Heterotaxy

Author:

Jacobs Jeffrey Phillip1,Pasquali Sara K.2,Morales David L. S.3,Jacobs Marshall Lewis4,Mavroudis Constantine4,Chai Paul Jubeong1,Tchervenkov Christo I.5,Lacour-Gayet Francois G.6,Walters Hal7,Quintessenza James Anthony1

Affiliation:

1. The Congenital Heart Institute of Florida (CHIF), All Children’s Hospital and Children’s Hospital of Tampa, University of South Florida College of Medicine, Cardiac Surgical Associates of Florida (CSAoF), Saint Petersburg and Tampa, FL, USA

2. Departments of Pediatrics, Duke University School of Medicine, and Duke Clinical Research Institute, Duke University Medical Center, Durham, NC, USA

3. Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, and Division of Congenital Heart Surgery, Texas Children’s Hospital, Houston, TX, USA

4. Cleveland Clinic Foundation, Cleveland Clinic Lerner School of Medicine, Case Western Reserve University, Cleveland, OH, USA

5. The Montreal Children’s Hospital of the McGill University Health Centre, Montreal, Quebec, Canada

6. Children’s Hospital at Montefiore, New York, NY, USA

7. Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI, USA

Abstract

According to The International Society for Nomenclature of Pediatric and Congenital Heart Disease (ISNPCHD), “Heterotaxy is synonymous with ‘visceral heterotaxy’ and ‘heterotaxy syndrome’. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as ‘situs solitus’, or patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as `situs inversus’.” or patients with complete mirror-image arrangement of the internal organs along the left–right axis, also known as situs inversus. The purpose of this article is to review the data about heterotaxy in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. The investigators examined all index operations in the STS Congenital Heart Surgery Database over 12 years from January 1, 1998 to December 31, 2009, inclusive. This analysis resulted in a cohort of 77 153 total index operations. Of these, 1505 operations (1.95%) were performed in patients with heterotaxy. Of the 1505 index operations performed in patients with heterotaxy, 1144 were in patients with asplenia and 361 were in patients with polysplenia. In every STS -EACTS Congenital Heart Surgery Mortality Category, discharge mortality is higher in patients with heterotaxy compared with patients without heterotaxy (EACTS = European Association for Cardio-Thoracic Surgery). Discharge mortality after systemic to pulmonary artery shunt is 6.6% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 10.8% in single-ventricle patients with heterotaxy. Discharge mortality after Fontan is 1.8% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 4.2% in single-ventricle patients with heterotaxy. The STS Congenital Heart Surgery Database is largest congenital heart surgery database in North America. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that heterotaxy is a challenging problem with increased discharge mortality in most subgroups.

Publisher

SAGE Publications

Subject

Cardiology and Cardiovascular Medicine,General Medicine,Pediatrics, Perinatology, and Child Health,Surgery

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