Abstract

Palliation of hypoplastic left-heart syndrome involves use of the morphologic right ventricle as the systemic ventricle and the tricuspid valve (in cases of mitral atresia/stenosis) or the common atrioventricular valve (in cases of malaligned atrioventricular canal) as the systemic atrioventricular valve. To determine the relationship between tricuspid or common atrioventricular valve function and the ultimate outcome of palliative surgery, 100 patients with hypoplastic left-heart syndrome were evaluated preoperatively by Doppler echocardiography to determine the degree of tricuspid regurgitation. These patients were then followed serially to assess changes with time in the functional status of the tricuspid or common atrioventricular valve and to determine the correlation of tricuspid or common atrioventricular valve regurgitation with survival. We discovered that tricuspid or common atrioventricular valve regurgitation is common in hypoplastic left-heart syndrome. Thirty-seven percent of the patients had mild, 13% had moderate, and 3% had severe tricuspid or common atrioventricular valve regurgitation on their preoperative Doppler echocardiograms. Throughout the first 2 postoperative years most patients had no significant change in the degree of tricuspid or common atrioventricular valve regurgitation when findings were compared to those of the preoperative echocardiogram. Patients with moderate or severe tricuspid or common atrioventricular valve regurgitation preoperatively had a significant reduction in their survival when contrasted with patients with no or mild atrioventricular valve regurgitation. We therefore conclude that tricuspid or common atrioventricular valve competence is a significant factor in long-term survival after palliative surgery for hypoplastic left-heart syndrome. This function, however, appears to be unaffected by palliation and remains relatively constant over the first 2 postoperative years.

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