Ivemark syndrome is a rare congenital condition that affects multiple organ systems of the body. Ivemark syndrome is classified as a heterotaxy disorder or a laterality disorder. These terms refer to the failure of the internal organs of the chest and abdomen to be arranged in the proper location within the body. It is characterized by the absence (asplenia) or underdevelopment (hypoplasia) of the spleen, heart malformations, and the abnormal arrangement of the internal organs of the chest and abdomen. Symptoms vary greatly depending on the specific abnormalities present; however if heart malformations are complex, the prognosis is often poor. The exact cause of Ivemark syndrome is not known. Most cases are sporadic (isolated and seemingly random). Unlike some other heterotaxy disorders, causative gene mutations have not been identified. There is no cure for Ivemark syndrome. Treatment might include surgical repair of heart malformations when appropriate and prophylactic antibiotic therapy to reduce the incidence of infection due to the absence or poor function of the spleen.
|Signs and Symptoms||Approximate number of patients (when available)|
|Abnormal lung lobation||-|
|Agenesis of corpus callosum||-|
|Anomalous pulmonary venous return||-|
|Atria septal defect||-|
|Autosomal recessive inheritance||-|
|Complete atrioventricular canal defect||-|
|Pulmonary artery atresia||-|
|Right atrial isomerism||-|
|Situs inversus totalis||-|
|Ventricular septal defect||-|
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