Hypoplastic Left Heart Syndrome: Treatment Options

Abstract

The publication by Norwood and colleagues from Boston Children’s Hospital in 1983 represented a paradigm shift in the care of children with hypoplastic left heart syndrome (HLHS) (Norwood et al. 1983). Prior to this surgical innovation, children were given compassionate care only and survival without intervention was and remains zero at 1 year (Siffel et al. 2015). The focus of this chapter will be on the first-stage reconstruction, and interstage period of children with HLHS, bidirectional Glenn/Hemi-Fontan, and the modified Fontan are covered elsewhere. Early operative mortality for stage I reconstruction was significant, 34 % at 30 days, but has improved significantly in a number of centers (Murdison et al. 1990; Hraska et al. 2000; McGuirk et al. 2006). The initial operation has also changed significantly over time, from the central shunt originally described to modified Blalock-Taussig shunts with a tendency toward smaller shunt size. Although the Norwood operation provided children with HLHS an opportunity for surgical intervention, a number of children were still dying on the first postoperative day from acute cardiovascular collapse. Jobes and colleagues at Philadelphia Children’s Hospital were able to demonstrate that the precise control of CO₂ levels would ameliorate the coronary steal and provide a more optimal postoperative course (Jobes et al. 1992). Despite this, discovery balance of the systemic and pulmonary circulations remained problematic and sudden cardiovascular collapse was not completely eliminated and mortality in the late 1990s remained in 15–20 %(Bartram et al. 1997).