15 years of Giessen hybrid approach to the hypoplastic left heart

Objective: Since the classical Norwood pathway became treatment of choice for patients with hypoplastic left heart syndrome (HLH-S), this therapy experienced a continous improvement in its technical approach as well as in its results. In Giessen we started an alternative pathway to the therapy of HLH-S (yndrome) or -C (omplex) in 1998 consisting of interventional ductal stenting + surgical bilateral pulmonary artery banding (stage I) as an hybrid therapy followed by aortic arch reconstruction + bidirectional Glenn anastomosis (comprehensive stage II) or, in cases of HLHC, biventricular correction (BVR) after 4 to 6 months.

Methods: Between 1998 and 2013 107 patients (pts) with the diagnosis HLHS (MA/AA, MS/AA, MA/AS/ MS/AS + variants) underwent stage I of the Giessen hybrid approach. 84 pts were treated after an interstage period of 4 to 6 months with the Stage II procedure. The creating of the Fontan pathway was completed creating a total cavopulmonary anastomosis (Stage III) in 51 pts. Previous to Stage II, 7 patients showed an adequate growth of the left ventricle subsequently undergoing biventricular repair. 7 children not eligible for a further treatment were transplanted in different stages of our approach.

Results: Out of the 107 patients undergoing Stage I, 105 (98,1%) survived. Interstage mortality was n = 7 (6,6%). 91 pts. reached Stage II (5 pts. still awaiting). These patients were either treated with a Stage II procedure (n = 84), or BVR (n = 7). Comprehensive StageII mortality was n = 7 (8%), but no mortality in BVR pts. Fontan circulation is completed in 54 pts, 21 pts. are awaiting the completion. Overall survival is 81% (n = 87 pts.). The freedom from death or HTX in our cohort is 75% after 15 years.

Conclusion: The Giessen hybrid approach is an alternative to the conventional treatment of children with HLHS and HLHC. Due to avoiding surgery in the early postnatal period, newborns with HLHS/C have the chance of an advanced postnatal decision finding, more time to recover from postnatal low cardiac output, a chance for biventricular correction or even a HTX after a prolonged waiting time. The procedure gives the patients more perspectives at a lower mortality.