Segmental Aortic Stiffness in Children and Young Adults With Connective Tissue Disorders: Relationships With Age, Aortic Size, Rate of Dilation, and Surgical Root Replacement.

BACKGROUND: Aortic diameter is an imperfect predictor of aortic complications in connective tissue disorders (CTDs). Novel indicators of vascular phenotype severity such as aortic stiffness and vertebral tortuosity index have been proposed. We assessed the relation between aortic stiffness by cardiac MRI, surgical root replacement, and rates of aortic root dilation in children and young adults with CTDs. METHODS AND RESULTS: Retrospective analysis of cardiac MRI data on children and young adults with a CTD was performed to derive aortic stiffness measures (strain, distensibility, and ß-stiffness index) at the aortic root, ascending aorta, and descending aorta. Vertebral tortuosity index was calculated as previously described. Rate of aortic root dilation before cardiac MRI was calculated as change in echocardiographic aortic root diameter z score per year. In 83 CTD patients (median age, 24 years; range, 1-55; 17% <18 years of age; 60% male), ascending aorta distensibility was reduced in comparison with published normative values: median z score, -1.93 (range, -8.7 to 1.3; P<0.0001 versus normals). Over a median follow-up period of 2.7 years, there were no aortic dissections or deaths, but 16 of 83 (19%) patients underwent surgical aortic root replacement. In multivariable analysis, lower aortic root strain (P=0.05) and higher vertebral tortuosity index (P=0.01) were independently associated with aortic root replacement. Lower ascending aorta strain (P=0.02) was associated with a higher rate of aortic root dilation. CONCLUSIONS: Higher aortic stiffness is associated with higher rates of surgical aortic replacement and aortic root dilation in children and young adults with CTDs.

Association Between Baseline Blood Pressures, Heart Rates, and Vasovagal Syncope in Children and Adolescents.

BACKGROUND: Vasovagal syncope is the most common cause of syncope in children and adults, accounting for 50-66% of unexplained syncope. There are no studies establishing the relationship between syncope, baseline heart rate, and blood pressure. OBJECTIVE: To identify a possible association between baseline blood pressure and heart rate with syncope. DESIGN/METHODS: We conducted a questionnaire-based chart review study. A questionnaire was distributed to the guardian of children between eight and 18 years of age who attended the Pediatric Ambulatory Care Clinic at Flushing Hospital Medical Center. Based on the responses in the questionnaire, subjects were classified either as cases (positive for syncope) or controls (negative for syncope). Children and adolescents with neurological, cardiac, or any medical condition that can cause syncopal episodes were excluded from the study. Data collected from the questionnaire included age, gender, ethnicity, medical history, family history of syncope, and the amount of salt used in food. Anthropometric and vital signs for the current visit (height, weight, BMI, blood pressure, and heart rate) and vital signs from two previous visits were collected from electronic medical records. The data was analyzed using t-test and chi-square test with Microsoft Excel software (Microsoft Office Standard, v. 14, Microsoft; 2010); p<0.05 was considered significant. RESULTS: A total of 197 subjects were included in this study. There were 18 cases and 179 controls. Of the cases, (4/18) 22.2% were more likely to have a systolic blood pressure lower than the 10th percentile for their gender, age, and height as compared with controls (7/179) 3.9%, p = 0.003. The subjects with a history of syncope were more likely to add salt to their food (p = 0.004). There were no significant differences between cases and controls for age, gender, ethnicity between cases and controls for systolic blood pressure. No significant difference was observed between the heart rates of cases and controls. CONCLUSIONS: Children and adolescents with syncope were more likely to have a systolic blood pressure lower than the 10th percentile, and there was no difference in the baseline heart rate. In addition, children with syncope were more likely to add salt to their food.