X-linked, right/left transposition of thoracic and/or abdominal organs. This condition is caused by mutations in the ZIC3 gene, is inherited in an X-linked recessive fashion , and is usually seen in males. Physical features include heart abnormalities such as dextrocardia , transposition of great vessels , ventricular septal defect , patent ductus arteriosus , pulmonic stenosis ; situs inversus, and missing (asplenia) and/or extra spleens (polysplenia).Affected individualscan also experience abnormalities in the development of the midline of the body, which can cause holoprosencephaly , myelomeningocele , urological anomalies, widely spaced eyes (hypertelorism), cleft palate , and abnormalities of the sacral spine and anus. Heterotaxia with recurrent respiratory infections are called primary ciliary dyskinesia . The phenotype is EFO_0009136 (heterotaxy, visceral, x-linked).