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Heterotaxy syndrome.

Kim SJ - Korean Circ J (2011)

Bottom Line: Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body.This broad term includes patients with a wide variety of very complex cardiac lesions.Recent advances in medical management, and improvements in surgical techniques have resulted in improved survival for these patients, and the surgical outcomes are comparable to those with Fontan circulation irrespective of the presence or absence of heterotaxy.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Konkuk University Hospital, Seoul, Korea.

ABSTRACT
Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. This broad term includes patients with a wide variety of very complex cardiac lesions. Patients with heterotaxy can be stratified into the subsets of asplenia syndrome and polysplenia syndrome, or the subsets of heterotaxy with isomerism of the right atrial appendages and heterotaxy with isomerism of the left atrial appendages. Treatment of patients with isomerism is determined by the nature and severity of the associated cardiac and extracardiac lesions. Most cardiac operations for patients with isomerism are palliative in nature, since normal anatomy is rarely achieved and mortality rates remain high for patients with heterotaxy syndrome. Patients with left isomerism in general have less severe cardiac malformations than those with right isomerism and, hence, more chance of biventricular repair. For almost all patients with right isomerism, and for many with left isomerism, biventricular repair will not be feasible, and all palliative protocols are then staging procedures prior to a Fontan-type repair. Recent advances in medical management, and improvements in surgical techniques have resulted in improved survival for these patients, and the surgical outcomes are comparable to those with Fontan circulation irrespective of the presence or absence of heterotaxy.

No MeSH data available.


Related in: MedlinePlus

A venovenous collateral.25)
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Figure 2: A venovenous collateral.25)

Mentions: After a neonatal palliative procedure, most can opt only for surgical palliation directed toward function as a single ventricle such as with bidirectional cavopulmonary shunts, and, finally, modified Fontan procedures.23) When there is interruption of the inferior caval vein, bidirectional cavopulmonary anastomosis (Kawashima operation)24) will result in all of the systemic venous return being diverted to the lungs except for that returning from the hepatic veins. It has been widely used in patients with left isomerism and is similar to the completion of a fenestrated Fontan circulation. Moreover, these procedures have many late outcomes such as venovenous collaterals25) and the development of pulmonary arteriovenous fistulas (Figs. 2 and 3).26)27) Patients with left isomerism seem to be particularly prone to this complication. There is increasing evidence that a hepatic factor may be important in preventing this development. Therefore, a Kawashima operation requires subsequent completion of a Fontan procedure.28)29)


Heterotaxy syndrome.

Kim SJ - Korean Circ J (2011)

A venovenous collateral.25)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3116098&req=5

Figure 2: A venovenous collateral.25)
Mentions: After a neonatal palliative procedure, most can opt only for surgical palliation directed toward function as a single ventricle such as with bidirectional cavopulmonary shunts, and, finally, modified Fontan procedures.23) When there is interruption of the inferior caval vein, bidirectional cavopulmonary anastomosis (Kawashima operation)24) will result in all of the systemic venous return being diverted to the lungs except for that returning from the hepatic veins. It has been widely used in patients with left isomerism and is similar to the completion of a fenestrated Fontan circulation. Moreover, these procedures have many late outcomes such as venovenous collaterals25) and the development of pulmonary arteriovenous fistulas (Figs. 2 and 3).26)27) Patients with left isomerism seem to be particularly prone to this complication. There is increasing evidence that a hepatic factor may be important in preventing this development. Therefore, a Kawashima operation requires subsequent completion of a Fontan procedure.28)29)

Bottom Line: Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body.This broad term includes patients with a wide variety of very complex cardiac lesions.Recent advances in medical management, and improvements in surgical techniques have resulted in improved survival for these patients, and the surgical outcomes are comparable to those with Fontan circulation irrespective of the presence or absence of heterotaxy.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Konkuk University Hospital, Seoul, Korea.

ABSTRACT
Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. This broad term includes patients with a wide variety of very complex cardiac lesions. Patients with heterotaxy can be stratified into the subsets of asplenia syndrome and polysplenia syndrome, or the subsets of heterotaxy with isomerism of the right atrial appendages and heterotaxy with isomerism of the left atrial appendages. Treatment of patients with isomerism is determined by the nature and severity of the associated cardiac and extracardiac lesions. Most cardiac operations for patients with isomerism are palliative in nature, since normal anatomy is rarely achieved and mortality rates remain high for patients with heterotaxy syndrome. Patients with left isomerism in general have less severe cardiac malformations than those with right isomerism and, hence, more chance of biventricular repair. For almost all patients with right isomerism, and for many with left isomerism, biventricular repair will not be feasible, and all palliative protocols are then staging procedures prior to a Fontan-type repair. Recent advances in medical management, and improvements in surgical techniques have resulted in improved survival for these patients, and the surgical outcomes are comparable to those with Fontan circulation irrespective of the presence or absence of heterotaxy.

No MeSH data available.


Related in: MedlinePlus