Abstract

Between January, 1963 and June, 1985, 67 patients underwent corrective surgery for this complex anomaly. Symptoms and signs of atrial septal defect were dominant in the majority of subjects. History of rheumatic fever (46%), paroxysmal nocturnal dyspnea (33%), presence of an opening snap, mitral diastolic murmur, or pansystolic murmur provided clinical clues to document assiciated mitral valve disease. Open mitral valvotomy was accomplished in 39 subjects, while in the remainder (28 subjects) the valve required replacement. Partial anomalous venous connection was encountered in 12 subjects. Recognition and attention to the associated tricuspid incompetence is a high priority, and 21 subjects underwent concomitant annuloplasty. The overall hospital mortality was 13.4%, with no deaths in the last 22 consecutive patients. The period of follow-up ranged from 1 year to 22 years, with a mean ± SD of 9.34 ± 6.61 years. We belleve, with other authors, that since the hemodynamic and therapeutic considerations are very similar, both the stenotic and regurgitant lesions should be included in the same syndrome.

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