I27.0 I27.8 I27.9
Conditions
Interventions
Group 1: First of all current symptom scores and a 6 Minute walk distance test will be performed
Patients with PAH will then undergo a comprehensive and unique set of respiratory regulation tests wit
Sponsors
Department für Neurologie - Klinik für Schlafmedizin und neuromuskuläre Erkrankungen (Direktor: Univ.-Prof. Dr. med. Peter Young)
Eligibility
Sex/Gender
Male
Age
18 Years to 70 Years
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) - APAH due to (echocardiographically) proven congenital heart disease or due to (antinuclear antibody positive) connective tissue disease (systemic sclerosis) or IPAH with comprehensive investigations in order to exclude other causes of PAH - 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 - Presence of SDB on cardiorespiratory polygraphy (AHI > 15/h) - Age > 18 years and < 70 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 |
|---|---|
| Presence (type and severity) of sleep disordered breathing (SDB) [no SDB vs. obstructive SDB vs. central SDB; mild vs. moderate vs. severe] | — |
Secondary
| Measure | Time frame |
|---|---|
| Apnoea-Hypopnoea-Index on polygraphic recording [/h], Hypopnoea-Index [/h], central Apnoea-Index [/h], obstructive Apnoea-Index [/h], cycle lengths of obstructive events [sec], cycle lengths of central events [sec], length of CSA-CSR [sec], rightventricular ejection fraction on MRI [%], leftventricular ejection fraction on MRI [%], hypercapnic ventilatory Response testing [l/min/mmHg endexspiratory CO2], Epworth Sleepiness Scale [points], Berlin Sleep Apnoea Questionaire [points], Pittsburgh Sleep Quality Index [points], Minnesota Living with Heart Failure Questionaire [points], Heart rate [bpm], systolic blood pressure [mmHG], diastolic blood pressure [mmHG], stroke volume index [L/min/m²], high frequency component of diastolic blood pressure variability[%], low frequency component of heart rate variability [%], respiratory rate [/min], tidal volume [ml], minute ventilation [L/min], oxygen saturation [%], high frequency component of heart rate variability [%], low frequency component of diastolic blood pressure variability [%], Systolic blood pressure [mmHG], diastolic blood pressure [mmHG], stroke volume index [L/min/m²] | — |
Countries
Germany
Contacts
Public ContactJens Spiesshoefer
Department für Neurologie - Klinik für Schlafmedizin und neuromuskuläre Erkrankungen Universitätsklinikum Münster
Outcome results
None listed