Prevalence and Significance of Late Gadolinium Enhancement in Children and Adolescents With Hypertrophic Cardiomyopathy

Abstract

s S97 BACKGROUND: We have previously shown that infants of diabetic mothers (IDM) have increased aortic stiffness in the first 6 months of life, and aortic stiffness is related to poor maternal glycemic control in pregnancy. Increased left ventricular (LV) posterior (LVPW) and interventricular septal (IVS) wall thickness is observed in IDM in late gestation and early infancy, predominantly in mothers with worse glycemic control. Although it is generally believed that the LV hypertrophy in IDM regresses after the first 3 months, we have recently shown that hypertrophy persists even in late infancy. In this study we sought to explore the relationship between aortic stiffness and LV hypertrophy in IDM. We hypothesized that LVPW and IVS wall thickness would be greater in infants with greater aortic stiffness. METHODS: Diabetic pregnancies were prospectively recruited in the mid trimester. Their infants were evaluated in infancy by echocardiography. LVPW and IVS wall thickness was measured by m mode and measurements were compared to normative data for the calculation of z scores based on body surface area. Aortic wall stiffness was assessed with echocardiography, using 2D and pulsed Doppler interrogation of the ascending aorta and distal arch. Aortic pulse wave velocity (PWV) was calculated as the distance from the ascending aorta to distal arch, divided by the time interval from the QRS to the onset of flow for the two interrogated sites (m/s). RESULTS: Twenty-one IDM were investigated at a median age of 7 weeks (range 2-48 weeks). Mean aortic PWV was 4.7 1.7m/s, and the mean IVS and LVPW z scores were 1.3 1.2 and LVPW 1.4 1.1, respectively. Although no relationship was observed between PWV and IVS, there was a weak but positive correlation between aortic PWV and LVPW z score (r1⁄40.50, p1⁄40.027). CONCLUSION: Our study suggests that a potential relationship exists between the degree of stiffness of the aorta and LV wall thickness. This could be due to a common prenatal exposure (hyperglycemia) or to a causal relationship (greater aortic stiffness leading to evolution or persistence of LV hypertrophy). Further studies are underway to determine the etiology of increased aortic stiffness and LV hypertrophy and the natural course of these changes. 025 PREVALENCE AND SIGNIFICANCE OF LATE GADOLINIUM ENHANCEMENT IN CHILDREN AND ADOLESCENTS WITH HYPERTROPHIC CARDIOMYOPATHY L Grosse-Wortmann, J Windram, D Andreea, S Yoo, L Mertens, D Wong, L Benson