WALL THICKNESS NORMALIZATION IN PEDIATRIC HYPERTROPHIC CARDIOMYOPATHY: CHARACTERIZING PREDICTORS AND PATIENT OUTCOMES
CCC ePoster Library. Lynch A. 10/26/19; 280305; 247
Dr. Aine Lynch
Dr. Aine Lynch
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Abstract
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BACKGROUND: Pediatric hypertrophic cardiomyopathy (HCM) is usually progressive. However, some patients demonstrate improvement in wall thickness over time. Our aim was to determine the frequency and predictors of echocardiographic normalization of septal and posterior wall thickness in HCM patients and assess the association with transplant-free survival.

METHODS AND RESULTS: There were 177 HCM patients ( < 18 years old) identified from the SickKids Heart Failure Database diagnosed between January 1, 2001 and July 1, 2017. Echocardiographic normalization was defined as a z-score for both septal thickness and posterior wall thickness < 2 on the same echocardiogram at any time during follow-up. Kaplan-Meier survival analysis was used to assess transplant-free survival and a multivariable regression model was used to determine patient factors associated with wall thickness normalization. Thirty-six patients (20%) had wall thickness normalization. Patients with normalization were less likely to have familial or genetic HCM (42% vs. 60%, p=0.05), and be younger at diagnosis (1.0 vs. 7.9 years, p = 0.009). There was no difference between patients with and without normalization for the presence of a pathogenic mutation (36% vs. 33%, p=0.55) or congenital syndromes (11% vs. 7%, p=0.51). Patients with normalization were less likely to have ischemic changes on baseline electrocardiogram (18% vs. 29%, p=0.0003). On baseline echocardiogram, patients with normalization were more likely to have smaller z-score values for septal (4.4 vs.7.9, p=0.0004) and posterior wall thickness (1.7 vs 2.9, p = 0.05) as well as larger LV end-diastolic dimension z-scores (-0.6 vs -1.4, p=0.006). Interestingly, patients with normalization were more likely to develop moderate or severe LV dysfunction during follow-up (43% vs. 4%, p < 0.0001) although no difference in transplant-free survival was observed between the two groups (78% vs. 90%, p=0.09; Figure 1). In addition, the development of at least moderate to severe ventricular dysfunction was not an independent predictor of wall thickness normalization. Variables independently predictive of wall thickness normalization are shown in Table 1.

CONCLUSION: Although normalization of wall thickness in HCM patients was associated with the development of moderate to severe ventricular dysfunction, ventricular dysfunction was not an independent predictor for normalization. In addition, normalization of wall thickness was not associated with worse transplant-free survival indicating that there is a subgroup of HCM patients who have thinning of their wall thickness without developing ventricular dysfunction. Those HCM patients are potentially demonstrating favorable myocardial remodeling and need to be further characterized.
BACKGROUND: Pediatric hypertrophic cardiomyopathy (HCM) is usually progressive. However, some patients demonstrate improvement in wall thickness over time. Our aim was to determine the frequency and predictors of echocardiographic normalization of septal and posterior wall thickness in HCM patients and assess the association with transplant-free survival.

METHODS AND RESULTS: There were 177 HCM patients ( < 18 years old) identified from the SickKids Heart Failure Database diagnosed between January 1, 2001 and July 1, 2017. Echocardiographic normalization was defined as a z-score for both septal thickness and posterior wall thickness < 2 on the same echocardiogram at any time during follow-up. Kaplan-Meier survival analysis was used to assess transplant-free survival and a multivariable regression model was used to determine patient factors associated with wall thickness normalization. Thirty-six patients (20%) had wall thickness normalization. Patients with normalization were less likely to have familial or genetic HCM (42% vs. 60%, p=0.05), and be younger at diagnosis (1.0 vs. 7.9 years, p = 0.009). There was no difference between patients with and without normalization for the presence of a pathogenic mutation (36% vs. 33%, p=0.55) or congenital syndromes (11% vs. 7%, p=0.51). Patients with normalization were less likely to have ischemic changes on baseline electrocardiogram (18% vs. 29%, p=0.0003). On baseline echocardiogram, patients with normalization were more likely to have smaller z-score values for septal (4.4 vs.7.9, p=0.0004) and posterior wall thickness (1.7 vs 2.9, p = 0.05) as well as larger LV end-diastolic dimension z-scores (-0.6 vs -1.4, p=0.006). Interestingly, patients with normalization were more likely to develop moderate or severe LV dysfunction during follow-up (43% vs. 4%, p < 0.0001) although no difference in transplant-free survival was observed between the two groups (78% vs. 90%, p=0.09; Figure 1). In addition, the development of at least moderate to severe ventricular dysfunction was not an independent predictor of wall thickness normalization. Variables independently predictive of wall thickness normalization are shown in Table 1.

CONCLUSION: Although normalization of wall thickness in HCM patients was associated with the development of moderate to severe ventricular dysfunction, ventricular dysfunction was not an independent predictor for normalization. In addition, normalization of wall thickness was not associated with worse transplant-free survival indicating that there is a subgroup of HCM patients who have thinning of their wall thickness without developing ventricular dysfunction. Those HCM patients are potentially demonstrating favorable myocardial remodeling and need to be further characterized.
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