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ASSESSMENT OF AORTIC STIFFNESS AND BLOOD PRESSURE IN YOUNG TURNER SYNDROME PATIENTS
Abstracts
tumors in various organs, including the heart. Commonly called cardiac rhabdomyomas (RHM) it is most frequently found during childhood. RHM regresses in most cases spontaneously but can be complicated by serious arrhythmias or obstruction that may compromise cardiac output. METHODS: We conducted a retrospective analysis of 30 patients and then classified patients according to the initial volume of RHM in early childhood. We subsequently conducted a prospective cardiac evaluation of mass. We compared the prevalence of arrhythmia based on the initial volume of the RHM comparatively between the case and the family control subjects with TSC but no RHM. RESULTS: The median follow-up time was 4.5 years. The Prevalence of RHM was 46.8% (37 RHM) between 0-5 years, 39.2% (31 RHM) between 12-33 years and 13.9% (11 RHM) between 6-11 years. RHM causes in 20% an obstacle to inflow or outflow tract. Patients with RHM were classified according to the total volume of initial RHM among whom 16 cases (64%) with history of small RHM (˂25% of left ventricular mass) at birth versus 11 (36%) with history of large RHM. The encountered benign arrhythmias founded were isolated ventricular premature beats (55%) and supraventricular premature beats (50%), which were 2 times fold and 4 times fold more compared to reported prevalence in the general pediatric population, respectively (p<0.05). Significant arrhythmia was noted in 3 (30%) patients with large RHM: one patient with Wolff Parkinson White syndrome had supraventricular tachycardia treated with anti-arrhythmic agent, one case had atrial fibrillation equally treated with anti-arrhythmic agent, with resolved arrhythmia concomitantly with the regression of the tumor, a third patient had symptomatic paroxysmal supraventricular tachycardia requiring percutaneous cryoablation. There was a trend which did not reach statistical significance difference in the prevalence of cardiac arrhythmias between cases with small versus large RHM (p¼0.07). CONCLUSION: In this series, arrhythmia was more prevalent in TSC compared to general pediatric population, with a trend towards higher prevalence in cases with larger RHM. This is the first series to report RHM size correlation with the prevalence of arrhythmia included cases with absent RHM. It is possible that such arrhythmias are caused by subanatomical lesions.
064 ASSESSMENT OF AORTIC STIFFNESS AND BLOOD PRESSURE IN YOUNG TURNER SYNDROME PATIENTS
S33
well recognized cause of cardiovascular death, with an estimated incidence of 1.4 per 100 patients with TS. The biophysical properties of the aorta, including pulse wave velocity (PWV), characteristic impedance (Zc), input impedance (Zi), elastic pressure-strain modulus (Ep), and beta index (b-index) have not been well studied in TS. This population is also known to have increased rates of hypertension (HTN). Ambulatory blood pressure monitoring (ABPM) is a more sensitive method of diagnosing HTN than resting BP. The purpose of this study was to assess aortic stiffness, aortic dilation and ABPM in TS. Our hypothesis was that aortic stiffness would be increased and associated with HTN in TS patients. METHODS: TS patients were recruited from the Endocrinology Clinic at British Columbia Children’s Hospital and were compared with healthy controls (C). An echo Doppler method was used to measure the aortic dimensions, PWV, Zc, Zi, Ep and b-index. Values were compared to C. ABPM was performed in TS and interpreted based on accepted guidelines. ABPM readings were designated abnormal if there was evidence of hypertension or systolic or diastolic non-dipping overnight. RESULTS: There were 16 TS patients and 28 C. Median ages, weights and body surface areas were similar; TS patients were shorter (147 vs. 161cm, p¼0.004). Aortic dimensions and resting blood pressure were similar. PWV (447 vs. 372 cm/s, p<0.001) and Zc (190 vs. 136 dyne s/cm5, p<0.001) were increased in TS. Zi, Ep and b-index were similar. ABPM was abnormal in 7/14 patients. Ascending aorta dimension (2.02 vs 2.19 cm, p¼0.609) and PWV (451 vs 451 cm/s, p¼0.482) were similar in patients with normal and abnormal ABPM. CONCLUSION: This study shows that young patients with TS have stiffer aortas than C. This occurred in the absence of resting hypertension or aortic dilation. Though abnormalities on ABPM were identified in half of the patients, this was not correlated to increases in PWV. Further studies with larger numbers are needed to determine the etiology of aortic stiffening in this population and whether or not it is a precipitating factor for the aortic dilation and dissection that is frequently seen in TS patients. Rare Disease Foundation, Department of Pediatrics, University of British Columbia
065 LEFT VENTRICULAR REPOLARIZATION ANOMALIES AFTER EXERCISE TESTING IN ASYMPTOMATIC MODERATE AORTIC REGURGITATION W Mawad, L Beauséjour, H Gravel, D Curnier, N Dahdah
DA Fox, KT Kang, JE Potts, JM Dionne, LL Stewart, GG Sandor
Montréal, Québec
Vancouver, British Columbia
BACKGROUND:
BACKGROUND:
Bicuspid aortic valve and aortic dilation are common in Turner Syndrome (TS). Aortic dissection is a
Aortic regurgitation (AR) results in a volume overload of the left ventricle. Although this volume-load brings on left ventricular eccentric hypertrophy and
tumors in various organs, including the heart. Commonly called cardiac rhabdomyomas (RHM) it is most frequently found during childhood. RHM regresses in most cases spontaneously but can be complicated by serious arrhythmias or obstruction that may compromise cardiac output. METHODS: We conducted a retrospective analysis of 30 patients and then classified patients according to the initial volume of RHM in early childhood. We subsequently conducted a prospective cardiac evaluation of mass. We compared the prevalence of arrhythmia based on the initial volume of the RHM comparatively between the case and the family control subjects with TSC but no RHM. RESULTS: The median follow-up time was 4.5 years. The Prevalence of RHM was 46.8% (37 RHM) between 0-5 years, 39.2% (31 RHM) between 12-33 years and 13.9% (11 RHM) between 6-11 years. RHM causes in 20% an obstacle to inflow or outflow tract. Patients with RHM were classified according to the total volume of initial RHM among whom 16 cases (64%) with history of small RHM (˂25% of left ventricular mass) at birth versus 11 (36%) with history of large RHM. The encountered benign arrhythmias founded were isolated ventricular premature beats (55%) and supraventricular premature beats (50%), which were 2 times fold and 4 times fold more compared to reported prevalence in the general pediatric population, respectively (p<0.05). Significant arrhythmia was noted in 3 (30%) patients with large RHM: one patient with Wolff Parkinson White syndrome had supraventricular tachycardia treated with anti-arrhythmic agent, one case had atrial fibrillation equally treated with anti-arrhythmic agent, with resolved arrhythmia concomitantly with the regression of the tumor, a third patient had symptomatic paroxysmal supraventricular tachycardia requiring percutaneous cryoablation. There was a trend which did not reach statistical significance difference in the prevalence of cardiac arrhythmias between cases with small versus large RHM (p¼0.07). CONCLUSION: In this series, arrhythmia was more prevalent in TSC compared to general pediatric population, with a trend towards higher prevalence in cases with larger RHM. This is the first series to report RHM size correlation with the prevalence of arrhythmia included cases with absent RHM. It is possible that such arrhythmias are caused by subanatomical lesions.
064 ASSESSMENT OF AORTIC STIFFNESS AND BLOOD PRESSURE IN YOUNG TURNER SYNDROME PATIENTS
S33
well recognized cause of cardiovascular death, with an estimated incidence of 1.4 per 100 patients with TS. The biophysical properties of the aorta, including pulse wave velocity (PWV), characteristic impedance (Zc), input impedance (Zi), elastic pressure-strain modulus (Ep), and beta index (b-index) have not been well studied in TS. This population is also known to have increased rates of hypertension (HTN). Ambulatory blood pressure monitoring (ABPM) is a more sensitive method of diagnosing HTN than resting BP. The purpose of this study was to assess aortic stiffness, aortic dilation and ABPM in TS. Our hypothesis was that aortic stiffness would be increased and associated with HTN in TS patients. METHODS: TS patients were recruited from the Endocrinology Clinic at British Columbia Children’s Hospital and were compared with healthy controls (C). An echo Doppler method was used to measure the aortic dimensions, PWV, Zc, Zi, Ep and b-index. Values were compared to C. ABPM was performed in TS and interpreted based on accepted guidelines. ABPM readings were designated abnormal if there was evidence of hypertension or systolic or diastolic non-dipping overnight. RESULTS: There were 16 TS patients and 28 C. Median ages, weights and body surface areas were similar; TS patients were shorter (147 vs. 161cm, p¼0.004). Aortic dimensions and resting blood pressure were similar. PWV (447 vs. 372 cm/s, p<0.001) and Zc (190 vs. 136 dyne s/cm5, p<0.001) were increased in TS. Zi, Ep and b-index were similar. ABPM was abnormal in 7/14 patients. Ascending aorta dimension (2.02 vs 2.19 cm, p¼0.609) and PWV (451 vs 451 cm/s, p¼0.482) were similar in patients with normal and abnormal ABPM. CONCLUSION: This study shows that young patients with TS have stiffer aortas than C. This occurred in the absence of resting hypertension or aortic dilation. Though abnormalities on ABPM were identified in half of the patients, this was not correlated to increases in PWV. Further studies with larger numbers are needed to determine the etiology of aortic stiffening in this population and whether or not it is a precipitating factor for the aortic dilation and dissection that is frequently seen in TS patients. Rare Disease Foundation, Department of Pediatrics, University of British Columbia
065 LEFT VENTRICULAR REPOLARIZATION ANOMALIES AFTER EXERCISE TESTING IN ASYMPTOMATIC MODERATE AORTIC REGURGITATION W Mawad, L Beauséjour, H Gravel, D Curnier, N Dahdah
DA Fox, KT Kang, JE Potts, JM Dionne, LL Stewart, GG Sandor
Montréal, Québec
Vancouver, British Columbia
BACKGROUND:
BACKGROUND:
Bicuspid aortic valve and aortic dilation are common in Turner Syndrome (TS). Aortic dissection is a
Aortic regurgitation (AR) results in a volume overload of the left ventricle. Although this volume-load brings on left ventricular eccentric hypertrophy and