Journal
INTERACTIVE CARDIOVASCULAR AND THORACIC SURGERY
Volume 33, Issue 4, Pages 588-596Publisher
OXFORD UNIV PRESS
DOI: 10.1093/icvts/ivab121
Keywords
Subaortic stenosis; Congenital heart disease; Left ventricular outflow tract
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This study identified significant differences in reintervention risk in children with subaortic stenosis based on discrete and tunnel-type SAS morphology, with independent risk factors including postoperative gradient, SAS morphology, hypoplastic aortic annulus, and age at intervention. Additionally, older children with a low LVOT gradient at diagnosis showed minimal progression during follow-up, supporting an expectative approach.
OBJECTIVES: Surgical repair of subaortic stenosis (SAS) is associated with a substantial reoperation risk. We aimed to identify risk factors for reintervention in relation to discrete and tunnel-type SAS morphology. METHODS: Single-centre retrospective study of paediatric SAS diagnosed between 1992 and 2017. Multivariable Cox regression analysis was performed to identify reintervention risk factors. RESULTS: Eighty-five children [median age 2.5 (0.7-6.5) years at diagnosis] with a median follow-up of 10.1 (5.5-16.4) years were included. Surgery was executed in 83% (n = 71). Freedom from reoperation was 88 +/- 5% at 5 years and 82 +/- 6% at 10years for discrete SAS, compared to, respectively, 33 +/- 16% and 17 +/- 14% for tunnel-type SAS (log-rank P <0.001). Independent risk factors for reintervention were a postoperative gradient >20 mmHg [hazard ratio (HR) 6.56, 95% confidence interval (CI) 1.41-24.1; P= 0.005], tunnel-type SAS (HR 7.46, 95% CI 2.48-22.49; P < 0.001), aortic annulus z-score <-2 (HR 11.07, 95% CI 3.03-40.47; P < 0.001) and age at intervention <2 years (HR 3.24, 95% CI 1.09-9.86; P = 0.035). Addition of septal myectomy at initial intervention was not associated with lesser reintervention. Fourteen children with a lower left ventricular outflow tract (LVOT) gradient (P < 0.001) and older age at diagnosis (P = 0.024) were followed expectatively. CONCLUSIONS: Children with SAS remain at risk for reintervention, despite initially effective LVOT relief. Regardless of SAS morphology, age <2 years at first intervention, a postoperative gradient >20 mmHg and presence of a hypoplastic aortic annulus are independent risk factors for reintervention. More extensive LVOT surgery might be considered at an earlier stage in these children. SAS presenting in older children with a low LVOT gradient at diagnosis shows little progression, justifying an expectative approach.
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