Heart 1998;80:163-169 ( August )

Marfan syndrome in children and adolescents: predictive and prognostic value of aortic root growth for screening for aortic complications

M Groenink,^a L Rozendaal,^b M S J Naeff,^b R C M Hennekam,^c A A M Hart,^d E E van der Wall,^e B J M Mulder^a

^a Department of Cardiology, Academic Medical Centre, Amsterdam, Netherlands, ^b Department of Paediatric Cardiology, Academic Medical Centre, ^c Department of Paediatrics and Institute for Human Genetics, Academic Medical Centre, ^d Department of Clinical Epidemiology and Biostatistics, Academic Medical Centre, ^e Department of Cardiology, Leiden University Medical Centre, Leiden, Netherlands

Correspondence to: Dr B J M Mulder, Department of Cardiology (Rm B2-240), Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands.

Accepted for publication 4 March 1998

ObjectiveTo assess and measure the diagnostic and prognostic value of individual aortic root growth in children and adolescents with Marfan syndrome.
DesignFrom 1983 to 1996, 250 children were screened for Marfan syndrome. Serial echocardiographic aortic root measurements of 123 children (57 Marfan, 66 control) were available for evaluation of aortic root growth. Aortic root diameters were correlated with body surface area. Based on individual growth of the aortic root a discrimination formula was derived to distinguish Marfan patients from control subjects.
ResultsSensitivity and specificity of this method, which were dependent on the number of measurements, were 84% and 73%, respectively, for three serial measurements. The discrimination score also predicted a five year complication free survival in all patients.
ConclusionsIn children and adolescents with Marfan syndrome, aortic root growth differs significantly from subjects in whom Marfan syndrome was definitely ruled out during screening. Measurement of individual aortic root growth may serve to establish diagnosis at an early stage and is of prognostic value for occurrence of aortic complications.

Keywords: Marfan syndrome;  aortic root growth;  echocardiography;  children;  adolescents

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© 1998 by Heart
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