Abstract

BackgroundData on the outcomes of hypertrophic cardiomyopathy (HCM) with biventricular obstruction are limited.ObjectiveOur aim is to compare mid-term outcomes of biventricular outflow tract obstruction (BVOTO) HCM, left ventricular outflow tract obstruction (LVOTO) HCM and nonobstructive hypertrophic cardiomyopathy (NO-HCM) in children and adolescents who were treated with standard medication or surgical resection.MethodsThis retrospective study identified 21 BVOTO patients and recruited 27 LVOTO and 24 NO-HCM patients younger than 18 years presenting at our institution. The primary endpoint was all-cause death, and secondary endpoints were cardiovascular events.ResultsMore BVOTO patients (61.9%) than LVOTO (19.2%) and NO-HCM patients (25%) exhibited New York Heart Association (NYHA) III/IV status (p < 0.01). Fourteen BVOTO and 16 LVOTO patients obtained a significant reduction of outflow tract pressure gradients after surgery (vs. preoperative baseline, p < 0.001). One of the 14 BVOTO patients died, whereas no deaths occurred among LVOTO patients. Three of 14 BVOTO surgery patients had complete heart block (CHB) and 4 had new right bundle branch block (RBBB), while no CHB or RBBB occurred in the LVOTO surgery patients. The BVOTO patients had a longer duration of aortic cross-clamping and postoperative hospital days than the LVOTO patients (p < 0.05). During a median 42-month follow-up, no deaths occurred among the remaining patients. The primary and secondary endpoint-free survival rates of the BVOTO group were comparable to those of the LVOTO and NO-HCM groups.ConclusionsIn children and adolescents, BVOTO patients were associated with more severe symptoms than LVOTO and NO-HCM patients; however, good mid-term outcomes similar to those of the LVOTO and NO-HCM groups can be achieved with the application of contemporary cardiovascular treatment strategies. Notably, BVOTO surgery was associated with an increased risk of CHB and RBBB compared to LVOTO surgery.

Highlights

  • Hypertrophic cardiomyopathy (HCM) is a common inherited heart disease characterized by marked heterogeneity in phenotypic and genetic expression, clinical presentation, and natural history [1]

  • biventricular outflow tract obstruction (BVOTO) patients were associated with more severe symptoms than left ventricular outflow tract obstruction (LVOTO) and NO-hypertrophic cardiomyopathy (HCM) patients; good mid-term outcomes similar to those of the LVOTO and non-obstructive hypertrophic cardiomyopathy (NO-HCM) groups can be achieved with the application of contemporary cardiovascular treatment strategies

  • BVOTO surgery was associated with an increased risk of complete heart block (CHB) and right bundle branch block (RBBB) compared to LVOTO surgery

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Summary

Objective

Our aim is to compare mid-term outcomes of biventricular outflow tract obstruction (BVOTO) HCM, left ventricular outflow tract obstruction (LVOTO) HCM and nonobstructive hypertrophic cardiomyopathy (NO-HCM) in children and adolescents who were treated with standard medication or surgical resection

Results
Conclusions
Introduction
Study design and population
Discussion
Study limitations

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