Correlation of Plasma B-Type Natriuretic Peptide with Shunt Severity in Patients with Atrial or Ventricular Septal Defect

The goal of this study was to test the utility of bedside plasma concentration of B-type natriuretic peptide (BNP) assay as a screen for large shunts in pediatric patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). Thirty-five children at a mean age of 70 ± 129 weeks with ASD or VSD were included in the study. Nine patients had VSD and 26 had ASD. Plasma BNP values were compared with the Q _p/Q _s ratios derived from quantitative Doppler flow measurements. Mean BNP was 29 ± 42 pg/ml, with a range between <5 pg/ml and 208 pg/ml. Sixteen patients had Q _p/Q _s values >1.5 and 19 had values <1.5. The difference of mean BNP in these patient groups was statistically significant (45 ± 56 vs 14 ± 17, p = 0.03). BNP was positively correlated with shunt significance. Receiver operating characteristic curve analysis revealed a sensitivity of 69% and a specificity of 79% at a plasma BNP cut-off level of ≥20 pg/ml. Bedside measurement of BNP correlates with magnitude of ASD and VSD in children. BNP can provide information for the management of children with ASD or VSD. It can be used as part of the evaluation of a child with a preliminary diagnosis of a congenital defect.     

Noninvasive Prediction of Pulmonary Artery Pressure in Patients with Isolated Ventricular Septal Defect

Management of patients with isolated ventricular septal defect (VSD) requires information regarding pulmonary artery pressure (PAP). The purpose of this study was to evaluate the individual predictive value of noninvasive methods for assessment of PAP and to determine if any combination of techniques significantly improved their predictive power. We reviewed the clinical history, electrocardiogram, and echocardiogram of 31 patients (age 1.9 ± 1.73 years) who underwent catheterization for isolated VSD. Noninvasive data were compared for patients with mean PAP <20 mmHg (group 1) and those with mean PAP ≥20 (group 2) at catheterization. Fourteen (45%) patients were in group 1 and 17 (55%) in group 2. Doppler estimation of VSD gradient, right ventricular hypertrophy by echocardiogram, interventricular septal orientation, and VSD size had predictive value for elevated mean PAP (p < 0.01). All patients (n= 6) with normal findings in all four variables had normal PAP. All patients (n= 12) with at least three of four abnormal findings had elevated PAP. Six patients in group 1 had at least one variable that incorrectly predicted high PAP, whereas 3 patients with normal findings on three of the four variables nevertheless had elevated PAP. No single noninvasive variable accurately predicted PAP in all cases. However, normal findings for all four significant variables did predict normal PAP and suggest that cardiac catheterization is unnecessary in that setting. However, any other combination of normal and abnormal findings for the four significant variables did not reliably predict PAP and such patients may require catheterization to directly measure PAP.     

Severe Left Ventricular Hypoplasia in Patients with Unbalanced Incomplete Atrioventricular Septal Defect and Pulmonary Hypertension: Feasibility of Biventricular Repair

We report three patients with partial atrioventricular septal defect who presented in the neonatal period with excessive left to right shunting and progressive pulmonary hypertension. Successful biventricular repair was accomplished despite the fact that left ventricular area and left sided structures did not meet the criteria established previously for biventricular management in children with critical aortic stenosis or complete atrioventricular septal defect. Indication for biventricular management was based on the fact that none of our patients had morphological mitral or aortic stenosis and that the hypoplastic left ventricle proved capable to maintain the systemic circulation following closure of the arterial duct. Fenestrated closure of the atrial septum and an individualized approach regarding the closure of the cleft in the left sided atrioventricular valve appear to be important surgical options to reduce postoperative left atrial pressure and to allow the ventricles to adapt to the new loading conditions.     

内皮素-1和von Willebrand因子在室间隔缺损并肺动脉高压患儿肺血管的表达及意义

目的观察室间隔缺损（VSD）并肺动脉高压（PH）患儿内皮素-1（ET-1）与von Willebrand因子（vWF）在肺血管内膜表达的差异，探讨两者在PH发生发展及肺血管重建中的作用。方法VSD无PH患儿10例为对照组；VSD并PH患儿20例为PH组（PH组），根据肺动脉平均压分为中度PH组（Ⅰ组）和重度PH组（Ⅱ组）。取右肺中叶肺组织，免疫组织化学染色，测光密度值。结果肺小动脉vWF、ET-1表达在对照组与PH组、对照组与Ⅰ组、对照组与Ⅱ组、Ⅰ组与Ⅱ组之间，均有显著性差异（P均〈0．01）；而肺小静脉vwF、ET-1表达均无统计学差异（P〉0．05）。同组别肺小动脉与肺小静脉vwF、ET-1表达有显著性差异（P均〈0．01）。ET-1、vWF、肺动脉平均压、VSD直径与主动脉直径之比四者间相互呈正相关（P〈0．01，0．05）。结论ET-1及vWF与PH的发生发展、肺血管重建有关。肺组织中ET-1的主要来源可能是肺小动脉。     

Unusual Hemodynamic Changes in an Infant with a Restrictive Ventricular Septal Defect

A 7-month-old asymptomatic infant was known to have a restrictive membranous ventricular septal defect partially closed by an aneurysm of the membranous septum. At 13 months of age, he developed unexpected pulmonary hypertension, with no clinical sign of cardiac failure. Cardiac catheterization assessed the pulmonary artery pressure at a systemic level with significant left-to-right shunt. After surgical closure, the pulmonary arterial pressure decreased by 50%. We suspect an enlargement of the ventricular septal defect caused by the rupture of the aneurysm of the membranous septum—a rare complication.     

Anomalous Origin of Left Pulmonary Artery from Aorta with Atrial Septal Defect

Anomalous origin of left pulmonary artery (ALPA) from aorta with atrial septal defect (ASD) is a rare congenital cardiac malformation. We report a case of ALPA from the aorta with ASD in a 1.8-kg baby. Repair was needed at an early age due to progressive congestive heart failure. Surgical correction was performed with cardiopulmonary bypass for closure of ASD and direct anastomosis of the ALPA onto the main pulmonary artery.     

Percutaneous Closure of Ventricular Septal Defect Associated with Tunnel-Shaped Aneurysm Using the Amplatzer Duct Occluder

Different devices have been used for percutaneous closure of ventricular septal defects (VSDs). Over the past 6 months, we have used the Amplatzer duct occluder for VSDs associated with tunnel-shaped aneurysm in three patients here at our institution. Due to the anatomic resemblance between such VSDs and the duct occluder, we selected this device for percutaneous closure. There was trivial residual shunt in one patient. All three patients tolerated the procedure well and there were no untoward complications.     

室间隔缺损患儿血清氮末端脑钠素原测定的意义

目的研究血清氮末端脑钠素原(NT-Pro-BNP)在室间隔缺损(VSD)并心力衰竭(心衰)中的意义,为先天性心脏病并心衰的诊断和治疗提供依据。方法测定51例儿童血清NT-Pro-BNP水平。其中包括VSD患儿29例,正常儿童22例。同时行心脏彩超心功能测定,包括左心室射血分数(LVEF)和左室短轴缩短率(LVFS)等指标。结果NT-Pro-BNP值呈对数正态分布,随心衰加重,NT-Pro-BNP水平依次呈升高趋势。但无心衰组与轻度心衰组NT-Pro-BNP差异无统计学意义。重度心衰组彩超心功能测定LVEF、LVFS值与其他组间有差异,其他组之间无差异。各组心功能测定值均未达到诊断心衰的参考标准。NT-Pro-BNP水平与临床诊断心衰有较好的相关性(r=0.826),但LVEF、LVFS值与心衰无相关性。结论血NT-Pro-BNP水平可反映VSD并心衰的严重程度,且可依据其血清水平进行心衰分级。LVEF、LVFS反映小儿VSD伴心衰时不敏感,无临床意义。     

Congestive Heart Failure During Early Infancy in Patients with Ventricular Septal Defect Relative to Early Closure

The hemodynamic features of 44 patients requiring surgical closure of a ventricular septal defect (VSD) during early infancy were evaluated. The VSD was closed within the first 6 months of life in 29 patients (group A) and during the second 6 months in 15 patients (group B). The left-to-right (L–R) shunts were significantly greater in group A than in group B, although in all patients they were more than 50%. In contrast, the left ventricular end-diastolic volume was significantly greater in group B than in group A, whereas the right ventricular end-diastolic volume was greater in group A; the difference was not statistically significant. The step-up in blood oxygen saturation in the lower right atrium, which was significantly more in group A, indicated the L–R shunt across the patent foramen ovale (PFO). The L–R shunt across the VSD during diastole was also detected in all patients angiographically. With early infantile VSD, the larger the L–R shunt the greater was the likelihood of early surgery. Right ventricular volume overload caused by the L–R shunt across a PFO as well as through the VSD during diastole is a useful indicator of a large shunt in infants with VSD.     

Nesiritide for the Treatment of Pulmonary Hypertension and Cor Pulmonale in an Infant

Nesiritide is a synthetic form of B-type natriuretic peptide. It is approved for the treatment of acute exacerbations of congestive heart failure in hospitalized adult patients. It is currently under investigation for use in other settings and other patient populations. This article describes administration of nesiritide to an infant patient with severe pulmonary hypertension and cor pulmonale. No adverse reactions occurred during administration of the drug. Specifically, there was no hypertension, vomiting, arrhythmia, or changes in renal function. No changes in renal function occurred in the months subsequent to treatment. This case report illustrates that nesiritide can be safely administered to critically ill infants with pulmonary hypertension and cor pulmonale. Our patient experienced a decrease in pulmonary pressure and improved clinical condition during and after the infusion. However, further study is required to fully evaluate the safety and efficacy of nesiritide for these patients.     

婴幼儿室间隔缺损合并肺动脉高压的外科治疗

目的 报道婴幼儿先天性室间隔缺损(VSD)伴肺动脉高压的手术修补结果及体会。方法 1998年1月-2002年12月，对86例VSD伴肺高压的婴幼儿采用中深低温体外循环行手术治疗。术中术后加强心肌保护和呼吸道管理。结果 治愈出院83例，死亡3例，死亡率为3．49％(3／86)，均为肺高压临床4级病例。术后主要并发症为肺部感染、肺高压危象、低心排综合征。出院病人随访6月～5年，无死亡病例，心功能(NYHA)一级62例，二级18例，三级3例。结论 婴幼儿VSD合并肺动脉高压者，应积极早期手术。围术期的心肌保护和呼吸道管理是手术成败的关键。     

B-Type Natriuretic Peptide and Heart Failure in Patients with Ventricular Septal Defect: A Pilot Study

In adults without congenital heart disease, B-type natriuretic peptide (BNP) has been shown to be a very sensitive and specific marker of heart failure. The utility of BNP as a marker of clinical heart failure in children with a ventricular septal defect (VSD) has yet to be determined. A prospective, observational study evaluated BNP levels and other measures of heart failure. Eligible patients were <2 years old, scheduled to undergo surgical repair of a VSD, and without other significant structural heart disease. Data collected before and after surgical repair included echocardiographic measurements, electrocardiographic (ECG) findings, Ross score, BNP measurements, and weight gain. A total of 21 patients were enrolled and 14 patients had complete postoperative follow-up data. For patients with complete data, mean BNP decreased by 94 pg/ml (118 pre vs. 24 post; paired t-test, p = 0.041), mean left ventricular end-diastolic dimension z-score decreased by 1.75 (+0.86 vs. ?0.89; paired t-test, p = 0.013), mean weight z-score change per month increased by 0.35 (?0.25 vs. +0.10; Wilcoxon test, p = 0.013), and the incidence of biventricular hypertrophy on ECG decreased (46% vs. 0%; McNemar test, p = 0.031). The change in BNP showed a trend toward a negative correlation with weight z-score change per month (r = ?0.531, p = 0.075). In conclusion, BNP, along with other measures of heart failure, decreased following VSD repair, and the change in BNP was most closely correlated with improved weight gain.     

脑钠肽在新生儿持续肺动脉高压诊断和治疗中的意义

目的 探讨脑钠肽(BNP)在新生儿持续肺动脉高压(PPHN)诊断和治疗中的临床应用价值,明确BNP是否可以作为判别PPHN的生化定量指标.方法 采用前瞻性对照研究,对54例发绀患儿及时行心脏彩色超声Doppler检查,结合临床鉴别出PPHN组29例、non-PPHN组25例,以同期住院无发绀患儿12例作为对照组.应用免疫荧光分析测定66例患儿血清BNP水平,比较各组患儿间BNP水平差异,应用SPSS 13.0统计软件描绘ROC曲线,评价该方法的价值及寻找判断PPHN的最佳临界值.结果 PPHN组新生儿BNP水平[(1 937.40±1 392.36) ng·L-1]显著高于non-PPHN组新生儿[(537.00±731.49) ng·L-1]和对照组[(50.72±36.50) ng·L-1](F=19.42,P<0.001),而non-PPHN和对照组BNP水平比较无统计学意义(P>0.05);ROC曲线下面积为0.903,95%可信区间0.818～0.988;判别PPHN的最佳BNP水平为807 ng·L-1,以此为标准,判别PPHN的灵敏度为0.828,特异度为0.880.结论 PPHN新生儿血清BNP水平明显升高,而BNP可作为一种动态观察指标,在判别PPHN的发生及指导合理治疗、判断预后中有重要临床意义.     

小儿膜周部室间隔缺损介入治疗的临床疗效

目的观察小儿膜周部VSD介入治疗的疗效、对心律的影响及封堵前后左心血流动力学指标变化。方法收集2006年8月-2009年12月本科44例接受介入治疗的膜周部VSD患儿。男17例,女27例;年龄2.4～13.0(5.20±2.68)岁。其中37例选用对称型封堵器,7例选用偏心型封堵器,经造影和经胸超声心动图证实封堵器位置良好,无成束残余分流,主动脉瓣和三尖瓣功能不受影响,即为封堵成功。术前,术后3 d、1个月、3个月、6个月、12个月检查其ECG及经胸超声心动图检测左心室舒张末期内径(LVDD)、左心室收缩末期内径(LVDS)、射血分数(EF)和左心室缩短分数(FS)。结果 43例封堵成功,成功率为97.7%。其中2例有不成束残余分流,分别于术后3 d和术后1个月时消失。术后新出现不完全右束支传导阻滞4例,1例12个月时转为完全性右束支传导阻滞,1例随访中不完全右束支传导阻滞消失,无高度房室传导阻滞发生。与术前相比,LVDD和LVDS在术后3 d和术后1个月时明显缩小(P<0.01,0.05),术后12个月时虽短于术前,但差异无统计学意义,EF和FS术前术后无显著变化。结论小儿膜周部VSD介入治疗是一种安全有效的方法。     

Pulmonary arterial changes in patients with ventricular septal defects and severe pulmonary hypertension

Summary In 25 patients, aged eight months to 31 years, with ventricular septal defect (VSD; isolated in 15, the others with atrial septal defect, PDA, coarctation or patent ductus arteriosus + coarctation), each with severe pulmonary artery hypertension (pulmonary artery systolic pressure [Ppa] at least 75% of systemic and an elevated pulmonary vascular resistance), we related morphologic and morphometric data from open-lung biopsy to hemodynamic measurements obtained at cardiac catheterization during the same hospital admission. Of the hemodynamic features measured, only the ratios of pulmonary-to-systemic flow and pulmonary-to-systemic resistance correlated significantly with structure. Neither pulmonary artery pressure (Ppa) nor pulmonary vascular resistance correlated significantly with any structural feature studied. The increased external diameter of respiratory bronchiolar arteries in those with the more advanced Heath-Edwards grades reflects dilatation and suggests that it is in the small arteries of the distal arterial bed that the changes of pulmonary hypertension are most significant. Neither age nor body weight correlated significantly with the degree of structural or hemodynamic abnormality. In the ten patients who underwent VSD closure, Ppa was measured postoperatively. The Heath-Edwards grade (no more than one grade-III lesion) and arterial density (at least one-half that normal for age) were the best correlates of the difference between preoperative Ppa and Ppa immediately after corrective surgery. The presurgical catheterization data, including pulmonary resistance and the resistance ratio, did not correlate significantly with change in Ppa following VSD closure. Lung biopsy is an important diagnostic aid because it helps in predicting the early response in postoperative Ppa in patients with VSD and elevated pulmonary vascular resistance.     

大型室间隔缺损并肺炎婴幼儿的外科治疗

目的 探讨大型室间隔缺损(VSD)并肺炎婴幼儿早期手术治疗的可行性、手术时机、适应证以及术中术后的处理.方法　选择2009年10月-2010年7月本院收治的大型VSD并肺炎患儿19例.男11例,女8例;年龄3～15(7.3±2.8)个月;体质量5～12(7.6±1.4) kg .其中VSD并房间隔缺损5例、并动脉导管未闭1例;膜周部VSD 16例,肺动脉瓣下VSD 3例.患儿均在低温、体外循环、冷晶体停跳液灌注下行VSD直视修补术及并发畸形矫治.16例膜周部VSD患儿均经右房切口修补;3例肺动脉瓣下VSD患儿中,2例经肺动脉横切口修补,1例经右室流出道切口修补.19例患儿VSD修补均采用补片修补法,其中15例应用牛心包补片,4例应用自体心包补片(戊二醛固定).患儿术中均应用膜式氧合器和术中平衡超滤.结果　19例患儿手术均获成功,术后主要并发症为肺部感染3例,切口愈合不良2例,肺不张和室间隔少量残余分流各1例,无全身感染、肺动脉高压危象以及严重低心排病例,19例患儿均治愈出院.门诊随访2～6个月,上呼吸道感染显著减少,营养发育状况改善.结论　把握手术时机和适应证,早期手术、防治术中和术后肺损伤,是治疗大型VSD 并肺炎婴幼儿的有效方法.     

Right-Sided Pulmonary Venous Obstruction Between a Right Aortic Arch and an Amplatzer Septal Occlusion Device Following Closure of a Secundum Atrial Septal Defect

Right-sided pulmonary venous obstruction between a right aortic arch and an Amplatzer Septal Occluder device developed following closure of a large secundum atrial septal defect. The obstruction was not apparent on postprocedure transesophageal echocardiogram but developed over time. The patient recovered completely following surgical removal of the device.     

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。     

Use of the Myocardial Performance Index to Assess Right Ventricular Function in Infants with Pulmonary Hypertension

Background This study aimed to measure and compare right ventricular (RV) function in normal infants and those with pulmonary hypertension (PHT) using the myocardial performance index (RV_MPI) and to investigate the relationship between RV function and pulmonary artery pressure. Methods A case-control study measured RV_MPI in 16 infants with PHT (9 of whom had congenital diaphragmatic hernia) and 28 normal control infants. For the PHT infants, 43 paired measures of RV_MPI and pulmonary artery pressure (estimated from tricuspid regurgitation jet velocity) were taken to allow investigation of the relationship between RV_MPI and pulmonary artery pressure. Results The mean RV_MPI for the control infants was 0.24 ± 0.09. The RV_MPI was significantly elevated in the PHT group (0.55 ± 0.17; p < 0.0001), including a subgroup of infants with PHT secondary to congenital diaphragmatic hernia (0.58 ± 0.18; p < 0.0001). The correlation between RV_MPI and pulmonary artery pressure in the infants with PHT (R ² = 0.05; p = 0.17) was poor. Conclusions In infants, RV_MPI allows quantification of right ventricular function and detection of RV dysfunction in PHT. No linear relationship exists between RV_MPI pulmonary artery pressure. Use of RV_MPI in the clinical setting must take into account the global and load-dependent nature of this measure.     

二维应变和应变率评估房间隔缺损患儿右心室功能的价值

目的 探讨超声右心室长轴整体收缩期应变(GLS)和应变率(GLSR)及右心室游离壁基底段应变(Sb)和中段应变(Sm)在房间隔缺损(ASD)患儿中的变化,评估二维应变和应变率对ASD患儿右心室功能的诊断价值.方法　选择ASD患儿32例.分为单纯ASD组(n=20),ASD+肺动脉高压(PAH)组(n=12).同时选择20例健康体检者作为健康对照组.保存二维超声心动图图像,导入GE VIVID 7 Echopac工作站进行脱机分析;应用二维应变分析软件,追踪右心室各节段的运动,得出右心室各节段的整体收缩期应变和应变率曲线.应用SPSS 13.0软件进行统计学分析.结果 单纯ASD组患儿GLS、GLSR、Sb、Sm水平较健康对照组显著增加(Pa＜0.05);ASD+PAH组GLS、GLSR、Sb及Sm水平均显著低于单纯ASD组(Pa＜0.05).ASD+PAH组GLS、Sb、GLSR均明显低于健康对照组,差异均有统计学意义(Pa＜0.05),Sm在健康对照组与ASD+PAH组间差异无统计学意义(P＞0.05).结论　应变和应变率可以很好地评估ASD患儿右心室功能.右心室心肌的应变和应变率受容量和压力负荷的双重影响.