Racial Distribution of Heterotaxy Syndrome

Study Overview
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Brief Summary
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Heterotaxy syndrome is a heterogeneous disease that is the result of a failure of normal right-left lateralization of the abdominal and thoracic organs during development. The major clinical manifestations include intestinal malrotation, functional asplenia and complex cyanotic heart disease. Hypothesis: There exists a yet, un-recognized, racial distribution in heterotaxy syndrome.

Detailed Description
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The exact etiology of heterotaxy is unknown, but presumed to be multifactorial. While candidate genes have been suggested, no common gene has proven responsible for this constellation of lesions. Various modes of inheritance have been described. Though there are reports of familial occurrences, no reports of racial/ethnic predominance exist. We, therefore, propose to examine the racial/ethnic distribution of heterotaxy syndrome with congenital heart disease in patients treated at this institution. In addition, the investigators will determine if race/ethnicity is a factor in clinical outcomes. Aim 1: To determine any pattern of racial/ethnic predominance within a cohort of patients with heterotaxy syndrome with congenital heart disease. Aim 2: To correlate race/ethnicity with disparities in morbidity and mortality in patients with heterotaxy syndrome with congenital heart disease. This study will be a retrospective, chart review of approximately 90 - 100 medical charts, conducted on the Egleston campus of Children's Healthcare of Atlanta. Charts reviewed will cover a period between January 1, 1990 and December 31, 2005. Data to be collected includes: DOB, diagnosis, surgical procedure, surgical outcome, survival, length of hospitalization, repeat surgical intervention, incidence of hospitalization, length of inotropic support, ventilator time, infection rate, insurance provider, list of antibiotics and zip code of residence at initial admission. In addition to the above information, data to be extracted from the medical chart of a decedent will include age at death, cause of death, laboratory data at time of death and autopsy results.

Official Title
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Racial Distribution of Heterotaxy Syndrome and Effects on Clinical Outcomes Protocol

Conditions
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Congenital Disorders

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: All medical charts diagnosed clinically and at autopsy with heterotaxy syndrome, left atrial isomerism, right atrial isomerism or situs inversus totalis with congenital heart disease over a 16 consecutive year period at Children's Healthcare of Atlanta at Egleston. Exclusion Criteria: Those who do not meet Inclusion Criteria

Ages Eligible for Study
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Maximum Age: 21 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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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Heterotaxy Syndrome, Intestinal Malrotation, Functional Asplenia, Complex Cyanotic heart disease