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双腔右心室中的对位不良型室间隔缺损。

Malalignment-type ventricular septal defect in double-chambered right ventricle.

作者信息

Wang J K, Wu M H, Chang C I, Chiu I S, Chu S H, Hung C R, Lue H C

机构信息

Department of Pediatric, National Taiwan University Hospital, Taipei, Taiwan.

出版信息

Am J Cardiol. 1996 Apr 15;77(10):839-42. doi: 10.1016/S0002-9149(97)89178-3.

DOI:10.1016/S0002-9149(97)89178-3
PMID:8623736
Abstract

Double-chambered right ventricle (DCRV) is commonly associated with ventricular septal defect (VSD). In this study, an assessment was made of the relevance of a malalignment-type VSD to hemodynamic and morphologic features in DCRV. During an 8.5-year period, 53 patients with DCRV were enrolled after study with echocardiography, catheterization, and angiography. Patients were divided into 2 groups: group I included 40 patients, aged 3.7 +/- 3.2 years, with a malalignment-type VSD; group II consisted of 13 patients, aged 8.6 +/- 2.7 years, without a malalignment-type VSD. History of congestive heart failure in infancy was present in 21 group I and 2 group II patients (53% vs 15%, respectively, p <0.05). The mean pulmonary-to-systemic flow ratio was significantly higher in group I than in group II (1.89 +/- 0.74 vs 1.14 +/- 0.21, respectively, p <0.05). The mean pressure gradient across the right ventricular outflow tract was lower in group I than in group II (41 +/- 16 vs, 73 +/- 33 mm Hg, respectively, p <0.05). Among 42 patients who had a series of echocardiograms recorded, progression of pressure gradient was evident in 35: 28 in group I and 7 in group II. A subaortic ridge was present exclusively in 29 group I patients (73%). Prolapse of the aortic valve was present in 26 (49%): 20 group I (50%) and 6 group II (46%) patients. Aneurysm formation of the septal defect was found in 17 (43%) and 7 (54%) group I and II patients, respectively. It can be concluded that a history of congestive heart failure was more common in DCRV patients with a malalignment-type VSD. Malalignment-type VSD is significantly associated with a larger pulmonary-to-systemic flow ratio and subaortic ridge.

摘要

双腔右心室(DCRV)通常与室间隔缺损(VSD)相关。在本研究中,对不一致型室间隔缺损与双腔右心室血流动力学及形态学特征的相关性进行了评估。在8.5年的时间里,53例双腔右心室患者经超声心动图、心导管检查和血管造影检查后入组。患者分为2组:I组包括40例年龄为3.7±3.2岁的不一致型室间隔缺损患者;II组由13例年龄为8.6±2.7岁、无不一致型室间隔缺损的患者组成。I组21例和II组2例患者有婴儿期充血性心力衰竭病史(分别为53%和15%,p<0.05)。I组的平均肺循环与体循环血流量比值显著高于II组(分别为1.89±0.74和1.14±0.21,p<0.05)。I组右心室流出道的平均压力阶差低于II组(分别为41±16和73±33mmHg,p<0.05)。在42例进行了一系列超声心动图检查的患者中,35例压力阶差有进展:I组28例,II组7例。仅29例I组患者(73%)存在主动脉下嵴。26例(49%)患者存在主动脉瓣脱垂:I组20例(50%),II组6例(46%)。I组和II组分别有17例(43%)和7例(54%)患者存在室间隔缺损瘤形成。可以得出结论,有不一致型室间隔缺损的双腔右心室患者充血性心力衰竭病史更为常见。不一致型室间隔缺损与更大的肺循环与体循环血流量比值及主动脉下嵴显著相关。

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Unrepaired Congenital Heart Disease in Childhood: A Case of Ventricular Septal Defect and Double-Chambered Right Ventricle.儿童期未修复的先天性心脏病:一例室间隔缺损合并右心室双腔心病例
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Spontaneously healed membranous type ventricular septal defect with malaligned interventricular septal wall and double-chambered right ventricle in a 56-year-old patient.
一名56岁患者出现自发性愈合的膜周型室间隔缺损,伴有室间隔壁对位不良及右心室双腔心。
J Cardiovasc Ultrasound. 2011 Sep;19(3):148-51. doi: 10.4250/jcu.2011.19.3.148. Epub 2011 Sep 30.