Contribution of respiratory muscle strength and right ventricular pump function to exercise intolerance in patients with precapillary pulmonary hypertensio

Conditions: I27.0
I27.20
Primary pulmonary hypertension

Registration Number: DRKS00014695

Lead Sponsor: Institut für Schlafmedizin und neuromuskuläre Erkrankungen Universitätsklinikum Münster

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Complete

Sex: All

Target Recruitment: 86

Inclusion Criteria

a. Age > 18 years (no age limit)
b. able to consent
c. Diagnosis of Nizza class I or IV precapillary pumonary Hypertension according to the most recent Guidelines of the European Society of Cardiology (ESC); >12 weeks since the diagnosis of PH has been made; no hospitalisation for HF in the last 4 weeks; optimal medical therapy in accordance with the most recent Guidelines of the European Society of Cardiology (ESC) with no change in medication in the last 4 weeks and combination therapy for PAH -where there was no treatment with combination therapy in PAH patients then the reason must be documented

Exclusion Criteria

- secondary (postcapillary) pulmonary hypertension (mean pulmonary arterial hypertension > 25 mmHg; PCWP >15 mmHg)
- other severe internistic pre-existing conditions, especially insulin-dependent diabetes or severe renal impairment
- severe neurological pre-existing conditions such as stroke, especially brainstem infarction
- intake of opioids
- severe psychiatric pre-existing conditions
- contraindications to (cardiac) magnetic resonance imaging (MRI)

Study & Design

Study Type: observational

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Thickening Ratio on diaphragm ultrasound (n.U.)

Secondary Outcome Measures

Name	Time	Method
Fatigue Severity Scale [points]<br> 6 MWD [m], Medical Research Council Dyspnoea Scale [points], Borg Dyspnoea Scale, Minessota Living with Heart Failure Questionnaire [Points]<br>-> the above mentioned parameters will be assessed using questionnaires<br>Forced vital capacity [ml], FEV1/FVC [%]<br>-> the above mentioned Parameters will be assessed using Standard lung function testing<br>Rate of rise of diaphragmatic amplitude during tidal breathing on ultrasound [cm/sec], Rate of rise of diaphragmatic amplitude during Sniff maneuver on ultrasound, Rate of rise of diaphragmatic 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]<br>-> the above mentioned Parameters will be assessed using diaphragm ultrasound<br>right ventricular pumpfunction on a cardiac MRI scan [%]<br>pO2 and pCO2 on capillary blood gas analysis [mmHG]<br>