Abstract

Abstract Backgroung: Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and may be associated with a variety of cardiovascular and respiratory diseases. PH is defined by a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg at rest,assessed by right heart catheterization (RHC). Methods The Cardiology Rare Disease Outpatient Clinic was opened in 2021 in the Cardiology Division of S. Croce and Carle Hospital, Cuneo. In this ambulatory setting, patients with suspected or confirmed PH diagnosis were screened and followed up. The outpatient evaluation included cardiological physical examination, electrocardiogram (ECG) and transthoracic echocardiogram. In case of echocardiographic PH suspicion (intermediate or high probability) a comprehensive work–up was performed with first level diagnostic tools (respiratory function tests with DLCO, high–resolution chest CT, arterial blood gases, blood tests and overnight oximetry or polysomnography). Eventually, lung scintigraphy was performed and work up was completed with RHC with vasoreactivity test with nitric oxide (NO) if indicated. Multidisciplinary team approach proved to be an essential requirement for good practice in this setting. Results Between January 2021 and December 2022, 95 patients were evaluated in the PH outpatient clinic; Sixty–one (63%) patients were female, mean age was 66 years. Forty–two (42%) patients were referred from Rheumatology, 9 (9%) from Pulmonologiy, 13 (14%) from Emergency Medicine or Internal Medicine, 20 (21%) from Cardiology, and the remaining 13 (14%) patients from other departments or from other hospitals. Following clinical and echocardiographic evaluation, patients were stratified into low, intermediate and high PH–risk levels. In 23 (24%) patients RHC was performed and in one case it was associated with vasoreactivity test with NO. Eight (8%) patients with chronic thromboembolic PH (CTEPH) suspicion were referred to the Hub Center in Pavia to evaluate pulmonary endarterectomy. Seven (7%) patients were treated with specific therapy for group 1 PH and 3 (3%) patients for CTEPH. Two (2%) patients died during follow–up. Conclusions The aim of the dedicated PH outpatient clinic is to screen patients and identify those who need a complete work–up for diagnosis and classification of PH subtypes, in order to select those patients who could benefit from specific PH therapies, and to monitor the response at FU.

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