PDF(Pubmed)
Abstract：
OBJECTIVE: In genetic aortopathies (GA) particular attention is paid to aortic root dilatation which has an impact on morbidity and mortality. This study focuses on the effects of therapy with angiotensin-II-receptor-blockers (ARB) or beta-blockers (BB) on aortic root growth and the question which therapy should be initiated at which dosage and at what age.
METHODS: Since 1998 we diagnosed 208 patients with GA (170 FBN-1). 81 patients between 5 months and 18 years receiving either ARB or BB therapy were included. We retrospectively analyzed the progression of the dilatation of Sinus Valsalva aortae (SV) using calculated z-scores before and after therapy initiation and compared BB and ARB treatment.
RESULTS: Both ARB and BB (p < 0.05) therapy showed significant improvement in aortic root growth, while the effect is significantly more pronounced in ARB (p < 0.01) independent of age and genetic cause. A detailed comparison of the two drug groups showed a more sustained effect in limiting the progression of the dilatation of the aortic root in patients treated with ARB. Progression of dilatation of the SV was significantly lower in children treated with ARBs compared to BB (delta z-score, p < 0.05). In addition, ARBs were better tolerated and had a significantly lower discontinuation rate (3%) compared to BB (50%) (p < 0.01). Independently of age at initiation all children and adolescents were able to reach the target dose under ARB.
CONCLUSIONS: We demonstrated a significant change in both treatment options, with the effect of ARB being more pronounced while being better tolerated throughout the treatment period.
METHODS: Since 1998 we diagnosed 208 patients with GA (170 FBN-1). 81 patients between 5 months and 18 years receiving either ARB or BB therapy were included. We retrospectively analyzed the progression of the dilatation of Sinus Valsalva aortae (SV) using calculated z-scores before and after therapy initiation and compared BB and ARB treatment.
RESULTS: Both ARB and BB (p < 0.05) therapy showed significant improvement in aortic root growth, while the effect is significantly more pronounced in ARB (p < 0.01) independent of age and genetic cause. A detailed comparison of the two drug groups showed a more sustained effect in limiting the progression of the dilatation of the aortic root in patients treated with ARB. Progression of dilatation of the SV was significantly lower in children treated with ARBs compared to BB (delta z-score, p < 0.05). In addition, ARBs were better tolerated and had a significantly lower discontinuation rate (3%) compared to BB (50%) (p < 0.01). Independently of age at initiation all children and adolescents were able to reach the target dose under ARB.
CONCLUSIONS: We demonstrated a significant change in both treatment options, with the effect of ARB being more pronounced while being better tolerated throughout the treatment period.
摘要：
目的：在遗传性主动脉病变（GA）中，特别注意主动脉根部扩张对发病率和死亡率有影响。这项研究的重点是血管紧张素II受体阻滞剂（ARB）或β受体阻滞剂（BB）对主动脉根部生长的影响，以及应以哪种剂量和年龄开始治疗的问题。
方法：自1998年以来，我们诊断了208例GA患者（170FBN-1）。纳入81例5个月至18岁接受ARB或BB治疗的患者。我们使用计算的z评分在治疗开始前后回顾性分析了Valsalva主动脉窦（SV）扩张的进展，并比较了BB和ARB治疗。
结果：ARB和BB（p<0.05）治疗均显示出主动脉根部生长的显着改善，而与年龄和遗传原因无关，ARB的影响显着更为明显（p<0.01）。两个药物组的详细比较显示，在用ARB治疗的患者中，在限制主动脉根部扩张的进展方面具有更持久的作用。与BB相比，接受ARB治疗的儿童SV扩张的进展显着降低（deltaz评分，p<0.05)。此外,与BB（50％）相比，ARB的耐受性更好，停药率（3％）显着降低（p<0.01）。独立于开始时的年龄，所有儿童和青少年都能够达到ARB下的目标剂量。
结论：我们证明了两种治疗方案的显著变化，ARB的效果更明显，同时在整个治疗期间耐受性更好。
方法：自1998年以来，我们诊断了208例GA患者（170FBN-1）。纳入81例5个月至18岁接受ARB或BB治疗的患者。我们使用计算的z评分在治疗开始前后回顾性分析了Valsalva主动脉窦（SV）扩张的进展，并比较了BB和ARB治疗。
结果：ARB和BB（p<0.05）治疗均显示出主动脉根部生长的显着改善，而与年龄和遗传原因无关，ARB的影响显着更为明显（p<0.01）。两个药物组的详细比较显示，在用ARB治疗的患者中，在限制主动脉根部扩张的进展方面具有更持久的作用。与BB相比，接受ARB治疗的儿童SV扩张的进展显着降低（deltaz评分，p<0.05)。此外,与BB（50％）相比，ARB的耐受性更好，停药率（3％）显着降低（p<0.01）。独立于开始时的年龄，所有儿童和青少年都能够达到ARB下的目标剂量。
结论：我们证明了两种治疗方案的显著变化，ARB的效果更明显，同时在整个治疗期间耐受性更好。
