Cardiac Malpositions With Situs Ambiguus

In this group, the patient's heart may be either left- or right-sided. The situs is ambiguous because the aortic arch and the stomach are not on the same side. Under these circumstances, we are dealing with either asplenia or polysplenia syndrome. Patients with polysplenia syndrome tend to be acyanotic, running a milder clinical course, and frequently survive into adulthood. The associated lesions are bilateral left-sidedness, interruption of the inferior vena cava with azygos continuation (see Fig. 12-4), polysplenia, and a left-to-right shunt, most frequently an atrioventricular septal defect.17 Patients with asplenia syndrome, on the other hand, tend to be cyanotic and critically ill and die in infancy. The associated lesions are bilateral right-sidedness, asplenia, midline liver, and pulmonary stenosis or atresia with oligemic lungs. It is noteworthy that interruption of inferior vena cava has never been reported in patients with asplenia.

Other Abnormalities

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Essentials of Human Physiology

Essentials of Human Physiology

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