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Prevalence and associated risk factors for intervention in 313 children with subaortic stenosis
Tara Karamlou1, Rebecca Gurofsky, Alexandra Bojcevski
1Division of Cardiovascular Surgery, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Delaying subaortic resection in children with subaortic stenosis until the left ventricular (LV) gradient exceeds 30 mm Hg is recommended. Most children with lower LV gradients have stable disease, avoiding early intervention for subaortic stenosis.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiovascular Surgery
Background:
- Subaortic stenosis is a significant cause of left ventricular outflow tract obstruction in children.
- Early intervention for subaortic stenosis is debated, with a 1994 protocol recommending early resection if the LV gradient exceeds 30 mm Hg.
Purpose of the Study:
- To determine the prevalence of intervention for subaortic stenosis in children.
- To identify factors associated with intervention and progression of the LV gradient.
- To evaluate the longitudinal outcomes of the 1994 early resection protocol.
Main Methods:
- Retrospective review of 313 children diagnosed with subaortic stenosis (1975-1998).
- Cox proportional hazard models for intervention prevalence and factors.
- Mixed models of serial echocardiographic data (933 studies) for LV gradient trends.
Main Results:
- Freedom from initial resection was 40% at 16 years.
- Higher initial LV gradient, larger aortic annulus z-score, smaller body surface area, and smaller mitral annulus z-score were associated with earlier resection.
- Initial LV gradient >30 mm Hg, aortic valve thickening, and mitral valve attachment predicted faster LV gradient progression and worse aortic regurgitation.
Conclusions:
- Subaortic resection should be delayed until the LV gradient exceeds 30 mm Hg.
- Most children with initial LV gradients <30 mm Hg exhibit quiescent disease, supporting a conservative approach.
Background:
We sought to determine the prevalence of intervention and associated factors in children presenting with subaortic stenosis. We also investigated whether a protocol adopted in 1994 of early subaortic resection at a preoperative mean systolic gradient across the left ventricular outflow tract (LV gradient) greater than 30 mm Hg was supported by longitudinal outcomes.
Methods:
Record review of all children (n = 313) diagnosed with subaortic stenosis was conducted between 1975 and 1998 at our institution. Cox proportional hazard models determined the prevalence and associated factors for initial subaortic resection. Mixed models of serially obtained echocardiographic data (n = 933) established longitudinal LV gradient trends and identified factors associated with more rapid LV gradient progression.
Results:
Median age at presentation was 8 months. Freedom from initial subaortic resection was 40% at 16 years from diagnosis. Earlier progression to subaortic resection was associated with patient characteristics at presentation, including a higher initial LV gradient (p < 0.001), larger aortic annulus z-score (p = 0.005), smaller body surface area (p < 0.001), and smaller mitral annulus z-score (p = 0.003). Initial resection was also associated with a faster rate of LV gradient progression (p = 0.003). Factors determining the increased rate of LV gradient progression included an initial LV gradient greater than 30 mm Hg (p < 0.001), initial aortic valve thickening (p = 0.003), and attachment of subaortic stenosis to the mitral valve (p = 0.003). Worse aortic regurgitation grade with time was also associated with an initial LV gradient greater than 30 mm Hg (p < 0.001).
Conclusions:
Subaortic resection should be delayed until the LV gradient exceeds 30 mm Hg because most children with an initial LV gradient less than 30 mm Hg have quiescent disease.
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