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Abstract
Introduction The hybrid procedure (HP) is an alternative first step palliation in children with hypoplastic left heart syndrome (HLHS). Following the HP, patients undergo one of two pathways: ‘three-stage’ - the comprehensive stage II operation (CSII) followed by the total cavopulmonary connexion (TCPC); or ‘four-stage’: the Norwood procedure, the hemi-Fontan operation (HF), and finally TCPC. Decision to proceed depends on physiological and imaging determinants. This study describes changes in somatic growth observed in patients in both operative pathways.
Methods 55 patients with HLHS who underwent HP as first stage palliation were included. Weight-for-age z-score (WFAZ) and height-for-age z-score (HFAZ) were measured at each operative stage.
Results Weight (Figure 1): Three-stage: Patients were of low-normal weight at time of HP (WFAZ -0.3 ± 1.0). Weight decreased to time of CSII (WFAZ -0.8 ± 1.2), then increased significantly to TCPC (WFAZ -0.6 ± 1.4, p = 0.014).
Weight: Four-stage: Patients were also of low-normal weight at HP (WFAZ -0.8 ± 1.2). Weight decreased significantly from HP to Norwood procedure (WFAZ-2.8 ± 1.7. p = 0.03). Weight stabilised between Norwood and HF, and then increased significantly to TCPC (WFAZ -1.1 ± 0.8, p = 0.036).
Height (Figure 2): Three-stage: Height declined steadily from HP to TCPC. Significant decrease in height was observed between CSII and TCPC (p < 0.01).
Height: Four-stage: Height decreased from HP to Norwood, then to HF. Height increased from HF to TCPC. The changes were non-significant.
Conclusions Patients who undergo HP have poor weight gain prior to their superior cavopulmonary connexion (SCPC), as evidenced by decline in WFAZ scores. This was more prominent in the 4-stage group. Weight returned to baseline levels by time of TCPC. The early decline in weight may result from the volume overloading, cyanosis, increased myocardial work of the single ventricle and additional energy expenditure, factors which are corrected at SCPC. The changes in weight are similar to the somatic growth pattern observed in patients undergoing Norwood procedure as first stage palliation. Study of growth data identifies key time points for optimising interventions to address poor growth, a known risk factor for morbidity, prolonged hospitalisation and worse neurodevelopmental outcomes.