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find Keyword "先天性" 519 results
  • Open heart operation on neonates with critical congenital heart disease 推荐 CAJ下载 PDF

    Objective To summarize the experience of open heart operation on neonates with critical and complex congenital heart diseases and evaluate the methods of perioperative management. Methods From May 2001 to January 2003, 12 patients of neonates with congenital heart diseases underwent emergency operation. Their operating ages ranged from 6 to 30 days, the body weights were 2.8 to 4.5 kg. Their diagnoses included D-transposition of the great arteries in 4 cases, ventricular septal defect with atrial septal defect in 5 cases, complete atrioventricular septal defect, obstructed supracardiac total anomalous pulmonary venous drainage and cardiac rhabdomyomas in 1 case respectively. 12 cases were operated under moderate or deep hypothermic cardiopulmonary bypass. Results All cases were observed in ICU for 2-11 days and discharged 7-19 days after operation. The postoperative complications included low cardiac output, mediastinal infection, respiratory distress syndrome, systemic capillary leak syndrome and acute renal failure. All cases were cured and the follow-up (from 6 months to 2 years) showed satisfactory outcome. Conclusion A particular cardiopulmonary bypass and proper perioperative management is very important to ensure the successful outcome. Peritoneal dialysis is an effective and safe method for treating acute renal failure after cardiac operation in neonates.

    Release date:2016-08-30 06:27 Export PDF Favorites Scan
  • ANATOMICAL FEATURES OF CONGENITAL CHORDEE WITHOUT HYPOSPADIAS IN CHILDREN AND IMPLICATION FOR DIAGNOSIS AND TREATMENT

    Objective To investigate the anatomical features of congenital chordee without hypospadias in children and to discuss the diagnosis and treatment.Methods From August 1984 to December 2004, 94 children with chordee withouthypospadias treated in the West China Hospital of Sichuan University were classified and analyzed for anatomical alterations. Their ages ranged from 18 months to 13 years (mean 6.9 years). Ninety-four patients were divided into four groups. With intraoperation artificial erection, the patients with penis straightened after degloving were classified as type Ⅰ patients (skin-tethering), those with peins straightened after fibrotic tissue in Buck’s fascia released as type Ⅱ patients (dysgenetic fascia), those with normal urethra and orthoplasty failed after degloving and removing fibrotic tissue as type Ⅲ patients (corporal disproportion), and those with dysgenetic urethra tethering the corpora cavernosa as type Ⅳ patients (short urethra).Results In type Ⅰ (n=31, 32.9%) patients, the ventral skin and dartos fascia were contracted while Buck’s fascia and the urethra was normal, in some (7 cases) scrotal skin extended to the ventral portion of penis (webbed penis). In type Ⅱ (n=45, 47.9%), contracture of Buck’s fascia was evident and the thickening fibrotic tissue constituted the chief obstacle to orthoplasty, though in some skin was shortened. In type Ⅲ (n=6), the dorsal and ventral sides of the corpora cavernosa were disproportionated, and the morphologically normal urethra tightly adhered to the ventral aspect of corpora cavernosa. In some cases ventral skin and fascia were contracted, but orthoplasty could notbe achieved through releasing these layers. In type Ⅳ (n=12, 12.8%), the distal urethra was paper-thin and lacking corpus spongiosum, or dense fibrotic bandswere found to be deep to the urethra. The urethra tethered the corpora cavernosaand formed a bow-to-string relation. The overlying skin and fascia were contracted in varying degrees while none had significance in straightening the penis. After operation, the length of penis increased to 6.9 cm from 5.2 cm on average and the chordee was corrected to 1.6° from 42.6° before operation on average. The patients were followed up 1 months to 15 years. The results were satisfactory.Chordee remained in 2 cases, fistula and urethral stricture occurred in 2 casesrespectively, fistula in association with urethral stricture and diverticulum in 1 case; the operation was given again and the results was satisfactory. Conclusion Patients with chordee without hypospadias may be divided into four types depending on which layer of the ventral penis constitutes thechief contribution to chordee. A systematic approach with repeated artificial erection tests is needed in determining the classification and surgical correction. 

    Release date:2016-09-01 09:25 Export PDF Favorites Scan
  • 上腔静脉右肺动脉分流术治疗三尖瓣闭锁

    目的 总结上腔静脉右肺动脉分流术治疗三尖瓣闭锁(TA)的临床经验。 方法 2004年10月至2008年4月,采用上腔静脉右肺动脉分流术治疗TA 10例,男8例,女2例 ;年龄2~13岁,平均年龄5.6岁;体重10.0~33.5 kg,平均体重16.4 kg。TA合并大动脉错位(右位心)2例,房室间隔缺损3例,永存左上腔静脉1例。均在常温非体外循环下手术,切断上腔静脉,近端缝合,远端与右肺动脉做端侧吻合;1例合并永存左上腔静脉患者行双侧上腔静脉肺动脉吻合术。 结果 全组无死亡患者,术后末梢血氧饱和度由74%±9%上升至92%±5%。所有患者紫绀明显减轻,顺利出院。随访10例,分别随访6~36个月,血氧饱和度86%±3%,活动能力明显改善。 结论 上腔静脉右肺动脉分流术治疗TA效果满意,是比较理想的术式。

    Release date:2016-08-30 05:59 Export PDF Favorites Scan
  • 十字交叉心的外科治疗

    目的 总结4例十字交叉心外科治疗的临床经验,以提高对该病的认识。 方法 2004年3月~2008年1月手术治疗4例十字交叉心患者(平均年龄3.2岁;平均体重10.9 kg)。1例采用心内隧道修补术(intravetricular tunnel rapair IVR),1例采用双向Glenn手术,1例采用双侧双向Glenn术,1例采用IVR+大动脉转位术(arterial switch operation, ASO)。 结果 住院死亡1例,为采用IVR者,死于低心排血量综合征。2例行双向Glenn手术患者术后随访6~12个月,生长发育良好,SpO2 88%左右;1例行IVR+ ASO者,随访6个月,心脏较术前略缩小,生长发育良好。结论 十字交叉心病理解剖复杂,必须根据不同的解剖特点选择不同的手术方法,明确的术前诊断是优化手术方式的先决条件。

    Release date:2016-08-30 06:09 Export PDF Favorites Scan
  • THE LONG TERM RESULTS OF BILIARY RECONSTRUCTION IN CHILDREN

    Twenty five children with congenital biliary dilatation were treated with hepatico-jejuno-duodenostomy following excision of choledochal cysts between 1983 and 1985. The age ranged from two months to eleven years. The last follow-up ranged from 6-9 years (mean 7.5 years). All patients were free of jaundice with normal growth and development and none had peptic ulcer. The results from the last follow-up was better than that of the first one. This procedure was safe, effective and physiologically appealing.

    Release date:2016-09-01 11:38 Export PDF Favorites Scan
  • 婴幼儿体外循环术后机械通气模式的选择

    目的比较先天性心脏病婴幼儿体外循环术后容量控制通气(VCV)、压力控制通气(PCV)和压力调节容量控制通气(PRVC)3种呼吸模式的治疗效果。方法将2003年10月到2005年5月收治的106例婴幼儿先天性心脏病(CHD)患者分为3组,组Ⅰ(42例)为一般CHD患者,组Ⅱ(40例)为复杂CHD患者,组Ⅲ(24例)为伴有肺动脉高压(PH)的CHD患者。根据随机原则选择VCV、PCV、PRVC3种呼吸模式进行支持治疗。记录血流动力学、呼吸力学和血气分析指标并进行统计分析。结果组Ⅱ和组Ⅲ患者PRVC模式可以明显改善血气和降低气道压力,同时对血流动力学无明显影响;3种呼吸模式对组Ⅰ患者差异无统计学意义。结论婴幼儿体外循环术后3种呼吸模式对一般CHD患者无明显差异,对复杂CHD和伴有PH的CHD患者PRVC模式在呼吸力学和血气分析方面优于VCV和PCV模式。

    Release date:2016-08-30 06:23 Export PDF Favorites Scan
  • Sex hormone levels have little impact on outcomes of cardiac surgery in fertile women with congenital heart disease: A retrospective cohort study

    Objective To examine the influence of hormonal fluctuations on the perioperative outcomes of patients undergoing congenital heart surgery. Methods We conducted a retrospective analysis of clinical data from fertile women diagnosed with congenital heart disease at the Guangdong Provincial People's Hospital, between January 1, 2015, and July 30, 2019. Initially, patients were categorized into groups based on serum progesterone levels: a low progesterone group (n=31) and a high progesterone group (n=153). Furthermore, based on serum estrogen levels, they were divided into a low estrogen group (n=10), a medium estrogen group (n=32), and a high estrogen group (n=118) for comparative analysis. A control group (n=24) consisted of patients who received progesterone injections before their menstrual period. Results We finally included 184 patients. The patients’ average age was 27.6±5.7 years, with 142 (77.17%) presenting with complex congenital heart conditions. There were statistically significant differences in total postoperative standard thoracic drainage volume and postoperative albumin level between the high and low progesterone groups (P<0.05), while other perioperative outcome indicators showed no statistical differences (P>0.05). Among the different serum estrogen level groups, there were statistically significant differences in postoperative blood urea nitrogen levels, total postoperative standard thoracic drainage volume, and hospital stay (P<0.05), while other perioperative outcome indicators showed no statistical differences (P>0.05). ConclusionConsidering the overall clinical significance, the physiological changes in sex hormone levels appear to have a negligible effect on the perioperative outcomes of fertile women with congenital heart disease.

    Release date:2024-05-28 03:37 Export PDF Favorites Scan
  • 先天性冠状动脉瘘外科治疗

    目的总结外科手术矫治先天性冠状动脉瘘的临床结果及相关经验。方法回顾性分析 2010 年 1 月至 2019 年 6 月就诊于泰达国际心血管病医院的 25 例先天性冠状动脉瘘患者的临床资料,其中男 17 例、女 8 例,年龄 5 个月至 53 岁,中位年龄 5 岁。其中合并房间隔缺损 1 例,合并动脉粥样硬化 1 例。手术方式:直接缝闭瘘口 16 例,心包补片修补瘘口 9 例。1 例患者非体外循环下手术矫治;其余 24 例均在体外循环下行手术矫治,其中 2 例心脏未停跳下外科矫治。结果所有患者顺利出院,体外循环时间(59.75±22.75)min,主动脉阻断时间(34.55±13.22)min。术中探查发现均为单一瘘口。右冠状动脉-左室瘘 2 例(8.0%)、右冠状动脉-右房瘘 3 例(12.0%)、右冠状动脉-右室瘘 10 例(40.0%)、左冠状动脉-右室瘘 4 例(16.0%)、左冠状动脉-右房瘘 2 例(8.0%)、左冠状动脉-肺动脉瘘 4 例(16.0%),其中 1 例为罕见的窦房结动脉-右房瘘,窦房结动脉起自右冠状动脉。患者术后均给予阿司匹林抗血小板治疗 3 个月,其中 2 例随访中发现瘘口残端血栓形成。所有病例已扩张冠状动脉,未恢复正常内径。随访 1 个月至 3 年,无患者死亡和心肌缺血及残余瘘发生。结论有症状或者分流量明显的先天性冠状动脉瘘,应尽早手术矫治,术中根据解剖特点确定手术方式,外科手术结果满意。

    Release date:2020-12-31 03:27 Export PDF Favorites Scan
  • 完全性房室间隔缺损合并法洛四联症外科矫治的单中心临床经验

    目的分析单个临床中心完全性房室间隔缺损合并法洛四联症的外科治疗经验。方法回顾性分析广东省人民医院心外科 2008 年 5 月至 2017 年 3 月收治的 21 例经超声心动图诊断为完全性房室间隔缺损合并法洛四联症患者的外科矫治经验,其中男 17 例、女 4 例,年龄 10 个月(1 个月~20 岁),体重 10(2.5~68)kg。分析其临床结局,并随访生存病例,以初步了解其预后。结果住 ICU 时间 4(1~23)d,住院时间 12(6~21)d。死亡 3 例。21 例患者中,双心室矫治 6 例,单心室矫治 15 例。平均随访时间 3 年,5 年生存率 80%。结论完全性房室间隔缺损合并法洛四联症外科解剖矫治困难,多数病例仅可施行单心室矫治手术,部分病例可施行双心室矫治手术,远期预后良好。

    Release date:2019-07-17 04:28 Export PDF Favorites Scan
  • Chinese expert consensus on surgical treatment of aortic valve disease in children

    The consensus was authored by National Society of Congenital Heart Diseases. After employing the Delphi process and incorporating literature reviews and expert discussions, seven recommendations were ultimately formulated. The consensus provides a detailed elaboration on the pathoanatomy, pathophysiology, clinical manifestations, diagnostic methods, and surgical treatment approaches for aortic valve diseases in children. It emphasizes that the treatment of aortic valve diseases in children should take into account the needs of growth and development, and recommends surgical strategies for different age groups and types of lesions, including valve plasty, Ross procedure, valve replacement, and balloon dilation. Specifically, aortic valve plasty is recommended for neonates and infants, while surgical options for older children are more diversified. The consensus only discusses isolated aortic valve disease and does not cover cases complicated with other heart malformations.

    Release date:2024-11-27 02:45 Export PDF Favorites Scan
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