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目的 探讨雾化吸入伊洛前列素进行急性血管扩张试验的价值.方法 研究对象为2007年2月至2008年5月在广东省心血管病研究所住院的50例先天性心脏病合并肺动脉高压患儿,对所有患儿进行左右心导管检查,之后肺动脉内注射酚妥拉明或雾化吸入伊洛前列素进行急性血管扩张试验,试验后重复左右心导管检查.根据Fick公式计算血流动力学参数.综合判断肺动脉高压性质,将惠几分成两组:动力组和梗阻组.动力组患儿进行手术治疗,术后定期随访并修正术前诊断.结果 酚妥拉明会显著增高受试者的心率,而伊洛前列素对心率的影响较轻微;酚妥拉明和伊洛前列素都能够降低平均肺动脉压力和肺血管阻力,升高肺循环血流量;酚妥拉明同时会降低平均主动脉压力和体循环阻力,升高体循环血流量,而伊洛前列素对体循环没有明显的影响.在使用伊洛前列素的急性血管扩张试验中,平均肺动脉压力、肺血管阻力/体循环阻力和肺循环血流量/体循环血流量等参数的变化在动力组和梗阻组中差异无统计学意义(P值分别为0.016、0.024和0.030).而使用酚妥拉明的急性血管扩张试验中,平均肺动脉压力和肺血管阻力两项参数的变化在动力组和梗阻组中差异有统计学意义(P值分别为0.017和0.004).结论 在先天性心脏病合并肺动脉高压的患儿中,使用酚妥拉明或伊洛前列素进行急性血管扩张试验都能够有效区分动力性与梗阻性肺动脉高压.酚妥拉明用药前后,肺循环和体循环的压力、阻力和血流量都有明显变化.而伊洛前列素雾化吸入以影响肺循环为主,可以保持相对平稳的血流动力学,在安全性上优于酚妥拉明.     

Regression of Pulmonary Vascular Disease After Therapy of Abernethy Malformation in Visceral Heterotaxy

A 1-year-old boy who had left isomerism and corrected transposition of the great arteries (c-TGA) with moderate-sized ventricular septal defect, severe pulmonary artery hypertension (PAH), and pulmonary vascular disease with significant right-to-left shunting received a diagnosis of type 2 Abernethy malformation, which was partly responsible for disproportionate PAH in the child. The malformation was treated by plugging of the portosystemic shunt. Follow-up cardiac catheterization on sildenafil demonstrated significant left-to-right shunting (2.16:1) and a fall in pulmonary vascular resistance, making surgical correction possible. This case highlights the importance of searching for additional rare causes of PAH in patients with congenital heart diseases when the degree of pulmonary hypertension is disproportional to the defect size.     

先天性心脏病复合畸形的介入治疗

目的探讨介入治疗小儿先天性心脏病(CHD)复合畸形的可行性和疗效。方法对CHD复合畸形患儿10例施行联合介入治疗,其中房间隔缺损(ASD)并肺动脉瓣狭窄(PS)2例,室间隔缺损(VSD)并动脉导管未闭(PDA)4例,PDA并PS 2例,ASD并PDA 2例。结果7例1次治疗成功,余3例中2例分2次治疗,1例8个月患儿,因其在最初0.5年随访中发现VSD明显缩小,故先封堵了PDA,目前正在随访中。成功率100%,无严重并发症。经1个月-3年随访,患儿心脏缩小,心脏功能改善。结论在病例选择恰当情况下,对小儿CHD复合畸形进行介入治疗是安全、可行的。     

主肺动脉窗25例临床分析

目的 分析主肺动脉窗（APW）的发病、分型和伴发畸形情况,总结诊断及治疗经验。方法 回顾性分析复旦大学附属儿科医院2003至2013年诊治的25例APW患儿的临床症状、病理分型、合并畸形、诊断、治疗和预后资料。结果 25例APW患儿进入分析,男14例,女11例,年龄5 d至13岁（6月龄以下12例）,占同期住院先天性心脏病患儿的0.18%。Ⅰ型5例（20%）,Ⅱ型16例（60%）,Ⅲ型1例（4%）,Ⅴ型3例（12%）。单纯APW 10例,APW合并单纯卵圆孔未闭3例;余12例（48.0%）均合并其他需纠正的心血管畸形,其中合并单纯房间隔缺损（ASD）1例,单纯动脉导管未闭（PDA）2例,单纯室间隔缺损(VSD)3例,右室双出口和ASD 1例,主动脉瓣狭窄和右位主动脉弓1例,肺动脉瓣闭锁伴VSD、右位主动脉弓、永存左上腔静脉1例, 主动脉弓离断及PDA 3例。超声心动图诊断17例,误诊或漏诊8例（32%）中5例通过心导管检查及造影诊断,3例外科术中诊断。25例均有不同程度肺动脉高压,其中2例心导管检查评估为阻力性肺动脉高压,4例家长放弃诊治,其余19例施行一期根治术,术后死亡2例（10.5%）。17例术后随访3个月至9年3个月,平均（23±27）个月,其中15例临床无症状,复查超声心动图APW修补无残余,肺动脉压力逐渐下降至正常。结论 APW缺损主要发生在主动脉远端,常伴有间隔缺损、PDA和主动脉弓病变等;超声心动图为首选检查方法,但必要时需行心导管检查及造影。APW患儿易早期发生肺动脉高压,一经诊断尽早手术,可获得较好的中长期疗效。     

Catheter Interventional Closure of a Large Right Pulmonary Artery–to–Left Atrial Communication in a Neonate

Right pulmonary artery?Cto?Cleft atrial communication is a rare congenital vascular malformation that results in a right-to-left shunting. This report describes the case history of a neonate with a large right pulmonary artery?Cto?Cleft atrial connection resulting in cyanosis and severe heart failure who underwent successful early catheter interventional therapy. In the neonate, this lesion can be diagnosed accurately using transthoracic echocardiography. Closure of the communication can be achieved even in infants via percutaneous interventional catheterization with a low procedure-related risk and a good midterm follow-up result.     

Fatal pulmonary arterial hypertension in an infant girl with incontinentia pigmenti

We report the case of an infant girl with incontinentia pigmenti (IP) complicated by fatal pulmonary arterial hypertension (PAH). She was diagnosed with IP, based on the presence of specific skin lesions, neonatal seizures, hypereosinophilia and a maternal family history of IP. At the age of 2 months, she was diagnosed with PAH on systolic heart murmur due to tricuspid valve regurgitation. Despite several treatments for PAH but not including epoprostenol, severe PAH persisted and she died of pulmonary hypertensive crisis at the age of 5 months. On postmortem histopathology the pulmonary artery had severe intimal thickening, with occlusion or stenosis of the vascular lumen of the small pulmonary arteries as well as partial plexiform lesions, all of which were compatible with PAH. Modulation of nuclear factor‐κB signaling may be involved in the development of PAH in IP.     

Combined Treatment for Multiple Cardiac Defects with Interventional Techniques

Multiple congenital cardiac defects are usually addressed by cardiac surgery. We present our experience with simultaneous transcatheter treatment of multiple defects in children. Ten children, six females and four males, with multiple defects underwent treatment with interventional technique. The mean age was 4.4 +/- 2.6 years (range, 7 months to 8 years). The cardiac diagnosis was patent ductus arteriosus (PDA) and valvular pulmonary stenosis (PS) in two, atrial septal defect (ASD) and PDA in two, ASD and PS in two, PDA and aortic stenosis (AS) in three (severe left ventricular dysfunction in two), and perimembranous ventricular septal defect (VSD) and valvular PS in one. The ASDs were closed with an Amplatzer septal occluder (mean size, 16 +/- 4 mm), four PDAs were closed with an Amplatzer duct occluder, and three with a Cook's detectable coil. Mean balloon size used to dilate the pulmonary valve was 18 +/- 4 mm, and for the aortic valve this was 12 +/- 2 mm. There was a 70% (+/-15%) postprocedure reduction of gradients across the stenotic valves. The closure rate was 75% for PDAs in the catheterization laboratory, 80% for ASDs, and there was a mild residual intradevice leak in the VSD. In conclusion, interventional technology addressing multiple congenital cardiac defects as a combined procedure in the catheterization laboratory is safe and effective.     

Clinical Manifestations and Long-Term Follow-Up in Pediatric Patients Living at Altitude With Isolated Pulmonary Artery of Ductal Origin

This study's aim was to define the clinical manifestations and long-term outcome of pediatric patients living at altitude with isolated pulmonary artery (PA) of ductal origin (IPADO). This was a retrospective cohort study of 17 consecutive cases of IPADO at a single center. All patients lived at modest altitude (median 2050 m [range 1700 m to 3050 m]). Fifteen children (88%) were symptomatic at presentation. High-altitude pulmonary edema was present in 2 patients (12%) at diagnosis, and only 1 patient had episodes of hemoptysis during follow-up. Fourteen patients (82%) demonstrated evidence of pulmonary arterial hypertension (PAH). Among 14 patients with PAH, 11 patients had surgical interventions. PAH resolved in 5 of 11 patients (45%) undergoing surgical rehabilitation. One patient died during follow-up, and 7 patients are receiving oral vasodilator therapies due to residual PAH; 14 patients remained asymptomatic. Our study showed that early intervention in patients with IPADO at modest altitude can potentially rehabilitate the isolated PA and reverse PAH. Whether surgery is indicated for patients with this disorder in the absence of PAH is unknown.     

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??Objective??To summarize the clinical characteristics of drug-resistant tuberculosis in children and improve the level of diagnosis and treatment of the disease. Methods??The clinical data of 5 children with drug-resistant tuberculosis treated in Beijing Children’s Hospital from March 2013 to March 2017 were retrospectively analyzed. Clinical data included general information??tuberculosis exposure history??clinical and imaging performance??etiological examination??drug sensitivity test??treatment protocol and outcome. All five patients??4 male and 1 female?? had pulmonary tuberculosis??two had tuberculous meningitis??four had bronchial tuberculosis. Results??All had laboratory-confirmed drug-resistant tuberculosis??two were with rifampicin-resistant tuberculosis??RR-TB?? confirmed by Xpert MTB/RIF??one had confirmed by multidrug-resistant tuberculosis??MDR-TB?? cervical lymph node puncture fluid culture??one had by pre-extensive drug- resistant tuberculosis??Pre-XDR?? sputum culture and the other case was MDR-TB confirmed by the positive culture of his father’s sputum. All patients received regimens recommended by WHO??containing second-line anti-tuberculosis drugs??after diagnosis of drug-resistant tuberculosis. Two children were cured??two children were improved??but still receiving anti-tuberculosis treatment. Treatment failed in one child. All children had no severe adverse events. Conclusion??The drug-resistant tuberculosis in children can be diagnosed by molecular technique Xpert MTB/RIF and mycobacterium tuberculosis culture. The molecular technique is of great value in early and rapid diagnosis of drug-resistant tuberculosis. Most children with drug-resistant tuberculosis have a favourable prognosis after timely and effective treatment. The incidence of serious adverse effects of second-line anti-tuberculosis drugs is low in children.     

Cardiac catheterization in children with pulmonary arterial hypertension

Cardiac catheterization of childhood pulmonary arterial hypertension (PAH) is used to assess the severity of the disease as well as prognosis, selection of the most adequate pulmonary vasodilators, and evaluation of effectiveness. Sudden deterioration of cardiovascular hemodynamics, however, can be easily induced by pain, patient agitation, catheter manipulation, and by vasodilator provocation tests; these could trigger a pulmonary hypertension crisis, vagotony, respiratory distress, and hemoptysis resulting in critical complications, including death. Those patients with New York Heart Association functional class IV are at an especially high risk. It is noteworthy that pulmonary arteriography is a contraindication in patients with PAH. In a review of 7218 adult patients, 76 (1.1%) serious complications, including four deaths, were reported; with regard to the pediatric patients, 29 (10.7%) out of 270 patients with complications, including one with cardiogenic shock requiring cardiopulmonary resuscitation in addition to minor complications, were reported. To prevent serious complications, basic and routine precautions, such as oxygen and concomitant transcutaneous oxygen saturation and electrocardiogram monitoring during transportation to and from the catheter laboratory, are mandatory. Furthermore, the cooperation of experienced physicians and well informed medical staff in addition to meticulous preparation, for example, calculation of prior doses of catecholamine and confirmation of the presence of emergency equipment, is required.