Heterotaxy Syndrome and Intestinal Rotation Abnormalities - A Prospective Study

Completed

• Conditions: Heterotaxy Syndrome

• Registration Number: NCT01591928
• Lead Sponsor: University of Alberta

Brief Summary

Infants with heterotaxy syndrome (HS) are born with an abnormal arrangement of organs along the right-left body axis. Abnormalities of intestinal rotation and fixation are commonly associated with HS. Malrotation is the most worrisome intestinal rotation abnormality (IRA). Advances in cardiac surgery have improved HS mortality such that there is increasing attention to IRA and their management. The objective of this research project is to prospectively observe a cohort of infants with HS and IRA and evaluate their long term outcomes. Specifically, the investigators would like to determine what is the natural history of asymptomatic IRA in patients with HS and what is the morbidity and mortality secondary to an elective Ladd procedure for asymptomatic IRA in a population with HS? The investigators plan a prospective, multi-center, observational study to follow this complicated group of patients. This will be a web-based database collected from major cardiac tertiary care centers in both Canada and the United States. Patients with HS will be recruited by their primary site and clinical data will be collected by their primary site prospectively throughout childhood until they are at least five years of age. This patient population will be followed by their own clinical care givers; this is not an interventional study. No additional clinic visits will be required and the patients will not have to be contacted. Patient medical records will be accessed by a member of the study team at the primary site at least once per year or more frequently if interventions are required or complications develop.

Detailed Description

Not available

Study Timeline

• Study Start: 2012-03
• Study First Submitted: 2012-05-02
• Study First Submitted QC: 2012-05-03
• Study First Posted: 2012-05-04
• Primary Completion: 2016-12
• Study Completion: 2016-12
• Status Verified: 2018-12
• Last Update Submitted: 2018-12-14
• Last Update Posted: 2018-12-19

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 40

Inclusion Criteria

• All infants less than or equal to six months of age with a new diagnosis of heterotaxy syndrome

Exclusion Criteria

Not provided

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Midgut volvulus	First year of life	Surgical intervention

Secondary Outcome Measures

Name	Time	Method
Mortality secondary to a prophylactic Ladd procedure	Within one month of Ladd procedure
Morbidity secondary to a prophylactic Ladd procedure	Post Ladd procedure

Trial Locations

Locations (1)

Stollery Children's Hospital; 🇨🇦 Edmonton, Alberta, Canada