应用自体心包塑型三瓣叶肺动脉瓣的研究

目的总结8例自体心包塑型三瓣叶肺动脉瓣在婴幼儿右室流出道重建术中的应用。方法以白体心包构建三瓣叶肺动脉瓣行右室流出道重建术,并行随访观察。结果本研究8例患儿无围术期死亡病例。平均ICU治疗时间（46±12．5）h,平均呼吸机辅助通气时间（22±6．7）h,术后存在少量瓣膜返流3例,出ICU时SaO2为0．96±0．02。经（22±7．6）个月随访,均无管道钙化征象;2例轻度瓣膜返流,1例中度返流合并瓣膜水平之上狭窄。2年管道无功能障碍率为87．5％。结论自体心包塑型三瓣叶肺动脉瓣在婴幼儿,尤其是管道直径〈15mm的右室流出道重建术中具有良好的中期效果。     

应用国产封堵器治疗室间隔缺损对心脏瓣膜功能的影响

目的 探讨应用国产封堵器进行室间隔缺损(VSD)封堵术对心脏瓣膜功能的影响,并与Amplatzer封堵器、外科手术治疗VSD对心脏瓣膜功能的影响进行对比.方法 将VSD患儿分为三组,国产封堵器组76例,Amplatzer封堵器组34例,外科手术组56例;国产封堵器组分别与Amplatzer封堵器组、外科手术组在术后1周及3个月对三尖瓣关闭不全、三尖瓣狭窄及主动脉瓣关闭不全的发生率进行对比研究.两组间率的比较采用卡方检验.结果 在术后1周,国产封堵器组主动脉瓣关闭不全的发生率较外科手术组高(P<0.05),与Amplatzer封堵器组比较差异无统计学意义(P>0.05).术后3个月,国产封堵器组与外科手术组、Amplatzer封堵器组比较.主动脉瓣关闭不全的发生率差异无统计学意义(P>0.05).术后1周及3个月,国产封堵器组与外科手术组、Amplatzer封堵器组分别比较,三尖瓣关闭不全、三尖瓣狭窄的发生率差异无统计学意义(P>0.05).结论 国产封堵器作为封堵VSD的新器械,在术后及近期对心脏瓣膜功能的影响轻.为避免瓣膜功能受影响,应严格掌握介入术的指征,术中应用心脏超声监测,避免操作动作粗暴,选用合适大小的封堵器.     

Anterior urethral valves without diverticulae: A report of two cases and a review of the literature

Two unusual cases of anterior urethral valves (AUV) without diverticulae are presented. The first case is a male child born with prenatal diagnosis of bilateral hydronephrosis. On cystoscopy, iris‐like diaphragm valves were encountered about 3 mm distal to the skeletal sphincter. In the second case, an 18‐month‐old male child was investigated for recurrent febrile urinary tract infections and obstructed urinary symptoms. Cystoscopy confirmed the presence of slit‐like valves 5 mm distal to the skeletal sphincter. Fulguration of the AUVs was performed in both cases. It may be worthwhile to review all cases of anterior urethral obstruction collectively and re‐categorize them appropriately to include the unusual AUVs without diverticulum in that classification.     

Transcatheter Aortic Valve Implantation and Mitral Valve Repair: State of the Art

Surgical replacement of cardiac valves evolved significantly since its first use. Evolution of techniques and valves used was driven by the frequent complications encountered with surgically implanted valves. Transcatheter implantation of valves is a new technique which continues to be under investigation and promises to perform a task with less risk and potentially better outcome. In addition, new techniques are being developed to address minimizing mitral regurgitation. Progress in transcatheter valve implantation and catheter mitral annuloplasty is reviewed in this article.     

Posterior urethral valves in Senegalese children. Fourteen cases]

Fourteen pediatric cases of posterior urethral valves in patients aged 6 months to 14 years (with four infants and ten older children) are reported. The main symptoms were vesical, including dysuria, acute urinary retention and dribbling. One patient presented with diarrhea, vomiting and dribbling. Urethral valves were looked for as part of the evaluation for urinary lithiasis in one patient, and in another urinary lithiasis developed following the diagnosis of urethral valves. In half the cases, onset of symptoms occurred within one year of birth. Urethrocystography allowed to evidence the valves and to evaluate repercussions on the bladder including hyperplasia (7 cases) and diverticula (2 cases). The intravenous urogram disclosed bilateral ureterohydronephrosis in eight cases. Pathogens recovered from the urine included Klebsiella (3 patients), Pseudomonas (4 patients), Proteus (one patient), and E. coli (one patient). Most patients were treated by catheter lamination under antimicrobial therapy. Two patients died from renal failure. Results were considered satisfactory in nine cases with follow-ups ranging from 1 month to 4 years. Three patients were lost to follow-up. Early diagnosis is essential and management should rely mainly on endoscopic resection which reduces the length of the hospital stay and the risk of infection.     

Anterior urethral valves in children: an uncommon multipathogenic cause of obstructive uropathy

Introduction  Anterior urethral valves (AUVs) are uncommon congenital anomalies causing urethral obstruction in boys. Patients and methods  Medical records of 13 children were reviewed retrospectively. Each patient (pt) was evaluated with voiding cysto-urethrogram (VCUG) and renal ultrasonography. Pts older than 5 years performed an uroflowmetry. Serum creatine was determined in all children. Results  All pts had difficulty in voiding and eight had recurrent urinary tract infection. Renal function was normal in all pts. VCUG showed filling of Cowper’s duct in a pt. In a case, a vesicoureteral reflux was discovered. Uroflowmetry showed a flat trace in all cases. Transurethral endoscopic resection of the valves was carried out in 11 children while open resection was necessary in two children. At serial follow-up, all pts remain symptom-free and uroflowmetry documented regular pattern. Discussion  Anterior urethral valves are rare lesions that may create problems at different stages. Some AUVs may result from the obstruction distal lip of a ruptured syringocele. Improvements in endoscopic equipment allow for minimally invasive transurethral resection. Large diverticula are best managed with open diverticulectomy and reconstruction. In newborns with severe altered urinary tract drainage, particularly in low birth-weight infant, a vesicostomy may be necessary.     

The Doppler echocardiographic assessment of valvular regurgitation in normal children

To determine the frequency of valvular regurgitation, 174 children from birth to 18 years old (mean age 7.42+/-4.54 years), with structurally normal hearts were analyzed. Flow patterns across the four valves were examined by pulsed- (PW), continuous-wave (CW) and color Doppler. Regurgitation was detected in 59.7% of the valves. Tricuspid regurgitation (TR) was most commonly found in 32.8% (n=57), pulmonary regurgitation (PR) in 17.2% (n=30), mitral regurgitation (MR) in 8.6% (n=15), and aortic regurgitation (AR) in 1.1% (n=2). The range of peak velocities of the regurgitant flow with CW were as follows: TR:0.98-2.54 m/sec, PR:0.50-1.80 m/sec, MR:0.72-2.30 m/sec, and AR:1.22-1.25 m/sec. The mean peak velocities of the regurgitant flow were similar with CW and PW measurements (p>0.05). The length of the regurgitant flow with color Doppler was less than 1 cm beyond the mitral and aortic valves. It reached up to 1.8 cm behind the pulmonary and 1.83 cm behind the tricuspid valves. Frequency of valvular regurgitation was unchanged with age (p>0.05). Tricuspid regurgitation was more frequent in males (40.9% versus 23.5%; p=0.016). Regurgitation of tricuspid, pulmonary or mitral valves is relatively common in children with structurally normal hearts. Aortic regurgitation is scarce and the possible pathologic cause should always be carefully sought.     

Posterior urethral valves and testicular maldescent: An underreported association?

It is a generally held belief that posterior urethral valves (PUV) rarely coexist with anomalies other than those involving the urinary tract despite a report by Krueger et al. in 1980 of a significant association between cryptorchidism and posterior urethral valves. We reviewed 46 cases of PUV seen at our institution since 1959; 8 of these had undescended testes. Three of 4 patients with bilateral maldescent had associated syndromes or prematurity. In 5 cases (12%), there was no immediate explanation for the significantly increased incidence of maldescent (P <0.001). The presence of a large bladder or inguinoscrotal compression due to oligohydramnios may play a role. Correspondence to: P. A. King     

Agenesia of the pulmonary valves manifested by ventilation disorders. In the newborn]

Agenesia of the pulmonary valves is often responsible for severe disorders of ventilation by bronchial compression in newborns. This may be involved in case of respiratory distress, with a double murmur, expressing the stenosis of an avalvulated pulmonary ring and the massive pulmonary regurgitation. The bad tolerance is due to the association with an interventricular dilatation of the pulmonary artery, which characterizes this malformative complex. The prognosis is severe and the treatment is disappointing since all surgical attempts failed at this age.     

Anterior urethral valves: an uncommon cause of obstructive uropathy in children

PurposeAnterior urethral valves (AUV) are rare entities generally described in case reports. They are an uncommon cause of lower urinary tract obstruction in children and can be difficult to diagnose. In the present study, we present our experience in four children with AUV along with a literature review.Materials and methodsWe retrospectively identified four children with AUV presented between 1998 and 2005 at age 4–9 years.ResultsHematuria, urinary tract infection and weak voiding stream were the most common symptoms. Voiding cystourethrography (VCUG) confirmed the diagnosis of AUV. On cystourethroscopy, cusp-like valves in the anterior urethra were seen in all children. Transurethral endoscopic resection of the valves was carried out in three children using a pediatric resectoscope. In one child with a massive anterior urethral diverticulum, open resection of the valve, diverticulectomy and urethroplasty were performed. All patients were cured, none had complications as a result of surgery, and all reported a normal urinary stream at follow-up.ConclusionsChildren with poor stream and recurrent infections should be evaluated carefully and anterior urethral valves should be considered in differential diagnosis of obstructive lesions.     

后尿道瓣膜症

目的探讨后尿道瓣膜对上、下尿路的损害及治疗。方法回顾分析158例后尿道瓣膜病例。年龄14d～15岁，〈1岁59例（37．3％），平均3岁3个月。手术除经尿道内切开瓣膜外，曾做膀胱造口20例，输尿管皮肤造口5例以及睾丸固定3例。结果108例（68．4％）随访3个月～17年，平均2．5g。正常排尿54例，经常滴尿16例。因VUR或输尿管膀胱交界部梗阻需输尿管再植16例，肾切除5侧，肾造瘘4例，膀胱造瘘1例。死亡6例，生长发育落后2例。1例因并发前尿道瓣膜，2例尿道狭窄再次经尿道内切开，1例尿道闭锁做尿道吻合。结论后尿道瓣膜是男儿最常见的先天性下尿路梗阻性疾病，可致严重的上、下尿路损害，需尽早引流尿路，控制感染。减少肾功能损害。1岁以下婴儿如无合适切除镜时应先做膀胱造口。对排尿功能不良患儿应做尿动力学检查，以确定治疗方案。     

Two-Dimensional Echocardiographic Valve Measurements in Healthy Children: Gender-Specific Differences

The goal of this study was to create nomograms of echocardiographic two-dimensional valve dimensions based on a large group of children without heart disease. Children aged 0–18 years underwent standard echocardiographic evaluation. Referring diagnoses were chest pain, heart murmur, or syncope. Only patients with a structurally normal heart and normal systolic and diastolic function were included. All four valves were measured at their maximal dimensions. A total of 748 children (314 girls and 434 boys) met the inclusion criteria. Mean values and standard deviations were calculated, and z value nomograms based on body surface area were developed. Surprisingly, the boys had larger valve dimensions at all ages. These valve dimension differences were statistically significant for three of four valves even after adjustment for the differences in body sizes. The difference may be due to higher circulating blood volume in boys compared to that in girls. Because the differences are subtle, they reach statistical significance only when evaluated in a large group of subjects. Presented normal value data will be helpful in following cardiology patients and evaluating intervention strategy in patients with valve hypoplasia. An erratum to this article is available at .     

双向格林手术后房室瓣反流加重的原因分析

目的 探讨双向格林手术后房室瓣反流加重的原因.方法 2009年至2014年共523例患儿实施单纯的双向格林手术,其中术前存在左侧(体循环房室瓣)或共同房室瓣反流者420例,按原发畸形的种类及术前对房室瓣的描述加以登记.大多数患儿采用非体外循环下实施双向格林手术.术后复查心脏彩超评估房室瓣的反流变化.结果 术后体循环房室瓣反流改善的391例,术后体循环房室瓣反流无改善的18例,术后体循环房室瓣反流量增加的11例,其中死亡2例.术后体循环房室瓣反流少量及以下者,平均呼吸机辅助时间(6.3±2.4) h,术后房室瓣反流无改善或反流加重的患儿,平均呼吸机辅助时间(30.6±4.8)h.体循环房室瓣反流改善组患儿的原发畸形均为单心室类,体循环房室瓣反流于术后无改善者中8例为单心室畸形,10例为双心室类畸形,体循环房室瓣反流于术后加重者均为双心室类畸形.结论 双向格林术后原有的体循环房室瓣反流增加主要发生在双心室类畸形的患儿,术前房室瓣结构明显发育异常也是术后反流无改善的重要因素.     

儿童心脏瓣膜置换术

目的儿童心脏瓣膜疾病的外科处理，原则上应尽可能的采用修复技术进行治疗，但对瓣膜病变严重的患儿，则需行瓣膜置换术。本文旨在探讨儿童心脏瓣膜置换术的手术适应证，人工瓣膜的选择以及瓣膜置换术后长期抗凝等问题。方法总结43例16岁以下儿童瓣膜替换术的经验。结果全组长期存活41例，死亡2例，存活的41例中，除1例患儿发生右心室室壁瘤外，其余患儿心功能均恢复良好，无一例因生长发育出现心脏人工瓣膜相对狭窄及因抗凝引起的出血或血栓并发症。结论儿童人工心脏瓣膜置换术在慎重掌握手术适应证的基础上，有较好的临床效果。儿童心脏瓣膜置换应选择人工机械瓣膜。儿童瓣膜置换术后的抗凝需要经常定期地监测各项抗凝指标并随时做出适当的调整。     

Tetralogy of Fallot with Congenital Aortic Valvar Stenosis: The Tetralogy–Truncus Interrelationship

Two rare patients are reported with tetralogy of Fallot and congenital aortic valvar stenosis. The anatomic and developmental interrelationship between tetralogy of Fallot and truncus arteriosus is summarized. A study of 100 randomly selected postmortem cases of tetralogy revealed aortic valve pathology in 8%, myxomatous aortic valve leaflets without stenosis in 4%, bicuspid aortic valves without stenosis in 3%, and congenital aortic valvar stenosis in 1%. The frequency of systemic semilunar valve pathology in truncus was much higher (66%): moderate to marked myxomatous change in 44%, mild myxomatous change in 22%, truncal valvar stenosis in 11%, and truncal valvar regurgitation in 15%. Being aware of the tetralogy–truncus interrelationship and knowing that myxomatous aortic valves are prone to premature calcific aortic stenosis and/or regurgitation, physicians should follow the aortic valves of surgically repaired patients with tetralogy of Fallot and truncus arteriosus long term with great care. Timely aortic valvuloplasty or replacement may well prove life-saving in such patients.     

新生儿后尿道瓣膜19例诊治分析

目的 探讨新生儿后尿道瓣膜(posterior urethral valve,PUV)的早期诊断、治疗和疗效评估.方法 收集2009年2月至2015年10月我院收治的19例新生儿PUV患儿的临床资料,回顾性分析其诊断、治疗及疗效.术前行B型超声、排泄性膀胱尿道造影(voiding cystourethrography,VCUG)和CT尿路造影检查,所有患儿均行一期经尿道内瓣膜切开术(transurethral resection,TUR).术后随访排尿情况,行VCUG、B型超声和尿流动力学检查,评价泌尿系统结构和功能恢复情况.结果 1例术后因酸中毒、呼吸衰竭死亡,2例失访,16例获术后随访.随访6个月～6.5年,平均3.2年.6例患儿因瓣膜残留再次行CT尿路造影,术后有1例发生尿道狭窄,予多次扩张尿道后缓解.正常排尿12例,其中5例可见夜间遗尿;偶有湿裤2例,尿频2例.19侧膀胱输尿管反流(vesieoureteral reflux,VUR)中10侧完全消失,6侧减轻,因严重反流不能缓解需行输尿管再植2例.术前有12例膀胱评分为严重(5或6).16例患儿最后1次VCUG检查示,膀胱评分均改善至轻度.B型超声检查示肾积水、输尿管扩张缓解10例,6例减轻.12例患儿行尿流动力学检查,平均检查年龄为3.3岁;平均安全膀胱容量为(115±19)ml,与年龄匹配;8例表现为低顺应性;10例出现逼尿肌不稳定,并给予索利那新抑制膀胱过度活动,口服3个月后复查尿流动力学,7例逼尿肌不稳定获得改善.结论 新生儿期PUV改善一般情况后行尿道镜下瓣膜切除安全有效,部分患儿需二次切除.     

Anterior urethral valves

Posterior urethral valves (PUV) are the most frequent cause of obstructive uropathy in boys. Rarer causes of obstructive uropathy include bladder diverticulae, meatal stenosis and urethral or bladder stones. Anterior urethral valves are rare causes of urinary obstruction in boys and are ten times less frequent than PUV in the literature(1-6). This paper highlights our experience with 5 patients with anterior urethral valves.     

Hormonal analysis in post-pubertal patients with posterior urethral valves.

AIMS: Infertility in patients with posterior urethral valves (PUV) is known but the exact incidence and etiology is still speculative; it may be partly due to dysfunction of genital organs. The aim of this study is to analyse the hormonal profile of post-pubertal patients with PUV. MATERIALS AND METHODS: All post-pubertal patients with posterior urethral valves (age > 16 years) visiting the follow-up urology clinic of our department from 1985 to December 1999 were contacted and requested to participate in the study; 9 patients agreed and form the study group. The medical records were reviewed. Physical examination included examination of the genitalia and secondary sexual characteristics. Blood samples were taken for estimation of lutenizing hormone (LH), follicle-stimulating hormone (FSH), prolactin and testosterone. RESULTS: Ages ranged from 16 - 21 years (mean age = 17.5 years). Physical examination was normal for the entire study group except for 2 patients; one patient had hypertension and the other had unilateral undescended testis at the time of initial presentation. Secondary sexual characteristics were normal in all patients. At the time of the study, one patient had chronic renal failure; in the others the renal function tests were normal. LH and testosterone were normal in all patients including the patient with unilateral undescended testis, and one patient had marginally elevated FSH. Hyperprolactinemia was noted in 5 of 9 patients. CONCLUSION: In the present study, hyperprolactinemia was seen in 62 % (5 of 9) patients. Hyperprolactinemia is known to cause slow ejaculation and can have an impact on the subsequent fertility status of these patients. The bearing of these findings on subsequent sexual and fertility functions of PUV patients requires further investigation.     

Midterm Results of the Ross Procedure in a Pediatric Population: Bicuspid Aortic Valve Is Not a Contraindication

Our objective was to analyze retrospectively the short- and midterm results of the Ross operation in children and the impact of bicuspid aortic valve (BAV) disease on outcome. From 1991 to 2003, 41 patients (26 male, 15 female) underwent a Ross procedure. Aortic disease was congenital in all but one. Sixty-six percent had BAV. Mean age at operation was 10.13 ± 5.6 years (range, 0.4–18.3 years). Root replacement technique was performed in all but two (inclusion technique). There were two early deaths. Mean follow-up was 6 ± 3.8 years (range, 0.1–14 years). The autograft (neo-aorta) presented absent, trivial, mild, and moderate regurgitation in 42%, 46%, 10%, and 2%, respectively, at latest follow-up. Root dilation was seen in 64% of the patients (mean Z-score, +3.53 ± 0.04). Four patients (9%) required allograft replacement, two for endocarditis and two for stenosis. Allograft stenosis (gradient >20 mmHg) was detected in 44% of the remaining patients, without symptoms or the need for reintervention. Estimated freedom from allograft replacement at 5 and 10 years was 97% and 89%. Left ventricular dimensions and function were normal in all patients. No difference was found between patients with BAV and those with tricuspid aortic valves in aortic regurgitation or root dilation. BAV was not identified as a risk factor for root dilation. In conclusion, the Ross operation remains an excellent option for aortic valve replacement in children. BAV is not associated with a worse outcome and is not a risk factor for aortic root dilation at medium-term follow-up. Long-term follow-up is, however, required.     

Anterior urethral valves—a rare cause of urethral obstruction

Anterior urethral valves is a very rare congenital anomaly of the anterior urethra. It produces symptoms of varied intensity with the most severe ones leading to Urosepsis and renal failure(similar to as in posterior urethral valves) to the milder ones which may not produce any obstruction at all. However unlike the Posterior urethral valves this anomaly has milder and reversible sequelae in majority of the cases. The clinical presentation is mainly in the form of obstructed stream of urine and UTI and the diagnosis depends essentially on Micturating Cystographic studies and on the awareness and a high level of suspicion of its existence among Paediatricians and General Practitioners.