儿童机械瓣膜置换术121例的疗效

目的探讨儿童机械瓣膜置换术的疗效。方法14岁以下病童施行心脏机械瓣膜置换术121例,先天性心瓣膜病89例,风湿性心瓣膜病29例,心内膜炎24例(其中21例为先天性或风湿性心脏病患儿)。行二尖瓣置换62例,主动脉瓣置换49例,三尖瓣置换6例,双瓣置换4例。共置换机械瓣膜125枚。术后患儿均接受华法林抗凝。术后随访通过问卷或门诊检查完成。结果住院死亡15例,病死率12．4％(15／121);失随访32例。74例平均随访时间10．5年,晚期死亡1例,再手术2例。大部分存活病人心功能(纽约心脏协会分级)Ⅰ级-Ⅱ级。结论大多数儿童可以植入较大规格的机械瓣膜,保证远期生长发育需要。儿童机械瓣膜置换住院病死率高,合理的抗凝治疗可能提高远期疗效。     

儿童瓣膜置换术

对11例4～14岁的瓣膜病变患儿进行儿童瓣膜置换术，其中4例合并有其他心脏畸形予以同期修复，无住院死亡。晚期死亡1例，存活者心功能Ⅰ～Ⅱ级。认为：小儿应首选机械瓣；抗凝治疗是术后的主要问题；对左侧位（主动脉瓣、二尖瓣）瓣膜术后应用小剂量抗凝剂在凝血酶原时间标准化比值监测下是安全的；儿童应尽可能选用符合成人需要型号的瓣膜。     

Emergency valve replacement in rheumatic heart disease

BACKGROUND AND AIM OF THE STUDY: Emergency valve replacement is defined as surgery undertaken to correct valvular heart disease which otherwise would have proved fatal within 24 h. METHODS: Among 1,742 patients who underwent valve replacement during the past 15 years at the authors' institution, 61 who had emergency surgery formed two groups. Group 1 (n = 24) had a previous good circulatory status but suffered a sudden change in valvular function, in the initial period after closed mitral valvotomy (CMV), and in later years after balloon aortic valvotomy (BAV) or balloon mitral valvotomy (BMV) for aortic or mitral valvular stenosis. Group 2 (n = 37) had chronic decompensated rheumatic valvular disease and acute low cardiac output. RESULTS: Four deaths occurred in group 1 (17%) during the early stage of the study, but no deaths have occurred during the past five years. Mean follow up was 13.5 months (range: 5-30 months). Among 20 survivors, 14 were in NYHA class I, and six in class II. Ten deaths occurred in group 2 (27%), including two during the past eight years among patients who had surgery. Mean follow up was 37.7 months (range: 8-96 months). Among 27 survivors, 11 were in NYHA class I, 12 in class II, two in class III and two in class IV. CONCLUSION: In this potential terminally ill group of patients, surgery offers the only option for survival.     

Mechanical aortic valve replacement: long-term outcomes in children

BACKGROUND AND AIM OF THE STUDY: Early and late outcomes following mechanical aortic valve replacement (AVR) in children were analyzed. METHODS: Between January 1980 and December 2003, 55 patients underwent mechanical AVR at the authors' institution. Of these patients, 12 had aortic regurgitation (AR), 31 had aortic stenosis (AS), and 12 had mixed disease. Preoperatively, 25 patients (45.5%) were in NYHA classes III and IV. Among patients, 22 had a previous valvotomy and 19 had associated cardiac defects. Isolated AVR was performed in 37 cases. Twelve patients required root enlargement procedures, two had aortic root replacement, three had a double valve replacement, and one patient had a concurrent mitral annuloplasty. The mean prosthesis size was 22.6 mm (range: 16-31 mm). Mean follow up was 12.28 +/- 6 years (range: 1-23 years); total follow up was 665 patient-years (pt-yr). RESULTS: There was one late death. Actuarial survival at 20 years was 98 +/- 2%. Event-free survival at one, five and 20 years was 96 +/- 3%, 92 +/- 4% and 88 +/- 5%, respectively. Four patients required reoperation (two for valve outgrowth, one each for paravalvular leak and prosthetic valve endocarditis (PVE)). Freedom from reintervention at one, five and 20 years was 98 +/- 2%, 96 +/- 3% and 92 +/- 4%, respectively. There was one event of anticoagulation-related hemorrhage. Freedom from anticoagulant-related hemorrhage at 20 years was 98 +/- 2%, and freedom from PVE at five and 20 years was 98 +/- 2% and 96 +/- 3%, respectively. There were no instances of thromboembolism or structural valve dysfunction. Linearized rates of bleeding and endocarditis were 0.15 and 0.3% per pt-yr, respectively. At follow up, 54 children were in NYHA classes I or II. CONCLUSION: Mechanical AVR, with aortic root enlargement if necessary, is associated with low mortality and morbidity, and is an excellent treatment option in children. Late embolic and hemorrhagic complications are infrequent in the current era.     

Mitral valve repair with aortic valve replacement in rheumatic heart disease

From 1992 to 2001, 609 patients with rheumatic heart disease underwent aortic valve replacement with either mitral valve repair (n = 201) or mitral valve replacement (n = 408). Follow-up extended to 10 years. Thirty-day mortality was 1.4% for mitral valve repair and 0.7% for mitral valve replacement (p = 0.4). Survival at 9 years was 96.5 +/- 1.4% after mitral valve repair and 89.7 +/- 7.8% after mitral valve replacement (p = 0.73). Freedom from major bleeding at 9 years was 94.8 +/- 2.4% after mitral valve repair and 81 +/- 7.2% after mitral valve replacement (p = 0.03). Freedom from other valve-related complications and from mitral valve re-operation was similar for the two groups. This study showed that in patients with rheumatic heart disease the results of mitral valve repair with aortic valve replacement were comparable to those of double valve replacement. Major bleeding was less frequent after mitral valve repair with aortic valve replacement. Therefore, whenever feasible, mitral valve repair should be attempted in patients with rheumatic heart disease who need concomitant aortic valve replacement.     

Mechanical aortic and mitral valve replacement in infants and children

BACKGROUND: The aim of this study was to evaluate early and late outcomes after mechanical systemic heart valve replacement in pediatric patients. METHODS: Between October 1981 and December 2003, 32 children (mean age 7.2 +/- 5.4 years; 4 months - 15.9 years) underwent mechanical mitral (MVR, n = 17), aortic (AVR, n = 13) or double valve replacement (DVR, n = 2) with St. Jude Medical valves. Twenty-two patients (69 %) had undergone previous cardiac surgery. Anticoagulation self-management was used since 1995. RESULTS: The operative mortality was 3.1 %. Perioperative complications were complete heart block (n = 5), ventricular fibrillation (n = 1) and myocardial infarction (n = 1) and were exclusively related to patients with MVR. Mean calculated valve size ratio (geometric prosthesis orifice area/normal valve size area) was 1.72 (1.07 - 2.85) for AVR and 1.4 (0.88 - 3.12) for MVR. Mean follow-up was 9.1 +/- 6.6 years (range 0.4 - 23.2 years, cumulative 283 patient-years). There were two late deaths in patients with MVR. Actuarial survival after 10 years was 93.8 %. Late complications were endocarditis (n = 2), minor hemorrhagic event (n = 1), and stroke (n = 1). Anticoagulation self-management is well accepted by all patients/parents. Overall 10-year freedom from any anticoagulation-related adverse event with phenprocoumon was 89.1 % (1.2 %/patient year). Nine patients required reoperations: redo-MVR (outgrowth of prostheses (n = 3), pannus overgrowth (n = 2), closure of paravalvular leak after AVR (n = 2), partial aortic valve thrombosis (n = 1) and redo-DVR (n = 1 for endocarditis). Freedom from reoperation after 10 years was 80.9 %. CONCLUSIONS: Mechanical valve prostheses are a valuable option for left-sided heart valve replacement in pediatric patients. Perioperative morbidity was exclusively related to patients with MVR. Oversizing was often possible to avoid early reoperation for outgrowth. The operative mortality and long-term morbidity are acceptable. Anticoagulation self-management is safe and well accepted.     

Prosthetic valve replacement in adolescents with rheumatic heart disease

To assess long-term survival and anticoagulant-related complications after mechanical valve replacement in adolescents with rheumatic heart disease, 88 patients aged 相似文献   

儿童主动脉瓣置换术临床特点及中远期随访结果

目的 总结15岁以下儿童主动脉瓣置换术的临床特点及中远期随访结果,探讨手术指征、瓣膜选择等相关问题.方法 回顾性分析2006年1月至2014年1月上海交通大学医学院附属新华医院15岁以下29例行瓣膜置换患者临床资料.其中男性19例,女性10例,年龄2～15（10.66±3.55）岁.病因：先天性主动脉瓣发育异常24例,感染性心内膜炎5例.手术均在中度低温体外循环下进行,其中主动脉瓣置换22例、主动脉瓣二尖瓣双瓣置换术7例,均采用机械瓣膜.所有瓣膜置换均采用间断褥式缝合方法,术中采用Manouguian法行主动脉根部加宽5例;术后使用主动脉内球囊反搏（IABP）辅助2例.术后以华法林抗凝治疗,维持INR在2.0～3.0.结果 患者CPB时间（126.39±68.44）min,主动脉阻断时间（77.39±48.23）min.全组院内死亡1例.术后出现并发症3例,其中心律失常1例（窦性心动过缓、阵发性房室传导阻滞）,呼吸衰竭l例,急性肾衰竭1例,均经治疗后痊愈.随访1个月至8年,平均3.5年,随访期无死亡,所有患者机械瓣功能均良好,无再次手术,无抗凝相关并发症.结论 对于儿童主动脉瓣疾病患者,选择合适的手术时机进行主动脉瓣置换术,围术期及术后密切治疗,近期及中远期疗效均良好.     

Liver disease secondary to congenital heart disease in children

ABSTRACT

Introduction: Hepatic fibrosis and hepatocellular carcinoma (HCC) can develop in children with congenital heart disease. Although hepatic fibrosis and HCC are prone to develop after the Fontan operation, they can also develop in patients suffering from congenital heart disease who have not undergone Fontan operation.

Area covered: The history of cardiac hepatopathy including Fontan-associated liver disease is described. Patient characteristics, liver histology, imaging examinations and blood tests are reviewed to elucidate the mechanism of cardiac hepatopathy. In addition, a flowchart for the follow-up management of cardiac hepatopathy in children with congenital heart disease is proposed.

Expert opinion: Congestion and low cardiac output are the main causes of cardiac hepatopathy. Advanced hepatic fibrosis is presumed to be associated with HCC. HCC can develop in both adolescents and young adults. Regardless of whether the Fontan operation is performed, children with a functional single ventricle and chronic heart failure should be regularly examined for cardiac hepatopathy. There is no single reliable laboratory parameter to accurately detect cardiac hepatopathy; hepatic fibrosis indices and elastography have shown inconsistent results for detection of this disease. Further studies using liver specimen-confirmed patients and standardization of evaluation protocols are required to clarify the pathogenesis of cardiac hepatopathy.     

Mitral and aortic valve replacement in valvular rheumatoid heart disease

Specific endocarditis involving the aortic and mitral valves in a patient with peripheral rheumatoid arthritis is reported. The patient underwent prosthetic replacement of both valves. Typical rheumatoid nodules were detected histologically in the valvular tissues.     

Mitral and tricuspid valve closure in congenital heart disease.

Echocardiography was used to evaluate mitral and tricuspid valve closure in patients 1 day to 20 years of age. When possible, simultaneous phonocardiograms were obtained. The difference in time between the Q wave of the electrocardiogram and mitral closure and between Q and tricuspid closure was designated the delta value. Four groups of patients were assessed: 1) normals (40), secundum atrial septal defect (ASD) (10), mitral valve prolapse syndrome (Barlow's syndrome) (13), pulmonary hypertension (12), and pulmonic stenosis (6); 2) Ebstein's anomaly (10); 3) transposition of the great vessels (15); 4) right bundle branch block (RBBB) (25). Ten patients with surgically induced right bundle branch block were studied by phonocardiography alone. Group I had values of 50 msec or less (-5 to 50 milliseconds) and served as controls. Ebstein's anomaly showed prolongation of the delta value to 65 msec or greater in eight out of ten patients. Patients with transposition of the great vessels showed a striking difference from the preceding groups in that an average negative delta value was obtained. Twenty-two patients of group 4 (RBBB) had delta values within the normal range. This study has shown that a delta value greater than 65 msec is suggestive of Ebstein's anomaly. In addition, if the delta value is negative, transposition of the great vessels can be suspected.     

Outcome of pregnancy in women after pulmonary autograft valve replacement for congenital aortic valve disease

BACKGROUND AND AIM OF THE STUDY: The pulmonary autograft has been recommended as the valve of choice for aortic valve replacement (AVR) in young women contemplating pregnancy. However, current information on maternal and perinatal outcome of pregnancy in women with pulmonary autograft valve replacement is limited. METHODS: Using a nationwide Dutch registry (CONCOR) and a local Belgian tertiary care center database, 17 women (age range: 18 to 45 years) with pulmonary autograft valve replacement were enrolled into the study. Twelve pregnancies were observed among five different women, including one miscarriage and one elective abortion. RESULTS: Clinically significant (non-)cardiac complications were documented in two of 10 completed pregnancies. Complications included: (i) placental abruption necessitating Cesarean delivery at 29 weeks' gestation, further complicated by postpartum hemorrhage; and (ii) preterm premature rupture of the membranes resulting in premature delivery at 29 weeks' gestation with postpartum demise of the immature born child. Two women reported primary female infertility, but both became pregnant after hormonal substitution therapy. Four women reported irregularities of their natural menstrual cycle (menorrhagia, dysmenorrhea, polymenorrhea, oligomenorrhea, or amenorrhea). CONCLUSION: Successful pregnancy in women with pulmonary autograft valve replacement is possible, although serious and clinically significant events occurred during gestation. Infertility and menstrual cycle disorders appear to be more prevalent.     

Incidence of congenital heart disease in Qatari children

We surveyed the incidence of congenital heart disease in 49 887 native live born children in the period between 1984 to 1994 in Qatar. Each child with clinically suspected congenital heart disease underwent echocardiographic examination. Magnetic resonance imaging, cardiac catheterization and surgical intervention were done at the discretion of the patient's pediatric cardiologist. Virtually no postmortem examinations were performed. Children with congenital heart disease were entered into a computerized database and were then followed for 1–11 years. Congenital heart disease was diagnosed in 610 of 49 887 children for an incidence of 12.23/1000 live births. The reasons for the high incidence were high proportion of small muscular ventricular septal defects discovered before the time of their spontaneous closure, referral to and follow up by a single group of pediatric cardiologists, location of the pediatric cardiology service in the same setting where nearly all of the deliveries took place, freely available health care service, and echocardiographic examination of every child with a clinical diagnosis of congenital heart disease.