Syndrome of valvular pulmonary stenosis and valvular aortic stenosis with atrial septal defect.
Clinical and morphological findings are described in 2 adults with combined valvular pulmonary and aortic stenosis with atrial septal defect, an extremely rare combination of lesions. The direction of the shunt via the atrial septal defect in this combination appears to be dependent on the relative degree of outflow obstruction on the right side compared with the left side of the heart. In 1 patient the degree of obstruction at the pulmonary valve was nearly three times that at the aortic valve and, therefore, the shunt was right to left and the patient was severely cyanotic. In the other patient, the degree of obstruction at the pulmonary valve was slightly less than that at the aortic valve and, therefore, the shunt was entirely left to right and the patient was acyanotic. Diagnosis of an associated obstructive lesion on the left side of the heart in a patient with an obstructive lesion on the right side of the heart is essential because operative relief of the right-sided obstruction without relief of the left-sided obstruction may lead to rapid, fatal pulmonary congestion.
D000328:Adult; D001024:Aortic Valve Stenosis / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D004562:Electrocardiography; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011666:Pulmonary Valve Stenosis / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D013577:Syndrome