I27.0 I27.8 I27.9
Conditions
Interventions
Group 1: Patients with PAH will undergo a comprehensive and unique set of lung function and respiratory muscle strength tests within our lab of respiratory physiology.
This will be done at daytime.
Fi
Sponsors
Department für Neurologie - Klinik für Schlafmedizin und neuromuskuläre Erkrankungen
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
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
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| (change in) twitch transdiaphragmatic pressure after magnetic cervical stimulation of the phrenic nerves [cmH2O] | — |
Secondary
| Measure | Time frame |
|---|---|
| Fatigue Severity Scale [points], SF-36 [points], 6 MWD [m], Medical Research Council Dyspnoea Scale [points], Borg Dyspnoea Scale pre and post 6 MWD [m], fist muscle strength [kg], leg muscle strength [kg], Forced vital capacity [ml], FEV1/FVC [%], MIP [cmH2O], MEP [cmH2O], SNIP [cmH2O], peak cough Flow [L/sek], Rate of rise of diaphramatic amplitude during tidal breathing on ultrasound [cm/sec], Rate of rise of diaphramatic amplitude during Sniff maneuver on ultrasound, Rate of rise of diaphramatic amplitude during maximal inspiration on ultrasound, diaphragm thickness after maximal inspiration on ultrasound [cm], diaphragm thickness after maximal exspiration on ultrasound, diaphragm thickness after tidal breathing on ultrasound [cm], thickening ratio [n.u.], twitch mouth pressure after magnetic cervical stimulation of the phrenic nerves [cmH2O], twitch transdiaphragmatic pressure after magnetic cervical stimulation of the phrenic nerves [cmH2O], twitch mouth pressure after cortical stimulation of the phrenic nerves [cmH2O], twitch transdiaphragmatic pressure after magnetic cortical stimulation of the phrenic nerves [cmH2O], twitch gastric pressure after thoracic magnetic stimulation of the (expiratory) abdominal muscles [cmH2O], twitch mouth pressure after thoracic magnetic stimulation of the (expiratory) abdominal muscles [cmH2O] phrenic nerve conduction time after cortical magentic stimulation [ms], phrenic nerve conduction time after cervical magentic stimulation [ms], Amplitude of compound diaphragm muscle action potential after cortical magentic stimulation [mA], Amplitude of compound diaphragm muscle action potential after cervical magnetic stimulation [mA], central motor conduction time [ms], transdiaphragmatic pressure after voluntary maneuvers [cmH2O], mouth pressure after voluntary maneuvers [cmH20] Releaxation-rate of Twitch transdiaphragmatic pressure after magnetic cervical stimulation of the phrenic nerves [msec] | — |
Countries
Germany
Contacts
Public ContactJens Spiesshoefer
Department für Neurologie - Klinik für Schlafmedizin und neuromuskuläre Erkrankungen Universitätsklinikum Münster
Outcome results
None listed