In right atrial isomerism, both atria of the heart are morphological right atria leading to associated abnormalities in the pulmonary venous system. In addition, individuals with right atrial isomerism develop<unk> , a midline liver, malrotation of the small intestine and the presence of two morphologic right lungs. Individuals with left atrial isomerism, by comparison, have two morphologic left atria, polysplenia, intestinal malrotation and two morphologic left lungs. The majority of cases present at the time of birth or within a few days or weeks. Presenting signs and symptoms of the congenital heart defect may include cyanosis, breathlessness, lethargy and poor feeding.
Asplenia with cardiovascular anomalies
Isolated congenital asplenia
Renal–hepatic–pancreatic dysplasia
Hypertension and brachydactyly syndrome
Umbilical cord ulceration and intestinal atresia
Multisystem inflammatory syndrome in children
Childhood leukemia
Arthrogryposis–renal dysfunction–cholestasis syndrome