Functional Capacity and Quality of Life in Patients With Vascular Ring

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.

Study Overview

• Status: Recruiting
• Conditions: Vascular Ring
• Intervention / Treatment: Diagnostic test: Cardiopulmonary exercise test

Detailed Description

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).

• Study Type: Observational
• Enrollment (Anticipated): 30

Contacts and Locations

• Study Contact: Name: Lea Bentur, MD; Phone Number: +972-4-7774360; Email: l_bentur@rambam.health.gov.il
• Study Contact Backup: Name: Vered Nir, MD; Phone Number: +972-4-7774360; Email: v_nir@rambam.health.gov.il

Study Locations

• Israel
  • Haifa, Israel
    • Recruiting
    • Rambam Health Care Campus
    • Contact: Lea Bentur, Prof.; Phone Number: 972-4-777-4360; Email: l_bentur@rambam.health.gov.il

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 5 years and older (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

patients with history of vascualr ring

Description

Inclusion Criteria:

• patients with history of vascular ring

Exclusion Criteria:

• refusal to participate
• inability to complete cardiopulmonary testing

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Only
• Time Perspectives: Cross-Sectional

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
patients with history of vascular ring who didn't have corrective surgery	Diagnostic test: Cardiopulmonary exercise 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).; Other Names: 6 minute walk test; quality of life questionnaire (sf-36)
patients with history of vascular ring who underwent corrective surgery	Diagnostic test: Cardiopulmonary exercise 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).; Other Names: 6 minute walk test; quality of life questionnaire (sf-36)

What is the study measuring?

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)

Collaborators and Investigators

• Sponsor: Rambam Health Care Campus
• Investigators: Principal Investigator: Lea Bentur, MD, Head of Pediatric Pulmonary Institute

Publications and helpful links

General Publications

• 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.

Study record dates

Study Major Dates

• Study Start (Actual): March 15, 2018
• Primary Completion (Anticipated): December 1, 2021
• Study Completion (Anticipated): February 1, 2022

Study Registration Dates

• First Submitted: February 21, 2021
• First Submitted That Met QC Criteria: February 28, 2021
• First Posted (Actual): March 4, 2021

Study Record Updates

• Last Update Posted (Actual): March 4, 2021
• Last Update Submitted That Met QC Criteria: February 28, 2021
• Last Verified: February 1, 2021

More Information

Terms related to this study

• Keywords: Cardiopulmonary exercise testing,; Double aortic arch
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Congenital Abnormalities; Cardiovascular Abnormalities; Vascular Malformations; Aortic Diseases; Aortic Arch Syndromes; Vascular Ring
• Other Study ID Numbers: RMB-0218-17

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No