Canadian Approach to Hybrid HLHS
When introduced in to clinical practice, bilateral pulmonary artery banding and stenting of the arterial duct added a further dimension to management strategies for hypoplastic left heart syndrome (HLHS) by allowing a delayed arch reconstruction until the infant was ~4 months of age [1, 2]. The procedure was attractive, as postoperative care after the Norwood procedure was challenging, the infants not infrequently in a low cardiac output state for several days with over-circulation and myocardial ischemia an ever-present concern. Furthermore from a neurological standpoint, it was anticipated that the more mature infant would tolerate bypass with less central nervous system compromise during the so-called comprehensive stage II (arch and pulmonary artery reconstruction, stent removal, bidirectional cavopulmonary connection). Early investigations identified a number of drawbacks to this staged approach (i.e., the primary hybrid procedure), which included the risk of retrograde arch obstruction from the ductal stent , loose bands , difficulties management of the size of the atrial septum , low systemic blood flow immediately after the procedure , and problems with left pulmonary artery hypoplasia after comprehensive stage II  to name a few. Despite these limitations we enthusiastically embraced this as one of the treatment strategies offered to parents of infants with HLHS. However, our program approach to the infant with HLHS was lacking as not being uniform, i.e., not protocolized, but rather reflected the biases of the parents (having gone to the web) and the referring physicians. As such, questions arose as to where to place the hybrid procedure in the overall treatment algorithm. An interesting analogy to the adoption of a management option (or new innovation) can be seen in the Gartner Hype Cycle , which is a graphical representation of the adoption of a new technology (Fig. 31.1a). Under this construct, the innovation, in this case the hybrid procedure, undergoes rapid incorporation into clinical management, the so-called ‘boom’ phase. After widespread application, weaknesses in the strategy become apparent and centers either abandon the procedure, do not incorporate it into their treatment algorithm, work through the identified problems (e.g., impact of an arch coarctation, management of the atrial septum), or identify subgroups where it may be a better alternative to standard therapy (i.e., a primary Norwood) (Fig. 31.1b). The adoption of the hybrid innovation (boom phase) in our unit followed that pathway closely, as can be seen in Fig. 31.2, with rapid introduction of the procedure into clinical practice .
KeywordsHypoplastic Left Heart Syndrome Hybrid Procedure Atrial Septum Norwood Procedure Ductal Stenting
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