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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: the anatomy of the cardiac mass, the conduit position and the azygous drainage. The main mass of ventricle is on the same side of the azygous drainage and the hepatic conduit position is inevitably on the contralateral side of the azygous drainage. B: the relationship between the conduit position and the azygous drainage site is related to the pattern of persistent pulmonary arteriovenous fistula after Fontan completion.28) PAB: pulmonary artery banding, COA: coactation of aorta, DS: Damus-Kaye-Stansel procedure, TCPS: total cavo-pulmonary shunt.
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Figure 5: A: the anatomy of the cardiac mass, the conduit position and the azygous drainage. The main mass of ventricle is on the same side of the azygous drainage and the hepatic conduit position is inevitably on the contralateral side of the azygous drainage. B: the relationship between the conduit position and the azygous drainage site is related to the pattern of persistent pulmonary arteriovenous fistula after Fontan completion.28) PAB: pulmonary artery banding, COA: coactation of aorta, DS: Damus-Kaye-Stansel procedure, TCPS: total cavo-pulmonary shunt.

Mentions: Several reports demonstrated the development of pulmonary arteriovenous fistulas after the Fontan procedure.28) In these cases, it was closely associated with uneven perfusion of hepatic venous drainage and it excluded hepatic blood flow from the pulmonary circulation, similar to the Kawashima operation. These patients had left isomerism with an interrupted inferior vena cava, and had the conduit position on the contralateral side to the superior vena cava with azygous drainage (Fig. 5). It is essential to design the hepatic venous conduit for achieving balanced perfusion to the lungs bilaterally, and sometimes it is not easy to do so. The reason is that the position of the conduit may be inevitably in the contralateral side of the azygous continuation, if the main mass of ventricle is in the same side of the azygous continuation (Fig. 5).


Heterotaxy syndrome.

Kim SJ - Korean Circ J (2011)

A: the anatomy of the cardiac mass, the conduit position and the azygous drainage. The main mass of ventricle is on the same side of the azygous drainage and the hepatic conduit position is inevitably on the contralateral side of the azygous drainage. B: the relationship between the conduit position and the azygous drainage site is related to the pattern of persistent pulmonary arteriovenous fistula after Fontan completion.28) PAB: pulmonary artery banding, COA: coactation of aorta, DS: Damus-Kaye-Stansel procedure, TCPS: total cavo-pulmonary shunt.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 5: A: the anatomy of the cardiac mass, the conduit position and the azygous drainage. The main mass of ventricle is on the same side of the azygous drainage and the hepatic conduit position is inevitably on the contralateral side of the azygous drainage. B: the relationship between the conduit position and the azygous drainage site is related to the pattern of persistent pulmonary arteriovenous fistula after Fontan completion.28) PAB: pulmonary artery banding, COA: coactation of aorta, DS: Damus-Kaye-Stansel procedure, TCPS: total cavo-pulmonary shunt.
Mentions: Several reports demonstrated the development of pulmonary arteriovenous fistulas after the Fontan procedure.28) In these cases, it was closely associated with uneven perfusion of hepatic venous drainage and it excluded hepatic blood flow from the pulmonary circulation, similar to the Kawashima operation. These patients had left isomerism with an interrupted inferior vena cava, and had the conduit position on the contralateral side to the superior vena cava with azygous drainage (Fig. 5). It is essential to design the hepatic venous conduit for achieving balanced perfusion to the lungs bilaterally, and sometimes it is not easy to do so. The reason is that the position of the conduit may be inevitably in the contralateral side of the azygous continuation, if the main mass of ventricle is in the same side of the azygous continuation (Fig. 5).

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