TY - JOUR
T1 - Heterotaxy
T2 - Lessons Learned About Patterns of Practice and Outcomes From the Congenital Heart Surgery Database of the Society of Thoracic Surgeons
AU - Jacobs, Jeffrey Phillip
AU - Pasquali, Sara K.
AU - Morales, David L.S.
AU - Jacobs, Marshall Lewis
AU - Mavroudis, Constantine
AU - Chai, Paul Jubeong
AU - Tchervenkov, Christo I.
AU - Lacour-Gayet, Francois G.
AU - Walters, Hal
AU - Quintessenza, James Anthony
PY - 2011/4
Y1 - 2011/4
N2 - 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.
AB - 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.
KW - asplenia
KW - cardiac surgery
KW - congenital heart disease
KW - database
KW - heterotaxy syndrome
KW - isomerism
KW - outcomes
KW - polysplenia
KW - results of treatment
UR - http://www.scopus.com/inward/record.url?scp=84990366456&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84990366456&partnerID=8YFLogxK
U2 - 10.1177/2150135110397670
DO - 10.1177/2150135110397670
M3 - Review article
C2 - 23804985
AN - SCOPUS:84990366456
VL - 2
SP - 278
EP - 286
JO - World journal for pediatric & congenital heart surgery
JF - World journal for pediatric & congenital heart surgery
SN - 2150-1351
IS - 2
ER -