Functional Capacity and Quality of Life in Patients With Vascular Ring

Study Overview
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Brief Summary
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To investigate the long term functional capacity and quality of life of patients with a history of vascular ring whether they did or did not have corrective surgery. Patients will perform pulmonary function test, 6 minute walk test, cardiopulmonary exercise test and will fill a quality of life questionnaire.

Detailed Description
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Vascular rings are congenital vascular anomalies of the aortic arch that compress the esophagus and the trachea. The most common vascular rings are double aortic arch and right aortic arch with left ligamentum. Pulmonary artery sling is less common. Another cause of tracheal compression occurring only in infants is the innominate artery compression syndrome. When patients are symptomatic, surgical correction should be performed. data regarding long term functional capacity and quality of life is lacking. our aim is to investigate the long term functional capacity and quality of life of patients with history of vascular ring whether they did or did not have corrective surgery. Methods: a prospective study evaluating functional capacity and quality of life the patients will perform pulmonary function test, 6 minute walk test, cardiopulmonary exercise test and will fill a quality of life questionnaire (sf-36).

Official Title
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Functional Capacity and Quality of Life in Patients With Vascular Ring

Conditions
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Vascular Ring

Intervention / Treatment
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* Diagnostic Test: Cardiopulmonary exercise test


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: patients with history of vascular ring Exclusion Criteria: refusal to participate inability to complete cardiopulmonary testing

Ages Eligible for Study
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Minimum Age: 5 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| patients with history of vascular ring who didn't have corrective surgery<br>patients with history of vascular ring who didn't have corrective surgery | Diagnostic Test: Cardiopulmonary exercise test<br>* CPET using a cycle ergometer (COSMED, Rome, Italy) beginning with no resistance warmup lasting 1-3 minutes followed with an incrementing resistance adapted to the patient's functional capacities according to the examiner's free judgment by successive stages Six minute walk test - patients will be instructed to walk as far as possible for six minutes in a 30-meter long enclosed corridor with a flat and hard surface, marked at every meter. Meters will be calculated at the end of the test. SF-36 is a questionnaire that contains eight domains - physical functioning (10 items); role limitations due to physical functioning (4 items); role limitations due to emotional problems (3 items); energy and vitality (4 items); mental health (5 items); social functioning (2 items); bodily pain (2 items); and general health perceptions (5 items). Scores are summed for each domain and were transformed into scores of between 0 (worst possible health state) to 100 (best possible health state).<br>* Other names: quality of life questionnaire (sf-36);|
| patients with history of vascular ring who underwent corrective surgery<br>patients with history of vascular ring who underwent corrective surgery | Diagnostic Test: Cardiopulmonary exercise test<br>* CPET using a cycle ergometer (COSMED, Rome, Italy) beginning with no resistance warmup lasting 1-3 minutes followed with an incrementing resistance adapted to the patient's functional capacities according to the examiner's free judgment by successive stages Six minute walk test - patients will be instructed to walk as far as possible for six minutes in a 30-meter long enclosed corridor with a flat and hard surface, marked at every meter. Meters will be calculated at the end of the test. SF-36 is a questionnaire that contains eight domains - physical functioning (10 items); role limitations due to physical functioning (4 items); role limitations due to emotional problems (3 items); energy and vitality (4 items); mental health (5 items); social functioning (2 items); bodily pain (2 items); and general health perceptions (5 items). Scores are summed for each domain and were transformed into scores of between 0 (worst possible health state) to 100 (best possible health state).<br>* Other names: quality of life questionnaire (sf-36);|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Exercise capacity | Oxygen uptake evaluated by cardiopulmonary exercise testing (CPET) | Measured continuously during 15 minutes of the exercise test (CPET) | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Six minute walk test | The distance gained after six minute of habitual walking. | Six minutes of evaluation, completed pre cardiopulmonary exercise test (CPET) | 
| Quality of life - SF-36 | The calculated result of SF-36 questionnaire | Five minutes of evaluation, completed pre cardiopulmonary exercise test (CPET) | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Cardiopulmonary exercise testing,, Double aortic arch