Asplenic and polysplenic syndrome

Sometimes cardiovascular malformation can be part of larger features in which besides the heart diseases other anomalies are constantly present. Asplenia is characterized  by the absence of spleen and anomalous position of heart and abdominal internal organs and the tendency of pair organs to have a right morphology. Heart diseases in this syndrome are generally extremely complex. The children because of the absence of spleen are exposed to infective complications usually severe; they must have a continuous infective prophylaxis which is obtained with vaccination and antibiotics therapy.

Polyslenia is characterized by the presence of several little spleens, anomalous position of abdominal internal organs and the tendency of pair organs to have a left morphology. Heart diseases are frequent in this syndrome but less severe.

 

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