Respiratory muscle strength in patients with pulmonary arterial hypertension before and after effective treatment

Recruiting

• Conditions: I27.0; I27.8; I27.9; Primary pulmonary hypertension; Other specified pulmonary heart diseases; Pulmonary heart disease, unspecified

• Registration Number: DRKS00013887
• Lead Sponsor: Department für Neurologie - Klinik für Schlafmedizin und neuromuskuläre Erkrankungen

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Study Start: 2018-06-15
• Last Update Posted: 19 August 2024

Recruitment & Eligibility

• Status: Recruiting
• Sex: All
• Target Recruitment: 20

Inclusion Criteria

Invasively obtained diagnosis of PAH (mean pulmonary arterial pressure = 25 mmHg; post-capillary wedge pressure =15 mmHg; pulmonary vascular resistance > 3 Wood units) in accordance with the most recent guideline of the European Society of Cardiology (ESC)
-APAH due to (echocardiographically) proven congenital heart disease or due to (antinuclear antibody positive) connective tissue disease (systemic sclerosis) in accordance with the most recent guideline of the European Society of Cardiology (ESC)
-No or ineffective treatment (untreated”), as defined by no clinical improvement (NYHA functional class > II) and/or persistent elevation or no near-to-normal reduction of NT-proBNP levels under current PAH medication
-Age > 18 years
-Free ambulation
-Written informed consent to participate in the study

Additional inclusion criterion for post-treatment evaluation:

Positive treatment response as reflected by clinical improvement (NYHA I or II) and/or normal or near-to-normal of NT-proBNP.

Exclusion Criteria

-Body Mass Index (BMI) > 35
-Heart failure with left ventricular ejection fraction <50%
-Chronic obstructive pulmonary disease, emphysema
-Presence of severe neurological comorbidities especially primary myopathy, muscular dystrophy, motor neuron disease, phrenic nerve disease, epilepsy, multiple sclerosis, parkinson’s disease
-Severe psychiatric disease including dementia
-Thoracic wall disease
-Cardiac pacemaker

Study & Design

• Study Type: observational
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
(change in) twitch transdiaphragmatic pressure after magnetic cervical stimulation of the phrenic nerves [cmH2O]<br><br>