Key result
Interstage growth failure in infants with hypoplastic left heart syndrome was associated with a significantly higher incidence of death or transplant prior to Fontan completion compared to those with adequate growth (39% vs 16%, p=0.03).
Why the study?
Because somatic growth is a potentially modifiable determinant of interstage mortality in infants with hypoplastic left heart syndrome, this study evaluated the incidence and risk factors of growth failure during an interstage monitoring program.
Does interstage growth failure increase the risk of death or transplant and delay Stage 2 palliation in infants with Hypoplastic Left Heart Syndrome?
Cohort (n=72)
No
Does interstage growth failure increase the risk of death or transplant and delay Stage 2 palliation in infants with Hypoplastic Left Heart Syndrome?
Interstage growth failure in infants with Hypoplastic Left Heart Syndrome is common and significantly associated with delayed Stage 2 palliation and increased risk of death or transplant before Stage 3 completion.
Answers are grounded in this paper and cite their sources.
May guide growth optimization within HLHS interstage protocols; leaves open whether modification improves survival.
Infants with Hypoplastic left heart syndrome (HLHS) are particularly vulnerable during their interstage period which is the time between Stage 1 palliation (S1P) and Stage 2 palliation (S2P). Interstage Monitoring Program (IMP) was established to reduce mortality after discharge following S1P and consists of close monitoring of certain key parameters including hypoxia, growth failure and occurrence of major events. As somatic growth is a potentially modifiable determinant of interstage mortality, we aimed to study the incidence and risk factors of growth failure among infants followed by our IMP over the last 10 years. We included HLHS infants who were enrolled in institutional IMP following discharge after S1P from May 2009 to April 2019. Growth failure was defined as per the NPC-QIC criterion as failure to achieve target interstage weight of 20–30 g per day and risk factors for growth failure were explored. A total of 87 patients were enrolled during the study period, of whom 72 (n = 83%) underwent S2P. About one third (23 patients) failed to achieve the target growth rate despite close monitoring through a robust IMP. Growth failure significantly delayed the time to more stable S2P circulation (median IS duration: 131 days vs 86 days, p = 0.002). Patients with growth failure had a significantly higher incidence of death/transplant prior to Stage 3 (Fontan) completion (39% vs 16%, p = 0.03). Interstage growth failure was significantly associated with a “Hybrid-type” of repair during S1P (p = 0.03); and with the need for opioids at discharge (p = 0.04). This study highlights that growth failure is common in HLHS patients, despite active intervention through an IMP program. These patients appear to have significantly worse transplant-free survival rates compared to their counterparts. Pre-interstage risk factors including use of opioids may need to be addressed to assist adequate somatic growth during interstage.
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Bates et al. (2024) conducted a cohort in Hypoplastic left heart syndrome (HLHS) (n=72). Interstage growth failure (≤20 g/day weight gain) vs. Adequate interstage growth (>20 g/day weight gain) was evaluated on Death or heart transplant prior to Stage 3 (Fontan) completion (p=0.03). Interstage growth failure in infants with hypoplastic left heart syndrome was associated with a significantly higher incidence of death or transplant prior to Fontan completion compared to those with adequate growth (39% vs 16%, p=0.03).
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