Heterotaxy Syndrome and Intestinal Rotation Abnormalities - A Prospective Study

NCT01591928 | Completed

• 1 other identifier: 2012-LR-01
• Study Type: observational
• Target: 40
• Locations: 1 country
• Sites: 1

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.

Conditions

Heterotaxy Syndrome

Keywords

Pediatrics; Congenital heart disease; Heterotaxy syndrome; Intestinal malrotation

Outcome Measures

Primary Outcomes (1)

• Midgut volvulus

  Surgical intervention

  First year of life

Secondary Outcomes (2)

• Morbidity secondary to a prophylactic Ladd procedure

  Post Ladd procedure

• Mortality secondary to a prophylactic Ladd procedure

  Within one month of Ladd procedure

Study Arms (1)

Infants with heterotaxy syndrome

Eligibility Criteria

• Age: 1 Day - 6 Months
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17)
• Sampling Method: Non-Probability Sample

Study Population

All infants with heteterotaxy syndrome whose families provide written informed consent.

You may qualify if:

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

Sponsors & Collaborators

• University of Alberta: lead

Study Sites (1)

Stollery Children's Hospital

Edmonton, Alberta, T6G 2B7, Canada

Location

MeSH Terms

Conditions

Heterotaxy Syndrome; Heart Defects, Congenital; Volvulus Of Midgut

Condition Hierarchy (Ancestors)

Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Splenic Diseases; Lymphatic Diseases; Hemic and Lymphatic Diseases; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Lindsay Ryerson, MD

  University of Alberta

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Assistant Professor

Study Record Dates

• First Submitted: May 2, 2012
• First Posted: May 4, 2012
• Study Start: March 1, 2012
• Primary Completion: December 1, 2016
• Study Completion: December 1, 2016
• Last Updated: December 19, 2018

Record last verified: 2018-12

Data Sharing

• IPD Sharing: Will not share

Locations

CA (1)