Augmentation index and the evolution of aortic disease in marfan-like syndromes.
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Augmentation index and the evolution of aortic disease in marfan-like syndromes. / Mortensen, Kai; Baulmann, Johannes; Rybczynski, Meike; Sheikhzadeh, Sara; Aydin, Muhammet Ali; Treede, Hendrik; Dombrowski, Ellen; Kühne, Kristin; Peitsmeyer, Philipp; Habermann, Christian; Robinson, Peter N; Stuhrmann, Manfred; Berger, Jürgen; Meinertz, Thomas; Von Kodolitsch, Yskert.
in: AM J HYPERTENS, Jahrgang 23, Nr. 7, 7, 15.04.2010, S. 716-724.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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T1 - Augmentation index and the evolution of aortic disease in marfan-like syndromes.
AU - Mortensen, Kai
AU - Baulmann, Johannes
AU - Rybczynski, Meike
AU - Sheikhzadeh, Sara
AU - Aydin, Muhammet Ali
AU - Treede, Hendrik
AU - Dombrowski, Ellen
AU - Kühne, Kristin
AU - Peitsmeyer, Philipp
AU - Habermann, Christian
AU - Robinson, Peter N
AU - Stuhrmann, Manfred
AU - Berger, Jürgen
AU - Meinertz, Thomas
AU - Von Kodolitsch, Yskert
PY - 2010/4/15
Y1 - 2010/4/15
N2 - BACKGROUND:The augmentation index at a heart rate of 75 beats/min (AIx@HR75) and central pulse pressure (CPP) can be measured noninvasively with applanation tonometry (APT). In this observational study, we investigated the relationship between AIx@HR75, CPP and aortic disease in patients with Marfan-like syndromes.METHODS:We performed APT in 78 consecutive patients in whom classic Marfan syndrome (MFS) had been excluded (46 men and 32 women aged 34 +/- 13 years). These patients comprised 9 persons with MFS-like habitus, 6 with a bicuspid aortic valve (BAV), 5 with MASS phenotype, 3 with vascular type of Ehlers-Danlos syndrome (EDS), 3 with familial thoracic aortic aneurysm, 2 with Loeys-Dietz syndrome (LDS), 1 with mitral valve prolapse syndrome, 1 with familial ectopia lentis, and 48 persons with Marfan-like features but no defined syndrome. During 20 +/- 18 months after APT, we observed progression of aortic diameters in 15 patients, and aortic surgery or aortic dissection in 3 individuals.RESULTS:All 11 patients with Marfan-like syndromes and progression of aortic disease exhibited AIx@HR75 > or =11%, including 8 individuals with aortic diameters < or =95th percentile of normal at baseline. Similarly, all 7 individuals without any defined syndrome but progression of aortic diameters exhibited AIx@HR75 >11%, including 6 individuals with aortic diameters < or =95th percentile at the time of APT. Aortic disease did not evolve at AIx@HR75 <11%. CPP is also related to aortic disease progression.CONCLUSIONS:Aortic disease evolution relates to AIx@HR75 and CPP in Marfan like syndromes. Larger studies with comprehensive clinical and echocardiographic follow-up over long time intervals will be required to establish APT for prediction of aortic disease evolution in Marfan-like syndromes.
AB - BACKGROUND:The augmentation index at a heart rate of 75 beats/min (AIx@HR75) and central pulse pressure (CPP) can be measured noninvasively with applanation tonometry (APT). In this observational study, we investigated the relationship between AIx@HR75, CPP and aortic disease in patients with Marfan-like syndromes.METHODS:We performed APT in 78 consecutive patients in whom classic Marfan syndrome (MFS) had been excluded (46 men and 32 women aged 34 +/- 13 years). These patients comprised 9 persons with MFS-like habitus, 6 with a bicuspid aortic valve (BAV), 5 with MASS phenotype, 3 with vascular type of Ehlers-Danlos syndrome (EDS), 3 with familial thoracic aortic aneurysm, 2 with Loeys-Dietz syndrome (LDS), 1 with mitral valve prolapse syndrome, 1 with familial ectopia lentis, and 48 persons with Marfan-like features but no defined syndrome. During 20 +/- 18 months after APT, we observed progression of aortic diameters in 15 patients, and aortic surgery or aortic dissection in 3 individuals.RESULTS:All 11 patients with Marfan-like syndromes and progression of aortic disease exhibited AIx@HR75 > or =11%, including 8 individuals with aortic diameters < or =95th percentile of normal at baseline. Similarly, all 7 individuals without any defined syndrome but progression of aortic diameters exhibited AIx@HR75 >11%, including 6 individuals with aortic diameters < or =95th percentile at the time of APT. Aortic disease did not evolve at AIx@HR75 <11%. CPP is also related to aortic disease progression.CONCLUSIONS:Aortic disease evolution relates to AIx@HR75 and CPP in Marfan like syndromes. Larger studies with comprehensive clinical and echocardiographic follow-up over long time intervals will be required to establish APT for prediction of aortic disease evolution in Marfan-like syndromes.
UR - http://ajh.oxfordjournals.org/content/23/7/716.long
M3 - SCORING: Zeitschriftenaufsatz
VL - 23
SP - 716
EP - 724
JO - AM J HYPERTENS
JF - AM J HYPERTENS
SN - 0895-7061
IS - 7
M1 - 7
ER -