists from Triveneto, along with a representative from the Pulmonary Hypertension Patients’ Association, convened in a series of meetings to discuss diagnostic and therapeutic pathways.
Results: Early identification of PAH should be based on the classification of symptomatic patients into three main phenotypes (cardiac, pulmonary, and not better specified), followed by a specific diagnostic process based on updated hemodynamic criteria. Therapeutic strategies should be defined according to risk stratification, with clear guidelines for comorbidity management and monitoring through quality indicators (Key Performance Indicators) to assess the effectiveness of care pathways.
Conclusions: The proposed roadmap represents a shared model for PAH management in the Triveneto region, promoting a multidisciplinary and integrated approach. Its adoption could enhance care quality, reduce regional disparities, and align with European guidelines, ultimately improving patient prognosis and quality of life.

Evolution of Treatments for Hereditary Angioedema in Italy: Use of Lanadelumab in Clinical Practice

Marta Nugnes — 2025-06-13

Introduction: Hereditary angioedema (HEA) is a rare genetic disorder characterised by recurrent episodes of
edema affecting various body districts. HEA therapy includes treatment of acute attacks and short- or long-term
prophylaxis.
Objective: To describe the main demographic and clinical characteristics of patients with HEA in real Italian clinical
practice, as well as the use of pharmacological treatments, the use of healthcare resources and costs with
particular reference to lanadelumab.
Methods: From administrative databases of healthcare institutions for about 9 million patients, between January
2010 and September 2020, all-age HEA patients were identified through hospitalisation, exemption code or specific
drugs. Demographic and clinical characteristics, medications prescribed for acute attacks and prophylaxis
were described at inclusion. For patients treated with lanadelumab, the switching of dosing regimen from 2 to 4
weeks was examined. Healthcare resource utilisation and costs were assessed at one-year follow-up.
Results: 258 patients with HEA were identified (~0.003% of the sample). Of them, 41.1% were male, and the
mean age was 44.6 years; 80% of patients were treated for acute attacks, and 20% were on prophylaxis. In
lanadelumab-treated, 85% started treatment with a 2-week regimen, and of them 75.8% switched to a 4-week
regimen. For these patients, the estimated annual cost—calculated using the weighted average of patients who
switched treatments—was €114,362.
Conclusions: The data from this analysis on therapeutic management of HEA patients in the Italian clinical practice
showed that, despite the limited number of patients treated with lanadelumab, many received one administration
every 4 weeks, suggesting a good disease control.

AboutOpen

Sharing Diagnostic and Therapeutic Pathways in Pulmonary Arterial Hypertension: A Roadmap for the Triveneto Region

Evolution of Treatments for Hereditary Angioedema in Italy: Use of Lanadelumab in Clinical Practice