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PREDICTORS OF AORTIC DILATATION IN TURNER SYNDROME
908 JACC April 5, 2016 Volume 67, Issue 13
Congenital Heart Disease PREDICTORS OF AORTIC DILATATION IN TURNER SYNDROME Moderated Poster Contributions Congenital Heart Disease Moderated Poster Theater, Poster Area, South Hall A1 Saturday, April 02, 2016, 4:15 p.m.-4:25 p.m. Session Title: Abnormalities of the Aorta in Congenital Heart Disease Abstract Category: 22. Congenital Heart Disease: Adult Presentation Number: 1163M-05 Authors: Anthonie Duijnhouwer, Roland van Kimmenade, Arie van Dijk, Menko Jan de Boer, Jolien Roos-Hesselink, Janneke Timmermans, Radboud University Medical Center, Nijmegen, The Netherlands, Erasmus Medical Center, Rotterdam, The Netherlands Background: Aortic dissection is the most devastating complication of aortic dilatation and is presumed to be more prevalent in Turner syndrome (TS) patients than in the general population. The risk of dissection was found to be strongly correlated with the aortic size and guidelines advice preventive aortic surgery when the aorta is dilated. Known predictors for aortic dilatation in TS are the presence of a bicuspid aortic valve (BAV), aortic coarctation (CoA) and hypertension. This study searches for new predictors of aortic dilatation in a large cohort of TS patients. Methods: Cross-sectional retrospective single centre study. All TS patients seen in last 10 years, who underwent a MRI were included. Clinical characteristics, echocardiographic and cardiac MRI data were collected. Correlation between absolute aortic diameters (7 levels) on MRI and variables was investigated by multiple regression analysis.
Results: Of the 289 included TS patients, 46 did not undergo a MRI (because of pacemaker, claustrophobia, etc.). The remaining 243 patients included for this study (mean age was 36.9 + 11.6 years) 49 (19.9%) had a BAV and 7 (2.8%) CoA. The aortic diameter was widest at the level of aorta sinus of Valsalva (SoV) and ascending aorta (AA). Multi regression analysis demonstrated (R2 = 0.387, adjusted R2 = 0.370) that age in years (b = 0.271, p = 0.0001) and BAV (b = 3.012, p = 0.0001), were the strongest predictors for AA diameter, followed by (in descending order of importance based on beta) weight in kg (b = 0.077, p = 0.0001), growth hormone treatment (b = 1.775, p = 0.014), hypertension (b = 2.211, p = 0.004) and karyotype 45X/0 (b = 1.554, p = 0.007). For SoV (R2 = 0.136, adjusted R2 = 0.124) the strongest predictors were age (b = 0.064, p = 0.002) and BAV (b = 2.580, P = 0.001), followed by karyotype 45X0 (b = 1.190, p = 0.013). Analysis of the residuals showed a normal distribution. Conclusions: Besides the already known predictors BAV and Age, we found previous growth hormone treatment and karyotype 45X0 as new predictors of AA size in TS. The presence of CoA was not found to be predictive. Whether these new factors predict the risk of dissection should be investigated further.
Congenital Heart Disease PREDICTORS OF AORTIC DILATATION IN TURNER SYNDROME Moderated Poster Contributions Congenital Heart Disease Moderated Poster Theater, Poster Area, South Hall A1 Saturday, April 02, 2016, 4:15 p.m.-4:25 p.m. Session Title: Abnormalities of the Aorta in Congenital Heart Disease Abstract Category: 22. Congenital Heart Disease: Adult Presentation Number: 1163M-05 Authors: Anthonie Duijnhouwer, Roland van Kimmenade, Arie van Dijk, Menko Jan de Boer, Jolien Roos-Hesselink, Janneke Timmermans, Radboud University Medical Center, Nijmegen, The Netherlands, Erasmus Medical Center, Rotterdam, The Netherlands Background: Aortic dissection is the most devastating complication of aortic dilatation and is presumed to be more prevalent in Turner syndrome (TS) patients than in the general population. The risk of dissection was found to be strongly correlated with the aortic size and guidelines advice preventive aortic surgery when the aorta is dilated. Known predictors for aortic dilatation in TS are the presence of a bicuspid aortic valve (BAV), aortic coarctation (CoA) and hypertension. This study searches for new predictors of aortic dilatation in a large cohort of TS patients. Methods: Cross-sectional retrospective single centre study. All TS patients seen in last 10 years, who underwent a MRI were included. Clinical characteristics, echocardiographic and cardiac MRI data were collected. Correlation between absolute aortic diameters (7 levels) on MRI and variables was investigated by multiple regression analysis.
Results: Of the 289 included TS patients, 46 did not undergo a MRI (because of pacemaker, claustrophobia, etc.). The remaining 243 patients included for this study (mean age was 36.9 + 11.6 years) 49 (19.9%) had a BAV and 7 (2.8%) CoA. The aortic diameter was widest at the level of aorta sinus of Valsalva (SoV) and ascending aorta (AA). Multi regression analysis demonstrated (R2 = 0.387, adjusted R2 = 0.370) that age in years (b = 0.271, p = 0.0001) and BAV (b = 3.012, p = 0.0001), were the strongest predictors for AA diameter, followed by (in descending order of importance based on beta) weight in kg (b = 0.077, p = 0.0001), growth hormone treatment (b = 1.775, p = 0.014), hypertension (b = 2.211, p = 0.004) and karyotype 45X/0 (b = 1.554, p = 0.007). For SoV (R2 = 0.136, adjusted R2 = 0.124) the strongest predictors were age (b = 0.064, p = 0.002) and BAV (b = 2.580, P = 0.001), followed by karyotype 45X0 (b = 1.190, p = 0.013). Analysis of the residuals showed a normal distribution. Conclusions: Besides the already known predictors BAV and Age, we found previous growth hormone treatment and karyotype 45X0 as new predictors of AA size in TS. The presence of CoA was not found to be predictive. Whether these new factors predict the risk of dissection should be investigated further.