左胸肋间入路微创封堵手术治疗干下型室间隔缺损

目的总结左胸肋间入路微创封堵手术治疗干下型室间隔缺损(VSD)的初步经验。方法2014年2月至2 015年9月我们对19例干下型VSD患儿施行外科微创封堵手术,其中男性10例,女性9例;年龄0.5~17岁,平均(3.2±4.3)岁;体重5.5~50.0 kg,平均(14.3±10.9)kg;缺损直径3.5~9.0 mm,平均(5.4±1.8)mm;合并主动脉瓣右冠瓣轻度脱垂6例,主动脉瓣局限~轻微反流3例,肺动脉瓣轻中度狭窄1例,轻度狭窄1例。均采用左胸肋间入路,在经食管超声心动图(TEE)监视下建立VSD输送轨道并置入封堵器,观察有无残余分流及主动脉瓣反流;于出院前、手术后定期复查经胸超声心动图和心电图。结果 18例封堵手术获成功,因新发主动脉瓣中度反流及残余分流而中转开胸手术1例。术中新发主动脉瓣轻微反流2例,术前合并主动脉瓣反流患者中有2例反流加重。手术时间35~85 min,平均手术时间(55.7±15.9)min。术中出血量3~200 mL,平均(22.7±43.3)mL。围手术期输血1例。术后住院时间3~13 d,平均(4.2±2.3)d。术后出现残余分流、上呼吸道感染、伤口感染各1例。随访6~25个月,平均(14.7±7.0)个月,随访期间1例新发主动脉瓣反流,1例术中新发主动脉瓣反流消失,2例术后仍有主动脉瓣反流患者反流无明显变化,1例残余分流愈合,无传导阻滞发生。结论左胸肋间封堵手术治疗干下型VSD手术安全,操作简单,创伤小,恢复快,近期效果满意,远期效果需进一步随访;对合并主动脉瓣脱垂尤其主动脉瓣反流的干下型VSD需慎重施行外科徽创封堵手术。     

主动脉瓣成形术治疗室间隔缺损并中重度主动脉瓣关闭不全

目的探讨室间隔缺损(VSD)并中重度主动脉瓣关闭不全(AI)患儿行主动脉瓣成形术的疗效。方法分析13例14岁以下VSD并中重度AI患儿行主动脉瓣成形术的临床资料。其中VSD为干下型9例,膜周部型4例;中度AI 4例,重度AI 9例。结果全组均无手术死亡。出院查体脉压差均恢复正常。术后彩色多普勒超声心动图检查平均左心室舒张末径与术前比较明显缩小(P<0.05),未发现有室间隔残余漏,2例有轻度AI。随访无手术死亡,患儿心功能均为纽约心脏协会(NYHA)Ⅰ或Ⅱ级。结论儿童主动脉瓣成形术效果良好,对于VSD并中重度AI患儿应首选主动脉瓣成形术。     

室间隔缺损并主动脉瓣脱垂患儿的介入治疗及疗效评价

目的探讨室间隔缺损(VSD)并主动脉瓣脱垂(AVP)介入治疗的安全性和可行性。方法选择2007年5月-2009年4月在本院住院的VSD并轻度AVP患儿43例,均行经皮VSD堵闭术,经胸超声心动图显示VSD位置及内径,左心室造影均显示VSD并AVP,造影显示VSD大小为2.6～8.3 mm。其中19例为嵴内型(又称膜周流出道型),15例为隔瓣后型,9例为膜周部。右冠瓣脱垂27例,无冠瓣脱垂12例,右冠瓣并无冠瓣脱垂4例,并局限主动脉瓣返流8例。术后进行超声心动图、心电图的随访观察。结果36例成功封堵VSD,封堵成功率83.7%;7例试封堵后再次行左心室及主动脉造影,显示主动脉瓣下有明显切迹,瓣膜返流加重而放弃堵闭术,择期进行外科手术。采用偏心封堵器15例(5～12 mm),大边小腰封堵器(4～14 mm)16例,普通对称型封堵器(5～10 mm)5例。术后即刻造影有微量残余分流4例,原有的主动脉瓣返流无明显加重;随诊3～18个月,超声心动图示4例残余分流,分别在术后3～6个月消失,主动脉瓣局限返流无进一步增加,其中6例返流减少,未出现三尖瓣狭窄、主动脉瓣狭窄及主动脉瓣穿孔,心电图示无Ⅲ度房室传导阻滞等严重心律失...     

小儿室间隔缺损伴主动脉瓣脱垂41例报告

主动脉瓣脱垂(AVP)是室间隔缺损(VSD)的重要并发症,占VSD10.3％。脱垂往往随年龄增长而逐渐加重,一旦出现主动脉瓣返流(AR),病情比单纯VSD易于恶化,预后差。因此AVP早期诊断至关重要,目前超声心动图普遍用于临床,对早期发现AVP提供方便。现将我院自1983～1991年间住院治疗的41例VSD伴AVP的心脏超声及手术结果报告如下。     

Doty法矫治小儿先天性主动脉瓣上狭窄的疗效

目的 总结Doty法矫治小儿先天性主动脉瓣上狭窄的临床疗效.方法 2009年1月至2013年6月,Doty法矫治小儿先天性主动脉瓣上狭窄12例,其中合并Williams综合征5例.其中男9例,女3例;年龄9个月～12岁[(5.3±4.2)岁];体质量7.5～ 32.0(11.5±5.3)kg.心脏超声和增强CT明确诊断,局限型9例,弥散型3例;跨主动脉瓣上收缩压差6.86 ～16.23(11.07±3.68) kPa,压差＞13.33 kPa者3例;合并左、右肺动脉分支狭窄3例,主动脉瓣二瓣化2例,主动脉瓣轻度返流2例,动脉导管未闭2例,轻度狭窄1例,主动脉瓣下膈膜1例,肺动脉瓣狭窄1例.1例二尖瓣中-重度关闭不全.12例患儿均在全麻体外循环下行Doty法矫治术,并处理合并畸形.结果 体外循环时间68～ 129(85±26) min;主动脉阻断时间43 ～68(51±16) min.术后早期无死亡,无严重低心排出量综合征;5例轻度ST段改变,3例偶发期前收缩;呼吸机应用7～58 h,心脏重症监护室治疗时间1 ～5 d;超声心动图检查提示跨主动脉瓣上收缩压差1.01 ～3.76(2.18±1.09) kPa;有效随访患儿11例,随访6个月～3年,无死亡,无头晕、心悸.除2例患儿外,其余残余压差均低于3.33 kPa.结论 Doty法治疗小儿先天性主动脉瓣上狭窄可获得满意的疗效.     

小儿主动脉瓣下狭窄的外科治疗及疗效观察

目的 探讨小儿主动脉瓣下狭窄(subvalvular aortic stenosis,SAS)的外科治疗方法并观察其治疗效果.方法 统计1990年1月至2010年12月我院收治并进行手术治疗的22例SAS,男15例,女7例,年龄8个月至15岁,平均(5.93±3.90)岁.随访时间1个月至13.5年,平均(7.30±5.60)年.22例中单纯SAS 5例,合并其他心内畸形为:室间隔缺损16例,右室双腔2例,动脉导管未闭2例.22例患儿全部进行外科手术治疗,行单纯隔膜状狭窄或肌纤维环切除术18例,加左室肥厚心肌切除术3例.1例合并动脉导管未闭患儿先经左胸行动脉导管结扎术,随访6年后再次手术行单纯隔膜狭窄切除术.结果 所有患儿手术顺利,22例中治愈19例(86.36％),复发3例(13.64％),无死亡病例.治愈病例中,临床症状均消失,经超声心动图检查,均未见主动脉瓣下狭窄复发,无左室到主动脉收缩期压力阶差.3例复发患儿随访时间分别为4年、10年、12年.结论 小儿主动脉瓣下狭窄的外科治疗关键是彻底解除左室流出道梗阻,避免二尖瓣、主动脉瓣及传导束损伤.术中梗阻解除不彻底以及术后局部组织过度生长可能是SAS术后复发的重要因素.     

儿童主动脉瓣病变的外科治疗策略

目的 探讨儿童主动脉瓣病变的外科治疗策略.方法 回顾性分析2010年1月至2016年1月间收治的13例主动脉瓣病变患儿的临床资料.其中,男9例,女4例;年龄1～12岁,年龄分布1～3岁6例,3～6岁3例,6～12岁4例;体重12～36 kg,平均24 kg.术前经心脏超声及心脏CT明确诊断,先天性主动脉瓣病变8例,后天获得性主动脉瓣病变5例.手术在全身麻醉、低温、体外循环下进行,结合超声、CT及术中情况决定手术方式.结果 全组13例中,行改良Ross手术4例,主动脉瓣成形术3例,主动脉瓣机械瓣置换手术3例,Bentall术1例,Ross手术2例.全组无手术死亡,术中采用冷血停跳液或冷晶体停跳液灌注,合并主动脉关闭不全时,切开直接灌注,术中均应用超滤.术后有效随访患儿11例,2例患儿未规律随访,随访时间6个月～5年,全部存活,正常生活不受影响.机械瓣置换的长期使用华法林抗凝,目前无并发症出现.2例随诊过程中发现瓣膜压差大于40 mmHg,一般状况可,随访中未发现明显瓣膜严重反流.结论 儿童主动脉瓣病变的外科治疗方法有多样性的特点,本文旨在为心外科医师在儿童主动脉瓣病变的治疗策略方面提供更多的临床经验,儿童主动脉瓣疾病治疗的临床效果还有待进一步探讨.     

儿童先天性主动脉瓣上狭窄的外科治疗

目的 回顾总结儿童先天性主动脉瓣上狭窄(SVAS)的外科治疗经验,提高治疗水平.方法 1990年至2006年10月25例先天性主动脉瓣上狭窄患儿接受手术治疗.其中男16例,女9例,手术平均年龄(5.4±2.4)岁,平均体重(12.6±3.5)kg.影像学检查发现22例为局限性狭窄,主动脉瓣上0.5～2.0 cm处有狭窄段,3例为弥漫性狭窄.平均压力阶差(81.7±21.5)mmHg,William's综合征12例.所有患儿均在体外循环下行补片扩大主动脉成形术,11例以"泪珠"状补片扩大成形修补,14例以"裤衩"状补片扩大加宽主动脉狭窄部.补片材料选择应用自体心包17例,Dacron涤纶补片2例,Gore-tex人工血管补片6例,其中内衬自体心包片1例.对伴发畸形同时加以纠治.结果 术后检查主动脉瓣上流速、压差均明显下降,平均(26.0±13.2)mmHg,住院死亡1例,存活24例.结论 应用"泪珠"状和"裤衩"状补片进行主动脉扩大成形术矫治儿童主动脉瓣上狭窄有效,尤以"裤衩"状补片为首选,婴幼儿补片材料以自体心包为宜.     

大动脉转换术后瓣膜反流的危险因素分析

目的 随访分析大动脉转换术(arterial switch operation, ASO)后的主动脉瓣反流和肺动脉瓣反流情况,以进一步评价ASO中长期疗效. 方法 1999年12月至2007年12月采用大动脉转换术纠治完全性大动脉错位(TGA)和右心室双出口伴肺动脉瓣下室间隔缺损(Taussig-Bing)术后生存288例,随访到228例,随访时间(20.4±18.6)个月.根据ASO术后彩色多普勒超声报告所测主、肺动脉瓣闭合情况,分析瓣膜反流的危险因素.结果 室间隔完整型大动脉错位(TGA/IVS),大动脉错位伴室间隔缺损(TGA/VSD),Taussig-Bing和Ⅱ期大动脉转换术(Stage-Switch),四组间的主动脉瓣反流发生率差异有统计学意义(P=0.010),而肺动脉瓣的反流率差异无统计学意义(P＞0.05).多因素分析提示主动脉瓣反流(AR)危险因素是手术时年龄(＞60d),AO/PA值(＞1.10),出院时主动脉瓣有反流.单因素分析提示肺动脉瓣反流(PR)危险因素与出院时肺动脉瓣是否存在反流有关.结论 主动脉瓣随着随访时间延长反流发生率增加.ASO术毕时主动脉瓣或肺动脉瓣的轻度反流,随访中可以消失或者减轻.ASO术后须定期随访,不但要注意吻合口的生长,还要观察主动脉瓣和肺动脉瓣的功能.     

经皮导管介入封堵治疗膜周部室间隔缺损合并主动脉瓣脱垂的疗效及预后

目的　分析经皮导管介入封堵膜周部室间隔缺损合并主动脉瓣脱垂（aortic valve prolapse,AVP）的疗效及预后。方法　回顾2011年1月至2017年1月上海交通大学医学院附属上海儿童医学中心收治的1108例经皮介入治疗膜周部室间隔缺损患儿的临床资料,主要包括手术前后超声心动图、术中造影、封堵器型号及社会人口（年龄、体重、性别）等资料。组间比较计量资料采用独立样本t检验、非参数Mann-Whitney U检验;配对设计计量资料不符合正态分布采用非参数Wilcoxon符号秩检验。计数资料采用Fisher确切概率法;成组设计R×C表格采用Monte Carlo近似确切概率法。多元Logistic回归分析术前合并AVP患儿术后主动脉瓣反流（aortic regulation,AR）出现或加重的危险因素。 结果　55例术前合并AVP患儿中8例（14.5%）发生术后AR出现或加重。1053例术前不合并AVP的患儿中10例（0.9%）发生术后AR出现或加重,但AR加重情况多发生在术后远期（＞1年）,且无一例中度以上的AR出现。合并AVP患儿介入手术前后左心室射血分数、左室短轴缩短率及AR加重程度均无统计学意义。结论　在选择合适封堵器类型及大小、操作规范并具有相当相关操作经验的儿童心血管中心,经皮导管介入封堵合并AVP的膜周部室间隔缺损可成为外科手术的有效替代方案。     

Absent aortic valve: A complex anomaly

Summary Seven patients (four previously cited and three new cases) with absent aortic valve cusps (leaflets), a rare and underrecognized complex congenital heart defect, are discussed. All patients were male, six full-term and one premature with nonimmunologic hydrops. None underwent operation; all died within the first week of life from low cardiac output and hypoxemia.In most instances, the only remnant of the aortic valve was a nonobstructive fibrous ridge; occasionally, it was accompanied by rudimentary leaflets or sinuses of Valsalva. Absent aortic valve was associated with other significant structural malformations in all instances, including atrioventricular valve atresia, hypoplasia or dysplasia, less commonly double outlet right ventricle, abnormal pulmonary venous connection, or left ventricular endomyocardial abnormalities. Recognition of this unusual lesion is important since it is associated with other complex malformations, causes hypoxemia (for which early positive pressure ventilation is indicated), and could be possibly palliated using the right ventricle as the systemic ventricle.     

Coronary Artery Dilatation and Aortic Outflow Tract Enlargement in Children with Unicommissural Aortic Valves

We evaluated the aortic outflow tract (AOT) and coronary artery dimensions in pediatric patients with unicommissural aortic valves. A retrospective review of an echocardiographic database identified 37 patients with unicommissural aortic valves. A total of 115 echocardiograms were reviewed, and the right coronary artery (RCA), left main coronary artery (LM), left anterior descending coronary artery aortic valve annulus, aortic root, sinotubular junction (STJ), and ascending aorta were measured and z scores determined. The aortic stenosis peak gradient and the amount of aortic regurgitation (AR) were also measured. The RCA diameter (z score, 1.85 ± 1.8, p = 0.03) and LM diameter (z score, 1.74 ± 1.47, p = 0.04) are significantly dilated, as are all the AOT measurements: aortic annulus (2.02 ± 1.9, p = 0.02), aortic root (2.25 ± 1.9, p = 0.02), STJ (2.22 ± 1.74, p = 0.01), and ascending aorta (4.38 ± 2.03, p < 0.001). Longitudinal follow-up showed that there was no significant variation over time in any variable. The AOT measurements were significantly correlated with each other. A trend was found in which an increasing amount of AR gave an increase in AOT measurements. The aortic gradient was not significantly associated with any measurement. Our study population demonstrated significant dilatation of the RCA and LM as well as the AOT. The dilatation of the AOT structures is likely caused by the same mechanism that accounts for the AOT dilatation in patients with bicommissural aortic valves. Dilatation of the coronary arteries may represent an intrinsic abnormality in the vessel wall. Further studies are needed to define possible changes. Work was performed at Johns Hopkins Hospital, Baltimore, MD 21287, USA.     

Echocardiographic Follow-Up of Congenital Aortic Valvular Stenosis

We investigated the morphology of the stenotic aortic valve, the progression of the stenosis, and the onset and progression of aortic regurgitation (AR) in patients with congenital aortic valvular stenosis (AVS). The medical records of 278 patients with AVS were reviewed, with the patients with concomitant lesions besides AR excluded. Very mild aortic stenosis was defined as a transvalvular Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 4.9 ± 4.3 years (range, 3 days to 15 years), and 203 (73%) were male. The number of the cusps was determined with two-dimensional echocardiography in 266 patients (95%): unicuspid in 3 patients (1%), bicuspid in 127 patients (48%), and tricuspid in 136 patients (51%). A total of 192 of all patients were followed for 2 months to 14.6 years (mean 4.2 ± 3.3 years) with medical treatment alone. Among 72 patients with very mild stenosis at initial echocardiographic examination, 20% had mild, 3% moderate, and 1% severe stenosis after a mean period of 3.7 years. In 70 patients with mild stenosis at initial echocardiographic examination, 28% had moderate and 9% severe stenosis after a mean period of 5 years. Among 44 patients with moderate stenosis at initial echocardiographic examination, 36% had severe stenosis after a mean period of 3.7 years. Among 192 patients, 40% had AR (3% trivial, 28% mild, and 9% moderate) at initial echocardiographic examination. After a mean period of 4.2 years, 58% of the patients had AR (13 % trivial, 25% mild, 16% moderate, and 4% severe). There was not statistically significant difference between catheterization peak systolic gradients (47 ± 16 mmHg) and Doppler estimated mean gradients (45 ± 9 mmHg) (p = 0.53), whereas Doppler PSIGs (74.9 ± 15.7 mmHg) were higher than catheterization peak systolic gradients (p < 0.0001) in 25 patients who were studied in the catheterization lab. Patients with very mild stenosis may be followed with a noninvasive approach every 1 or 2 years, and an annual follow-up is suggested for patients with mild stenosis. Nearly one-third of patients with moderate stenosis at initial echocardiographic examination had severe stenosis after a mean period of 3.7 years. Therefore, we recommend, that patients with moderate stenosis undergo noninvasive evaluation every 6 months. Doppler estimated mean gradient is very useful in predicting the need for intervention in children with AVS.     

自体心包修复儿童主动脉瓣狭窄9例病例系列报告

目的：总结并分析以自体心包修复儿童先天性主动脉瓣狭窄的手术方法和早期预后。方法：纳入2013年7月至2015年6月在复旦大学附属儿科医院行自体心包主动脉瓣成形术的先天性主动脉瓣狭窄患儿,收集患儿的一般资料,围手术期情况,并发症,术前、术中、术后和随访时的超声心动图资料。结果：符合本文纳入标准的9例患儿进入本文分析,男6例,女3例,年龄4月龄至9岁。术前超声提示,三叶式和二叶式主动脉瓣分别为3例和6例;重度狭窄6例,中度狭窄1例,重度狭窄伴中度返流2例。9例均以自体心包再造或扩大主动脉瓣瓣叶。术后即刻与术前超声心动图比较：主动脉瓣最大跨瓣压差［（31.6±9.4）mm Hg vs（73.0±22.2）mm Hg, P=0.000］和主动脉瓣平均跨瓣压差［（15.8±18.3）mmHg vs （35.8±18.3）mmHg, P= 0.004］均下降。术后随访24～48（32±8）月,无死亡和再干预病例,未见严重不良事件,未见主动脉瓣重度狭窄或重度反流、升主动脉狭窄或扩张、主动脉瓣瓣环狭窄与扩张、瓣膜脱垂或瓣膜赘生物病例;3例患儿分别在随访18、24和12个月时出现瓣叶活动僵硬,瓣叶开放不完全。术后左室后壁厚径均呈下降趋势;末次随访时,8例患儿左室后壁厚径Z值（Z-LVPWd）均下降至正常水平（＜2）。结论：以自体心包修复主动脉瓣可改善先天性主动脉瓣狭窄患儿的血流动力学,手术风险低,术后早期疗效可,避免或延缓儿童主动脉瓣置换术,减少施行ROSS手术机会。     

应用ROSS手术治疗儿童主动脉瓣病变的研究

目的评价应用Ross手术治疗儿童主动脉瓣病变的效果。方法回顾性分析1994年1月-2005年1月Ross手术治疗儿童主动脉瓣病变26例，其中男18例，女8例，平均年龄（12．4±5．3）岁。主动脉瓣赘生物7例，主动脉瓣二瓣叶畸形9例，主动脉瓣叶脱垂6例，瓣叶发育不良3例，主动脉瓣成形术后再发主动脉瓣关闭不全1例。所有患儿均采用自体带瓣肺动脉行主动脉根部替换，均应用同种肺动脉带瓣管道重建右室流出道。结果本组26例无手术死亡，1例术后大出血而2次开胸止血。在2例12年、24例（24．5±3．8）个月的随访期间，1例术后1年同种肺动脉瓣感染致感染性心内膜炎再次手术时死亡。25例血流动力学优良，23例无主动脉瓣反流，2例仅存在轻微主动脉瓣反流；4例有同种异体肺动脉瓣轻度反流，1年后左室舒张末径从术前的（65。24±12．7）mm显著缩小到（49±8．23）mm（P〈0．05）。结论应用Ross手术治疗儿童主动脉瓣病变可取得较好的围术期及近中期效果。     

Normal Mitral and Aortic Valve Areas Assessed by Three- and Two-Dimensional Echocardiography in 168 Children and Young Adults

Our purpose was to investigate the effects of body size on the sizes of mitral (MV) and aortic valve (AV) areas by three-dimensional (3-D) and two-dimensional (2-D) echocardiography and to create the normal values for 3-D echocardiography. A total of 168 healthy subjects aged 2–27 years were studied by digitized 3-DE, 2-DE, and Doppler echocardiography. 3-D echocardiography was performed by using rotational acquisition of planes at 18° intervals from a parasternal view with electrocardiogram gating and without respiratory gating. The annular levels of MV and AV were identified from short-axis cut planes and their areas were measured by planimetry. The diameters of mitral annulus, left ventricular outflow tract (LVOT), and aortic annulus were measured by 2-DE from the apical and parasternal long-axis views. Flow indices were measured by Doppler from MV inflow and the flow in LVOT and in the ascending aorta. Both MV and AV annular areas increased linearly in relation to body size. In the total study group the estimated areas for MV were 5.2 ± 0.9 cm²/m²by 3-DE, 3.7 ± 0.5 cm²/m² by 2-DE, and 2.0 ± 0.4 cm²/m² by continuity equation. The respective values for AV were 2.7 ± 0.5, 2.1 ± 0.3, and 1.8 ± 0.4 cm²/m². MV velocity time integral (VTI)/ascending aorta VTI increased from 0.80 (0.26) to 0.95 (0.23) with increased body surface area (BSA), whereas MV VTI / LVOT VTI was 1.2 (0.2) in all BSA groups. MV and AV annulus areas increase linearly in relation to body size. 3-DE gives greater estimates for the areas than 2-DE and Doppler equation methods. The data obtained from 168 healthy subjects may serve as a reference for clinical use in patients with various cardiac abnormalities.     

The Malignant Course of Acute Rheumatic Fever in the Modern Era: Implications for Early Surgical Intervention in Cases of Bivalvular Insufficiency With Impaired Ventricular Function

In recent years, the incidence of acute rheumatic fever (ARF) has drastically decreased in the United States while remaining common in developing nations. However, immigration to the United States from developing nations has resulted in the continued prevalence of ARF. Patients with ARF face the potential development of significant valvular insufficiency and rheumatic carditis, which are associated with significant morbidity and mortality. Potentially adverse outcomes may be avoided with advanced interventions such as aortic and/or mitral valve replacement or repair, the use of inotropic agents, and ventricular assist devices for mechanical circulatory support. This report describes a series of three ARF carditis cases. This series serves not only to emphasize proper ARF prevention, but also to demonstrate the need for timely surgical intervention once medical therapy has failed.     

Predictors of Left Ventricular Performance After Valve Replacement in Children and Adolescents with Chronic Aortic Regurgitation

Aortic valve replacement has been recommended in patients who have severe symptoms, in patients with extreme left ventricle (LV) dilatation (end diastolic dimension >4 SD above normal) or LV ejection fraction <50%. However, the occurrence of advanced symptoms or severe LV dilatation raises concern about irreversible LV dysfunction. This study sought to determine the influence of preoperative symptoms, LV size and function on mortality, and postoperative LV performance in children and adolescence after valve replacement for aortic regurgitation (AR). A total of 49 patients 18 years old or younger (mean, 13.9 ± 3) who underwent valve replacement for chronic AR between 1991 and 2001 were followed up for 1–10 years (mean, 3.3 ± 2.1). Baseline and postoperative characteristics were compared between 13 patients (group 1) with extreme LV dilatation and 34 patients (group 2) with a lesser degree of LV enlargement. Preoperative low ejection fraction (p < 0.008), extreme LV dilatation (p < 0.05), and LV end systolic dimension >4 SD above normal (p < 0.05) were predictors of persistent LV dysfunction. Extreme LV dilatation (p < 0.0003), LV end systolic dimension (p < 0.0007), and reduced LV ejection fraction (p < 0.01) predicted persistent LV dilatation. In the setting of chronic AR, preoperative symptoms, LV systolic function, and LV internal dimensions are the main predictors for persistent LV dysfunction and dilatation. Surgical correction should be performed before LV systolic dysfunction and/or extreme LV enlargement occurs.     

Pressure Recovery in Pediatric Aortic Valve Stenosis

This study was designed to evaluate the phenomenon of pressure recovery in pediatric patients with aortic stenosis and also to evaluate how observed differences between catheter and Doppler gradients can be predicted by Doppler echocardiography. Doppler measurements of aortic valve stenosis gradients are known to overestimate observed gradients in the catheterization laboratory. Pressure recovery has been shown to be a contributing factor to this discrepancy. However, the clinical relevance of correcting Doppler gradients using the pressure recovery equation has not been evaluated in the pediatric population. Simultaneously obtained catheter and Doppler gradients were studied in 14 patients (range, 0.03–18 years; mean, 4.1 years) with aortic valve stenosis. A total of 23 data points were measured because 9 patients underwent balloon valvuloplasty and had both a pre- and a post-balloon valvuloplasty data point in the study. The catheter gradients were then compared to peak, mean, and pressure recovery corrected Doppler gradients. Pressure recovery was calculated using a previously validated equation. As expected, measured echocardiographic continuous-wave peak Doppler gradients overestimated the observed catheter gradients (range, 16–93 mmHg; mean, 43 mmHg). The continuous-wave peak Doppler gradients, mean, and pressure recovery adjusted gradients were equally as good in correlating the observed catheter gradients to those obtained by Doppler echocardiography (r = 0.92). However, pressure recovery corrected Doppler gradients were in better agreement with catheter gradients than echocardiographic mean or peak Doppler gradients (95% limit of agreement: –9 to 19 mmHg for pressure recovery corrected gradients, –30 to 11 mmHg for mean Doppler gradients, and 2–83 mmHg for peak Doppler gradients). Measured continuous-wave peak Doppler gradients consistently overestimated catheter gradients. The noted differences may be predicted using the pressure recovery equation. Pressure recovery is a significant factor in children with aortic valve stenosis.