Abstract
Heterotaxy syndrome subjects with isomerism also usually have concomitant congenital heart defects (CHD). Isomerism is a complex syndrome that happens when the main axis of the body fails to rotate properly during developing in the womb in early embryonic period. Many patients have a variable range of severe CHD, while a lesser percentage have none or mild cardiac problems, and also each individual with heterotaxy is only one of its kind. A number of these patients have pulmonary complications, while for others the main concern is their gastrointestinal or immune systems. Indeed heterotaxy syndrome is characterized by a wide range of cardiac and extra-cardiac congenital defects [1, 2].
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References
Zhu L, Belmont JW, Ware SM. Genetics of human heterotaxias. Eur J Hum Genet. 2006;14:17–25.
Taketazu M, Lougheed J, Yoo SJ, Lim JS, Hornberger LK. Spectrum of cardiovascular disease, accuracy of diagnosis, and outcome in fetal heterotaxy syndrome. Am J Cardiol. 2006;97:720–4.
Lim JS, McCrindle BW, Smallhorn JF, Golding F, Caldarone CA, Taketazu M, Jaeggi ET. Clinical features, management, and outcome of children with fetal and postnatal diagnoses of isomerism syndromes. Circulation. 2005;112:2454–61.
Matthew Swisher, Richard Jonas, Xin Tian, et al. Increased postoperative and respiratory complications in patients with congenital heart disease associated with heterotaxy. J Thorac Cardiovasc Surg. 2011;141:637–644.e3.
Barbato A, et al. Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children. Eur Respir J. 2009;34:1264–76.
Kennedy MP, Omran H, Leigh MW, Dell S, Morgan L, Molina PL, Robinson BV, Minnix SL, Olbrich H, Severin T, Ahrens P, Lange L, Morillas HN, Noone PG, Zariwala MA, Knowles MR. Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia. Circulation. 2007;115:2814–21.
Icardo JM, de Vega Sanchez MJ. Spectrum of heart malformations in mice with situs solitus, situs inversus, and associated visceral heterotaxy. Circulation. 1991;84:2547–58.
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© 2014 Springer-Verlag London
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Alizadehasl, A., Sadeghpour, A. (2014). Heterotaxy Syndrome. In: Sadeghpour, A., Kyavar, M., Alizadehasl, A. (eds) Comprehensive Approach to Adult Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-6383-1_31
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DOI: https://doi.org/10.1007/978-1-4471-6383-1_31
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