Early onset neo-aortic valve insufficiency was associated with a 7.9-fold increased risk of mortality and a 5-year survival rate of 38.5% compared to 87.8% in late onset patients.
Observational (n=378)
No
Does the onset timing of significant neo-aortic valve insufficiency (early vs. late) after the Norwood procedure affect mortality and neo-aortic root dilatation in neonates with HLHS?
Early onset neo-aortic insufficiency after the Norwood procedure is associated with high perioperative mortality, whereas late onset is associated with progressive root dilatation requiring long-term surveillance.
Effect estimate: HR 7.87 (95% CI 1.66-37.33)
Absolute Event Rate: 38.5% vs 87.8%
p-value: p=0.009
In hypoplastic left heart syndrome (HLHS) and variants, the pulmonary valve functions as the neo-aortic valve after the Norwood procedure. Neo-aortic valve insufficiency (Neo-AI) represents an emerging complication with implications for long-term survival. This study investigated the clinical impact and patterns of Neo-AI development following staged palliation. Neonates undergoing the Norwood procedure (2001–2023) were retrospectively reviewed. Neo-AI was evaluated using serial echocardiograms, with patients classified into two groups: early onset (during the Norwood hospitalization) and late onset (after the Norwood discharge). Neo-aortic root dimensions were measured at four time points during staged palliation, with Z-scores calculated for patient growth. Among 378 patients, significant Neo-AI developed in 30 (7.9%) during median follow-up of 4.3 years. Early onset neo-AI occurred in 13 patients (43.3%) and late onset neo-AI in 17 (56.7%). Cox regression revealed 7.9-fold increased mortality risk in early onset patients (hazard ratios 7.87, p = 0.009), with 5-year survival of 38.5% versus 87.8% in late onset patients. All early onset deaths occurred within the first year. Late onset neo-AI patients demonstrated progressive sinus of Valsalva dilatation (annual progression 0.33 Z-score units). At Fontan completion, late onset neo-AI patients had larger Z-scores than early onset patients 5.14 vs. 2.18, p = 0.006, while surviving early onset patients demonstrated Neo-AI improvement after bidirectional cavopulmonary shunt with subsequent stable root dimensions. Early onset neo-AI associates with acute hemodynamic compromise and higher perioperative mortality. Late onset neo-AI demonstrates progressive root dilatation with superior survival, raising concerns for future valve intervention.
Matsubara et al. (Sat,) conducted a observational in Hypoplastic Left Heart Syndrome (n=378). Norwood procedure vs. No significant neo-aortic valve insufficiency was evaluated on 5-year survival rates (HR 7.87, 95% CI 1.66-37.33, p=0.009). Early onset neo-aortic valve insufficiency was associated with a 7.9-fold increased risk of mortality and a 5-year survival rate of 38.5% compared to 87.8% in late onset patients.