A 22-year-old female with cyanotic congenital heart disease was found to have veno-arterial shunting via a large secundum atrial septal defect despite normal pulmonary arterial and right ventricular pressures. Persistence of the Eustachian valve of the inferior vena cava was suspected preoperatively and confirmed at operation. This valve caused a large right-to-left shunt by favoring the flow of inferior vena caval blood into the left atrium via the defect. Surgical excision of the abnormal valve and closure of the atrial septal defect resulted in complete physiological and clinical cure.
A 19-year-old male was found to have combined supravalvular aortic stenosis and hypertrophic subaortic stenosis. Two possible hypotheses with supportive evidence in the literature may explain this association: (1) long-standing fixed supravalvular aortic stenosis may result in secondary hypertrophic subaortic stenosis, and (2) infantile hypercalcemia, a condition closely related to supravalvular aortic stenosis, may also be involved by continuous inotropic stimulation in the pathogenesis of muscular hypertrophy and subaortic stenosis of the left ventricle. We tend to favor the second theory in light of recent evidence in the literature.
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