The effects of heterotaxy may be subtle but are almost always present and can have significant clinical implications

We read with great interest the case report by Ali and colleagues in which they describe the case of a young lady found to have heterotaxy. Consisting of isomeric findings in the various organ systems that can often be diagnosed during fetal life, this entity is better described as bodily isomerism. The authors claim that and ldquo;Heterotaxy syndrome is usually asymptomatic and rdquo; although this is not accurate. Intra-cardiac lesions or abnormalities of systemic venous return are found in almost all, malrotation is noted in 40 to 90%, and splenic abnormalities are noted in over 75% of patients. While these are anatomic findings there are functional implications of these findings. Those with multiple spleens or even a single, normally located spleen can have functional asplenia with higher risk of bacteremia, thrombocytosis, and thrombosis, those with left bronchial isomerism are at higher risk for increased frequency of sinopulmonary symptoms, and those with heterotaxy may also have subtle attention and behavior deficits. Heterotaxy should not be taken lightly when it is come across and should warrant thorough evaluation of multiple organ systems. While functional implications of heterotaxy may be subtle, they usually are present. In fact, a majority of patients with heterotaxy will have significant cardiopulmonary effects, likely due to underlying ciliary dyskinesia.