The Natural History of the So‐Called Aneurysm of the Membranous Ventricular Septum in Childhood
The Natural History of the So‐Called Aneurysm of the Membranous Ventricular Septum in Childhood
复制标题
儿童时期所谓的膜性室间隔动脉瘤的自然史
作者：
R. Freedom;R. White;D. Pieroni;P. Varghese;L. Krovetz;R. Rowe
五十六例先天性心脏病国家自然史合作研究中的孤立性膜部室间隔缺损（VSD）患儿在约翰霍普金斯医院中心进行研究，以确定相关的“膜部间隔动脉瘤”（AMS）的发生和自然史。这些患者的年龄和VSD大小范围很广。每名儿童均接受两次心脏导管检查，间隔四年，并定期进行临床检查。AMS的诊断是基于血管造影标准，因此可能包括几个不同的解剖学lesions.20名儿童AMS在第一次导管插入术，所有的年龄都在2岁以上，除了一个有肺动脉与体循环流量比（QP/Qs）小于2.0，与正常的心内压。4年后，重复导管插入术显示该组动脉瘤的Qp/Qs和血管造影外观仅有微小变化。在第二项研究中还证实了12例AMS新病例;其中7例儿童在动脉瘤形成期间Qp/Qs显著降低。在我们的研究过程中，没有观察到自发解剖闭合的病例，也没有任何可归因于动脉瘤存在的严重并发症。总的来说，AMS在56例患者中有32例（57%），在44例Qp/Qs小于2.0的患者中有31例（70%）。这些结果表明AMS在小膜部VSD儿童中很常见，并且其在儿童期的病程通常是稳定的和无症状的。虽然在这项为期4年的研究中未观察到室间隔缺损的解剖学闭合，但我们的数据支持动脉瘤形成可能在某些相关膜性室间隔缺损功能尺寸自发缩小的病例中起重要作用的建议。
Fifty-six children with isolated membranous ventricular septal defect (VSD) included in the National Natural History Cooperative Study for Congenital Heart Disease were studied at the Johns Hopkins Hospital Center to determine the occurrence and natural history of an associated “aneurysm of the membranous septum’ (AMS). These patients represented a wide range of ages and VSD size. Each child was evaluated by two cardiac catheterizations four years apart, with regular clinical examinations in the interval. The diagnosis of AMS was based on angiographic criteria, and as such may encompass several distinct anatomic lesions.Twenty children had AMS at the first catheterization; all were over age two, and all but one had a pulmonic-to-systemic flow ratio (Qp/Qs) of less than 2.0, with normal intracardiac pressures. Four years later, repeat catheterization revealed only minor changes in Qp/Qs and angiographic appearance of the aneurysms in this group. Twelve new cases of AMS were also demonstrated at the second study; seven of these children underwent a significant diminution in Qp/Qs during the period in which the aneurysm was formed. No cases of spontaneous anatomic closure were observed in the course of our study, nor were there any serious complications which could be attributed to the presence of an aneurysm. Overall, AMS was found in 32 of 56 patients (57%), and in 31 of 44 (70%) of those with a Qp/Qs less than 2.0.These findings suggest that AMS is common among children with small membranous VSD, and that its course in childhood is usually stable and asymptomatic. Although anatomic closure of the VSD was not observed during this four year study, our data support suggestions that aneurysm formation may play an important role in some cases of spontaneous diminution in the functional size of an associated membranous VSD.