[Hypoplastic left-heart syndrome. Initial intensive care experiences with the Norwood operation in Vienna]

Palliative surgery of the hypoplastic left heart syndrome (HLHS), whereby both pulmonary and systemic circulation are restored, was first described by Norwood in 1983. Careful ventilatory and pharmacologic modulation of the ratio of pulmonary to systemic vascular resistance are a crucial part of pre-, peri- and postoperative management. We report our experience in 3 of 7 newborns with HLHS who underwent the Norwood operation. Hemodynamic and respiratory parameters were evaluated retrospectively in these patients and we analysed the influence of diagnostic and therapeutic interventions on the course of disease before and after operation. During prostaglandin therapy two of three patients required mechanical ventilation preoperatively because of pulmonary hyperperfusion. Decreased myocardial contractility, oliguria and increased pulmonary vascular resistance characterized the postoperative course. The management included a careful application of inotropic support when necessary, adaptation of the ventilatory setting in order to modulate pulmonary perfusion and, in addition, institution of peritoneal dialysis. One patient died from staphylococcus aureus and superinfection with respiratory syncytial virus on day 41 after the operation. Maintaining an optimal balance between pulmonary and systemic blood flow is an essential aspect of postoperative management. Serum lactate and central venous oxygen saturation are helpful parameters in monitoring therapeutic measures in these patients. We conclude from our preliminary experience, that the Norwood operation might be an alternative therapeutic approach for newborns with HLHS in whom heart transplantation is not possible.