Ivemark Syndrome is a rare heterotaxy syndrome affecting multiple organs. Mortality is common during infancy, but some patients may be found surviving to old age.
Alternative term: right atrial isomerism, asplenia-cardiovascular defect-heterotaxy, renal-hepatic-pancreatic dysplasia
Clinical findings include:
(1) congenital cardiopulmonary abnormalities (dextrocardia, anomalous pulmonary venous return, two right-sided lungs, 2 right-sided atria, septal defects, right ventricular hyperplasia, double-outlet right ventricle, valvular stenosis, transposition of the great vessels, other) often with cyanosis
(2) asplenia or polysplenia
(3) midline liver and and gallbladder
(4) intestinal malrotation and malposition, with left-sided colon and right-sided stomach and small intestine
(5) variable vena cava anomalies
(6) variable aortic anomalies
(7) variable polycystic kidneys
(8) variable agenesis of the corpus callosum
(9) variable pancreatic dysplasia
