Korean Circ J.  2011 May;41(5):227-232. 10.4070/kcj.2011.41.5.227.

Heterotaxy Syndrome

• Kim SJ

• KMID: 2297927
• DOI: http://doi.org/10.4070/kcj.2011.41.5.227

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.

Keyword

Heterotaxy; Isomerism

MeSH Terms

Atrial Appendage
Axis, Cervical Vertebra
Heterotaxy Syndrome
Humans
Isomerism

Figure

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  Fig. 1 Typical arrangement of the organs within the body in the setting of visceral heterotaxy.2)

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  Fig. 2 A venovenous collateral.25)

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  Fig. 3 A: a pulmonary angiogram of a patient who underwent the Kawashima operation showing typical pulmonary arteriovenous fistulas. B: lung perfusion scan results were positive for an intrapulmonary right-to-left shunt.26) RSVC: right superior vena cava.

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  Fig. 4 Comparison of Kaplan-Meier curves for cumulative survival between patients with and without heterotaxy syndrome.31)

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  Fig. 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.

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