Hypoplastic left heart syndrome. First experiences in intensive care afterthe Norwood operation in Vienna

Palliative surgery of the hypoplastic left heart syndrome (HLHS), whereby b oth pulmonary and systemic circulation are restored, was first described by Norwood in 1983. Careful ventilatory and pharmacologic modulation of the r atio 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 Nor wood operation. Hemodynamic and respiratory parameters were evaluated retro spectively in these patients and we analysed the influence of diagnostic an d therapeutic interventions on the course of disease before and after opera tion. During prostaglandin therapy two of three patients required mechanical vent ilation preoperatively because of pulmonary hyperperfusion. Decreased myoca rdial contractility, oliguria and increased pulmonary vascular resistance c haracterized the postoperative course. The management included a careful ap plication of inotropic support when necessary, adaptation of the ventilator y setting in order to modulate pulmonary perfusion and, in addition, instit ution of peritoneal dialysis. One patient died from staphylococcus aureus a nd superinfection with respiratory syncytial virus on day 41 after the oper ation. 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, th at the Norwood operation might be an alternative therapeutic approach for n ewborns with HLHS in whom heart transplantation is not possible.