Combined Norwood and cavopulmonary shunt as the first palliation in late presenters with hypoplastic left heart syndrome and single-ventricle lesions

Objective: A primary cavopulmonary shunt as a component of the initial Norwood palliation could be an option in patients with hypoplastic left heart syndrome and single-ventricle lesions. We present our initial experience with this approach in carefully selected patients with unrestricted pulmonary blood flow and low pulmonary vascular resistance. Methods: The study included 16 patients; the mean age was 137.9 +/- 84.2 days. All patients underwent a Norwood palliation consisting of atrial septectomy, Damus-Kaye-Stansel connection, and arch augmentation in addition to the cavopulmonary shunt as the initial palliation. Results: The mean preoperative pulmonary to systemic blood flow (Qp/Qs) ratio on room air (n = 9) and with 100% oxygen (n = 8) was 5.3 +/- 3.2 and 8.6 +/- 4.3, respectively. The mean pulmonary vascular resistance on room air (n = 10) and 100% oxygen (n = 9) was 4.8 +/- 3.1 and 1.7 +/- 0.97 WU/m(2), respectively. Delayed chest closure was needed in 12 patients, and 6 patients required postoperative inhaled nitric oxide. One patient underwent takedown of the cavopulmonary shunt and construction of the right ventricle to pulmonary artery conduit after 1 month. The mean intensive care unit stay was 18.9 +/- 15.4 days. There were 2 in-hospital deaths (48 hours and 8 days after surgery) and 2 postdischarge deaths (6 months and 2 years after hospital discharge). Seven patients have undergone the Fontan completion successfully, and 5 patients await further surgery. Conclusions: First-stage Norwood palliation with cavopulmonary shunt for patients with hypoplastic left heart syndrome or single-ventricle lesions is feasible in late presenters with low pulmonary vascular resistance.

Automated summary

This study demonstrates the feasibility of using a primary cavopulmonary shunt as part of the initial Norwood palliation in patients with unrestricted pulmonary blood flow and low pulmonary vascular resistance. Delayed chest closure and postoperative inhaled nitric oxide were required in some patients, but overall results suggest that the initial use of the cavopulmonary shunt is effective and provides a foundation for further surgical strategies.