A Decade of Staged Norwood Palliation in Hypoplastic Left Heart Syndrome in a Midsized Cardiosurgical Center

Abstract

Hypoplastic left heart syndrome (HLHS) is a challenge for the pediatric cardiologist and the surgeon. It is generally assumed that the postoperative outcome after surgery for congenital heart disease is influenced by the institutional size. We present the results of 43 patients with true HLHS (situs solitus and atrioventricular and ventriculoarterial concordance) referred for operation between 1992 and 2002 in our center. Two children had atrioseptostomy: one died soon after the operation, and the other one was transplanted successfully but died at the age of 6 months following acute rejection. The remaining 41 underwent Norwood I palliation, 21 stage II palliation, and 10 stage III palliation. Early mortality was 29% after stage I operation, 4.7% after stage II palliation, and 0% after stage III operation. Overall mortality was 39% after stage I, 9.5% after stage II, and 10% after stage III operation. Low birth weight was associated with a higher mortality (p < 0.05). Mortality declined with increasing experience, comparable to the results of very large cardiosurgical centers with many more patients. The quality of surgery and perioperative management in smaller pediatric cardiosurgical centers can reach the level of very large centers.

Staged Surgical Palliation in Hypoplastic Left Heart Syndrome and Its Variants

“…Five‐ and ten‐year survival following the Norwood procedure was 51.6%. These figures are consistent with previous reports in which the five‐year survival ranged between 40% and 59% 9,12,15 , 17–21 …”

Improvement in Interstage Survival in a National Pediatric Cardiology Learning Network

“…[1][2][3] Improvement in surgical and postoperative management has led to considerable improvement in early post-Norwood survival in the recent era. [4][5][6][7] However, after the Norwood procedure and before stage 2 palliation, a high-risk time period termed interstage, mortality has been previously been reported at 10% to 15%. [8][9][10] The rare nature of this disorder has limited robust learning about successful strategies to improve survival undertaken by single-surgical centers, and a gap exists in our ability to further improve mortality in this population.…”

Univentricular Heart

“…Overall mortality was recently reported to be 39% after stage I, 9.5% after stage II, and 10% after stage III. 55 A lower-risk subgroup with an 86% hospital survival rate after a Norwood stage I procedure was retrospectively identified. 56 When this staged approach cannot be performed, cardiac transplantation is considered.…”