Hypoplastic Left Heart Syndrome: The Giessen Approach – History, Technique, and Results

Abstract

Patients with small left heart belong to a spectrum of a wide variety of possible combinations of hypoplastic heart structures. The treatment of newborns with hypoplastic left heart syndrome (HLHS) follows a well-established classical three-step algorithm for most institutions worldwide [1–4]. In Giessen, the hybrid approach was developed as a primary palliation for newborns with HLHS and later on for patients with hypoplastic left heart complex (HLHC). The hybrid procedure has moved from a rescue approach to an alternative modality of a Norwood palliation [4–7]. The “Giessen hybrid” stage I consists of surgical bilateral pulmonary artery banding (bPAB) combined with percutaneous stenting of the arterial duct and atrial septum manipulation, if necessary. The lessons learned by the hybrid strategy for the treatment of HLHS, HLHC, and variants have added a novel impulse for treating selected patients with cardiovascular failure beyond the neonatal period [8–10]. In our center meanwhile all types of HLHS and variants are treated with the modified Giessen hybrid strategy [4, 11]. Since June 1998 almost 200 patients received the Giessen hybrid stage I procedure as an initial approach. The physiological objectives of the hybrid approach are similar as for the classical Norwood procedure by alternative techniques in controlling pulmonary blood flow, whereas unobstructed systemic perfusion is maintained via an open arterial duct. This strategy involves off-pump bilateral pulmonary artery banding (bPAB) and interventional stenting or continuous prostaglandin therapy of the ductus arteriosus mostly, but not exclusively performed in the neonatal period (hybrid stage I). Aortic arch reconstruction using cardiopulmonary bypass combined with a superior cavo-pulmonary connection summarized as comprehensive stage II or as indicated a biventricular correction is deferred until the age of 4–8 months [6, 10]. In our institution hybrid therapy has initially been proposed to overcome obvious drawbacks of the classical Norwood operation and aims to improve survival and outcome for these patients in a less invasive way [4, 7]. The hybrid approach could be established as a highly effective treatment in particular for newborns with cardiovascular collapse and even for premature babies or neonates to small for gestational age [4].