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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.
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).
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| patients with history of vascular ring who didn't have corrective surgery | patients with history of vascular ring who didn't have corrective surgery |
| |
| patients with history of vascular ring who underwent corrective surgery | patients with history of vascular ring who underwent corrective surgery |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Cardiopulmonary exercise test | Diagnostic Test | 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). |
| Measure | Description | Time Frame |
|---|---|---|
| Exercise capacity | Oxygen uptake evaluated by cardiopulmonary exercise testing (CPET) | Measured continuously during 15 minutes of the exercise test (CPET) |
| 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 |
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Inclusion Criteria:
Exclusion Criteria:
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patients with history of vascualr ring
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Lea Bentur, MD | Contact | +972-4-7774360 | l_bentur@rambam.health.gov.il | |
| Vered Nir, MD | Contact | +972-4-7774360 | v_nir@rambam.health.gov.il |
| Name | Affiliation | Role |
|---|---|---|
| Lea Bentur, MD | Head of Pediatric Pulmonary Institute | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Rambam health care campus | Recruiting | Haifa | Israel |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 35971243 | Derived | Nir V, Bentur L, Zucker-Toledano M, Gur M, Adler Z, Hanna M, Toukan Y, Masarweh K, Hakim F, Bar-Yoseph R. Functional capacity and quality of life in patients with vascular ring. Pediatr Pulmonol. 2022 Dec;57(12):2946-2953. doi: 10.1002/ppul.26112. Epub 2022 Sep 14. |
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| ID | Term |
|---|---|
| D000073872 | Vascular Ring |
| ID | Term |
|---|---|
| D054079 | Vascular Malformations |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D001015 | Aortic Arch Syndromes |
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| ID | Term |
|---|---|
| D005080 | Exercise Test |
| D000070857 | Walk Test |
| ID | Term |
|---|---|
| D006334 | Heart Function Tests |
| D003935 | Diagnostic Techniques, Cardiovascular |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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|
|
| Five minutes of evaluation, completed pre cardiopulmonary exercise test (CPET) |
| D001018 | Aortic Diseases |
| D014652 | Vascular Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D012129 | Respiratory Function Tests |
| D003948 | Diagnostic Techniques, Respiratory System |
| D016552 | Ergometry |
| D008919 | Investigative Techniques |