Primary Objective: To evaluate persisting pulmonary morbidity and echocardiographic signs of pulmonary hypertension in young adults born with CDH that interfere with physical fitness and participation in society.Secondary Objective(s): To evaluate…
ID
Source
Brief title
Condition
- Respiratory disorders congenital
- Cognitive and attention disorders and disturbances
- Pulmonary vascular disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Persistent pulmonary morbidity as reflected by:
* Irreversible small airway obstruction (FEV1 before and after bronchodilation,
FEF25-75 before and after bronchodilatation)
* Reduced diffusion capacity of the lungs after correction for total lung
capacity (DLOCc, KCORCL)
* Reduced maximal exercise capacity (VO2max)
* Abnormal pulmonary structure on chest CT (separate volume fractions of total
airways disease (%Dis), and trapped air (%TA))
Secondary outcome
* Abnormal morphology of the repaired diaphragm
* Echocardiographic signs of pulmonary hypertension and ventricular dysfunction4
* Total and subdomain scores participation (IPA)
* Health status (physical and mental scale of RAND-36)
* Total score fatigue (FSS)
* Dyspnea score
* Total score and domain scores social emotional wellbeing (scores on ASR)
* Airway artery (AA) dimensions using the AA-method
* Growth and nutritional status (BOD-POD)
Background summary
Information on outcomes in (young) adults born with CDH is scarce. Based on the
studies in adults born before extracorporeal membrane oxygenation (ECMO) was
available we have the following assumptions and hypothesis: The population of
CDH patients that survives nowadays has more severe lung hypoplasia than those
who survived in the 1980s before ECMO became available. Our hypothesis is that
the population of young adults who were treated for CDH in an era with improved
intensive care and use of ECMO, have persistent pulmonary morbidity and
microstructural changes in the lungs with obstructive lung function, decreased
diffusion capacity of the lungs, signs of pulmonary hypertension, and
diminished maximal exercise capacity. Moreover, we assume that they suffer more
frequently from fatigue, poor growth, altered nutritional status, and
social-emotional problems and that their health status and participation is
reduced compared to that of healthy peers.
Study objective
Primary Objective: To evaluate persisting pulmonary morbidity and
echocardiographic signs of pulmonary hypertension in young adults born with CDH
that interfere with physical fitness and participation in society.
Secondary Objective(s): To evaluate fatigue, social-emotional wellbeing,
nutritional and health status, and participation in society.
Study design
Observational, cross-sectional cohort study.
Study burden and risks
Participants are being asked to come one full day for assessments and physical
examination and are being offered a second visit to the outpatient clinic of
the hospital or a phone call to discuss individual results. Additional
questionnaires will take approximately one hour to fill in at home or otherwise
during the break between the other assessments. Assessments include lung
function measurement before and after bronchodilatation, maximal exercise test,
echocardiogram and electrocardiogram and a CT scan and questionnaires.
Bronchodilatation is performed using 12 mcg of formoterol which is the normal
dose used in routine patient care and considered of low risk. The lowest
radiation dose will be used to obtain a CT of diagnostic quality. The risks
related to this protocol are considered low. Benefits for the participant are
careful evaluation, individual results and advice for future care provided by a
medical specialist experienced in CDH. The results of this study will
contribute to the evidence for optimal care of CDH patients in adolescence and
adulthood.
Dr molenwaterplein 60
Rotterdam 3015GJ
NL
Dr molenwaterplein 60
Rotterdam 3015GJ
NL
Listed location countries
Age
Inclusion criteria
*Diagnosed with CDH within the first 7 days of life and alive at time of
recruitment
*Born between 1989 and 2001 (patient recruitment is ongoing in 2019; minimum
age is 18 years)
*Sufficient intellectual capacities and/or command of the Dutch language to
understand instructions
*Clinically stable for > 3 weeks
Exclusion criteria
*Serious comorbidity that might affect assessments (e.g. serious neurological
comorbidity)
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL67096.078.18 |