Pathophysiology of respiratory muscle stength and its contribution to exercise intolerance in patients with congenital heart disease

Conditions: Q21
Congenital malformations of cardiac septa

Registration Number: DRKS00014698

Lead Sponsor: Institut für Schlafmedizin und neuromuskuläre Erkrankungen Universitätsklinikum Münster

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Pending

Sex: All

Target Recruitment: 60

Inclusion Criteria

a. Age > 18 years (no age limit) (Group 1, 2 and 3)
b. able to consent (Group 1, 2 and 3)
c. Diagnosis of congenital heart disease according to the most recent Guidelines of the European Society of Cardiology (ESC); >12 weeks since the diagnosis of congenital heart disease 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 pulmonary arterial Hypertension due to congenital heart disease; where there was no treatment with combination therapy in PAH patients then the reason must be documented
d. Controls (matched for age and gender) with normal findings on echocardiogram, electrocardiogram and normal levels of natriuretic peptide hormone

Exclusion Criteria

a. Body Mass Index (BMI) > 35
b. Chronic obstructive pulmonary disease, emphysema
c. Presence of severe neurological comorbidities especially primary myopathy, muscular dystrophy, motor neuron disease, phrenic nerve disease, epilepsy, multiple sclerosis, parkinson’s disease
d.Severe psychiatric disease including dementia
e. Cardiac pacemaker

Study & Design

Study Type: observational

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Twitch transdiaphragmatic pressure after magnetic cervical stimulation of the phrenic nerves [cmH2O] [after Placement of two thin balloon catheters above and below the diaphragm allowing for continuous measurement of transdiaphragmatic pressure]

Secondary Outcome Measures