Uhl畸形是一种罕见的先天性心脏病,Uhl畸形患儿预后较差,手术治疗为目前主要治疗方法.现回顾既往相关文献,总结Uhl畸形患儿临床表现、诊断方法及手术治疗的方法及疗效,探讨Uhl畸形患儿的最佳诊治方法.

Noonan syndrome (NS) is a genetic syndrome causing obstructive hypertrophic cardiomyopathy (HCM) in infants. Studies of cardiac surgery in pediatric HCM patients with NS (NS-HCM) are lacking. We aim to characterize the early disease course of young NS-HCM patients before adolescence and assess their complications and survival condition after septal myectomy. Pediatric obstructive HCM patients who underwent septal myectomy at age 10 years or under were enrolled consecutively between 2009 and 2019. Ten patients with NS and 43 non-NS patients were enrolled in our study. NS-HCM patients were diagnosed at a younger age (5.00 ± 7.48 months vs. 18.73 ± 26.96 months) and more often had biventricular outflow tract obstruction (90.00% vs 30.23%). The surgical treatment can significantly reduce the pressure gradient of the outflow tract. More NS-HCM patients had biventricular reconstruction (70.00% vs 25.58%). The overall survival rates in all patients were 98.04% during 5-year follow-up, respectively. Complete heart block (CHB) was the most prevalent complication in patients with NS and tended to be more common than in non-NS patients (20% vs 7.32%). No sudden cardiac death (SCD) occurred in CHB patients who had pacemaker implantation. Biventricular reconstruction was the risk factor for CHB. Septal myectomy is safe and effective surgery to relieve obstruction in young age pediatric obstructive NS-HCM patients. Postoperative CHB is a common complication, especially in patients who underwent biventricular reconstruction. The implantation of pacemaker can protect CHB patients from SCD and improve their prognosis.

OBJECTIVEThis study aimed to investigate the association between long-term survival and different management of major aortopulmonary collateral arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.METHODSFrom November, 2009 to October, 2018, a total of 98 consecutive patients with pulmonary atresia, ventricular septal defect, major aortopulmonary collateral arteries, and hypoplastic pulmonary arteries treated with modified Blalock-Taussig shunt or right ventricle-pulmonary artery connection were included. Fifty-five patients who received occlusion or ligation of major aortopulmonary collateral arteries during or after palliative procedure were occlusion group, and the other 43 patients were no occlusion group. The early and late outcomes were compared.RESULTSThe mean duration of follow-up was 30.9 months in no occlusion group and 49.8 months in the occlusion group (p < 0.001). Multivariate analysis showed that only no occlusion of major aortopulmonary collateral arteries was predictive of total mortality (Hazard Ratio: 4.42, 95% CI: 1.27 to 15.42, p = 0.02). The Kaplan-Meier survival curves confirmed that patients without occlusion of major aortopulmonary collateral arteries demonstrated worse survival as compared with the occlusion group (p = 0.013). The Kaplan-Meier survival curves of patients who underwent different palliative procedures showed no differences.CONCLUSIONSFor patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries when a primary repair is not feasible, those without occlusion of major aortopulmonary collateral arteries have a higher risk of death following an initial palliative procedure compared with patients who underwent occlusion of major aortopulmonary collateral arteries. The occlusion of major aortopulmonary collateral arteries is not associated with a higher rate of complete repair or better improvement of pulmonary artery growth.

目的:探讨超声心动图在全腔静脉-肺动脉连接术(Total cavopulmonary connection,TCPC)后紫绀原因诊断中的应用.方法:回顾分析180例TCPC术后患者临床资料,并对25例术后出现紫绀患者的超声心动图结果进行分析.结果:10例围术期出现严重低氧血症,超声检查示膈肌麻痹、胸腔积液、肺不张,治疗后随访紫绀好转;5例超声检查示外管道开窗过大;4例遗漏单支肝静脉直接回流入心房;1例外管道内血栓形成并狭窄,术中结扎的导引静脉开放,形成下腔静脉-肝静脉-门静脉-导引静脉-共同肺静脉干-心房逆流;5例常规超声检查无明显异常,声学造影示肺动静脉瘘.结论:超声心动图显示TCPC术后患者心内结构及血流动力学变化,在术后紫绀原因诊断中发挥关键作用.

Background. Contemporary experiences regarding childhood hypertrophic obstructive cardiomyopathy are limited. This study aimed to describe the clinical presentation of childhood hypertrophic obstructive cardiomyopathy and its relevant surgical outcome. Methods. In all, 117 consecutive children with hypertrophic obstructive cardiomyopathy aged 0.6 to 17.5 years who underwent septal myectomy at our institution between February 2009 and December 2018 were included. Medical records and other patient-related data were reviewed. Results. In the present study, the anatomic and physiologic characteristics of childhood hypertrophic obstructive cardiomyopathy were highly heterogeneous, with simultaneous right ventricular outflow tract obstruction in 22 patients (18.8%), coronary myocardial bridging in 25 patients (21.4%), and intraventricular anatomic abnormalities in 61 patients (52.1%). The mean peak left or right ventricular outflow tract gradient, interventricular septal thickness, and degree of mitral regurgitation significantly decreased after surgery. One early death was noted in the study. During follow-up, three sudden cardiac deaths were noted. The overall survival rates at follow-up were 100% at 1 year and 96.5% at 3 years. The overall survival rates free from reoperation were 99.1% at 1 year and 98.0% at 3 years. Conclusions. In our cohort of children with hypertrophic obstructive cardiomyopathy undergoing septal myectomy, biventricular obstruction, myocardial bridging, and intraventricular anatomic abnormalities are frequent phenotypic components. Despite the complexity of childhood hypertrophic obstructive cardiomyopathy, surgical treatment results in a favorable outcome in carefully selected patients. (C) 2020 by The Society of Thoracic Surgeons

Partial anomalous pulmonary venous drainage (PAPVD) is a common congenital heart disease. If the insertion of an anomalous pulmonary vein (PV) is high into the superior vena cava (SVC), the traditional 1-patch or 2-patch surgical repair might be challenging. Unlike patch procedures, the cavoatrial anastomosis technique (Warden procedure) theoretically reduces complications such as sinus node dysfunction and venous obstruction. We investigate outcomes of the Warden procedure (WP) at a single institution. A total of 67 patients (42 males and 25 females) with PAPVD who underwent the WP between January 2011 and December 2018 were consecutively enrolled. The median age was 2.8 years (3–61 years old). 52 cases were younger than 18 years old. perioperative and follow-up clinical data were collected. In addition, we selected possible risk factors (a total of 18 risk factors) of SVC stenosis or obstruction. Univariate and multivariate analyses were conducted to confirm the independent risk factors. The average Cardiopulmonary bypass (CPB) time was 132.3 ± 46.3 min, and the aortic cross-clamp time was 85.2 ± 35.7 min. One patient died postoperatively in hospital. The mean follow-up time of the remaining patients (n = 66) was 15.8 ± 14.5 months (3–64 months). No abnormal cardiac function or sinus node dysfunction cases were identified during the follow-up. Of the survival patients, no venous obstruction was presented before the discharge. Numbers of patients identified with mild PV and SVC stenosis were 1 (1.5%) and 7 (10.6%, 4 of them turned negative eventually) at discharge. At the last follow-up, no PV obstruction was identified, whereas, 4 cases (6%) had SVC obstruction. Numbers of PV and SVC stenosis cases were 3 (4.5%) and 8 (12%). Univariate and multivariate analyses showed that only the diameter of SVC less than 10 mm was an independent risk factor for SVC stenosis or obstruction at the last follow-up. Warden procedure can effectively treat PAPVD (anomalous drainages into SVC), with satisfactory early and mid-term postoperative results. Different modifications of the Warden procedure may be selected according to the anatomic characteristics. The diameter of SVC less than 10 mm predicts SVC stenosis or obstruction after Warden procedure.

OBJECTIVES:Postoperative fluid overload is common in children after cardiac surgery, especially for those with right ventricular outflow tract obstruction, which is associated with poor outcomes. This study was conducted to investigate whether early peritoneal dialysis (PD) was associated with improved outcomes in these children at high risk of fluid overload.METHODS:Between January 2010 and January 2015, a total of 2555 consecutive patients with right ventricular outflow tract obstruction underwent anatomical repair. Using empirical risk evaluation, 219 patients at high risk of fluid overload were identified. A propensity score matching was performed to correct the selection bias and identify the comparable patient groups: the early PD group, in whom PD was initiated within 6 h of admission in paediatric ICU, and the control group, without early PD. The mechanical ventilation time, vasoactive-inotropic score and time to negative fluid balance were compared in 45 matched patient pairs (totally 90).RESULTS:After propensity matching, there were no statistically significant differences between the 2 groups in terms of demographics and preoperative characteristics. The early PD group had shorter mechanical ventilation time [median 49 h, interquartile range (IQR) 31-97 h vs median 76 h, IQR 55-166 h; P < 0.01]; lower vasoactive-inotropic score (median 17, IQR 16-21 vs median 22, IQR 18-26; P < 0.01); shorter duration of inotrope requirement (median 7 days, IQR 6-9 days vs median 8 days, IQR 7-13 days; P < 0.01); shorter time to negative fluid balance (median 20 h, IQR 13-34 h, vs median 48 h, IQR 40-74 h; P < 0.01) and a higher rate of negative fluid balance at 24 h (69% vs 29%, P < 0.01).CONCLUSIONS:When compared with the control group, the early PD group showed shorter mechanical ventilation time, less inotropic requirement and lower time to attain negative fluid balance. On the basis of our empirical risk-evaluation practice, early PD could improve immediate postoperative recovery in children with right ventricular outflow tract obstruction.

Background: Anomalous origin of coronary artery from the pulmonary artery (ACAPA) is a rare congenital coronary malformation with a high mortality whether in infants or adult patients. This study reviews 20 years of surgical treatment in a single center and aims to establish the optimal surgical strategies for this rare pathology.Methods and results: From April 1994 to March 2015, 96 consecutive patients aged from 3 months to 60 years underwent coronary repair surgery. The surgical procedures included ligation (3 cases), ligation along with CABG (6 cases), transpulmonary baffling (Takeuchi Procedure, 14 cases) and directly implantation of the anomalous coronary artery (ACA) into the aorta (73 cases). Postoperative extracorporeal mechanical circulatory support (ECMO) was necessary in 4 cases. Mitral valve repair was performed in 40 patients with moderate or severe mitral regurgitation (MR). Mitral replacement was performed in one patient with severe MR.There were one early and two late deaths. One patient underwent a second operation because Baffle leaks. During mean 10.45 +/- 8.96 year follow-up (1 month-18 years), both early and late improvement of left ventricular function was observed in most patients (8 patients lost of follow-up).Conclusions: The establishment of a two-coronary system is the main goal of surgical therapy today. In different procedures, the direct implantation of the ACA into the ascending aorta is the best method and has good long-term results. ECMO as a bridge to recovery that will play an integral part in moderns' surgical treatment. (C) 2016 Elsevier Ireland Ltd. All rights reserved.

Extended septal myectomy for children and adolescents with hypertrophic obstructive cardiomyopathy (HOCM) is a challenging procedure, and related data are currently limited. Our study objective was to assess the early outcomes in children and adolescents with HOCM after extended septal myectomy. From October 2007 to August 2015, 40 consecutive patients with HOCM underwent transaortic extended septal myectomy in Fuwai Hospital, Beijing, China. Patients clinical data were analyzed retrospectively. Mean age at the time of operation was 11.3 ± 4.3 (0.7–16.7) years. Mean body weight at the time of surgery was 40.8 ± 19.7 (4.3–92.0) kg. After myectomy, mean left ventricular outflow tract gradient decreased from 80.1 ± 33.8 to 14.7 ± 11.5 mmHg and mean degree of mitral regurgitation decreased from 1.9 ± 0.9 to 0.5 ± 0.5 (p < 0.001 for both). Concomitant surgical procedures were required in 13 patients (32.5 %). There was no early death. Residual systolic anterior motion and left ventricular outflow tract obstruction were reported in two and three patients, respectively. Moderate aortic regurgitation was found in one patient during a follow-up of 26.4 ± 15.1 months. Restrictive symptoms were improved in the patients with New York Heart Association functional class I or II. A 15.8-year-old patient died 16 months after operation. A permanent pacemaker was installed in one patient 3 months after operation. Extended septal myectomy is safe and effective in children and adolescents with HOCM, with excellent clinical and echocardiographic outcome at early follow-up.

Introduction: Data on the incidence, natural history and treatments of biventricular obstruction hypertrophic cardiomyopathy are limited. Objective: We aimed to the compare midterm outcomes of surgery and medication in biventricular outflow tract obstruction(BVOTO) HCM with left ventricular outflow tract obstruction (LVOTO) HCM and non-obstructive hypertrophic cardiomyopathy (NOHCM). Methods: Among 4945 HCM patients, we identified 43 with BVOTO and recruited age and sex matched 54 with LVOTO and 52 with NOHCM. The primary endpoint was all cause death and second endpoints were cardiovascular events. Results: Age and male were paired among BVOTO, LVOTO and NOHCM (26±17 ys, 30±18 ys, 31±16 ys P=0.3; 57%, 66%, 69%, p=0.5). More patients with BVOTO (69%) had NYHA III/IV than with LVOTO (51%)and NOHCM (15%) (p=0.000). And patients with BVOTO (5.6%±4 %) had higher risk of SCD than with LVOTO (3.9%±1.8%) and NOHCM (2.9%±2.1%)(p=0.000). In BVOTO, 20 patients had left-and-right sided correction (LRVC-B) (1 died and 5 had III AVB), 12 patients accepted left-sided correction (LVC-B) (1 died and 1 occurred III AVB) while 11 patients received medication. At the last follow-up, patients in LRVC-B and LVC-B had a reduction in left and right ventricular intracavity pressure gradient and NYHA class (vs before myectomy, p < 0.001). And no death occurred in the remained 147 patients. The second endpoint-free survival rates of BVOTO were less than LVOTO and NOHCM while the rates were similar among LRVC-B, LVC-B, LVC-L and NOHCM groups. (Fig1ab) Independent predictors of the second endpoint were age (HR 1.05,95%CI 1.0-1.1), RVOTO (HR 1.06,95%CI 1.03-1.10), myectomy (HR 0.16,95%CI 0.03-0.61) and pre-NYHA (HR 4.1,95%CI 2.1-8.33). Conclusions: Patients with BVOTO had more severe symptoms. Both LRVC-B and LVC-B could improve symptom but LRVC-B had a higher risk of III AVB. Mid-term outcomes after surgical correction in BVOTO were similar to that in NOHCM.

Background: Reoperation for congenital heart disease may be associated with cardiac or vascular injuries during repeat sternotomy, resulting in increased mortality and/or morbidity rates. The aim of this study was to determine the frequency of these cardiac injuries and the associated outcome.Methods: Between January 2012 and December 2013, 4256 sternotomy procedures were performed at the Pediatric Cardiac Center in Fuwai Hospital, including 195 repeat sternotomy procedures (RS). We retrospectively studied the clinical data of 195 RS patients and 250 randomly selected primary sternotomy (PS) patients. Demographic and operative details, major injures (MI), and clinical outcomes were compared between the two groups. We also assessed the risk factors for major injury and in-hospital mortality and morbidity.Results: Significant differences were observed between the RS and PS groups in terms of skin incision to cardiopulmonary bypass(CPB) time, overall CPB time, cross-clamp time and blood requirement, and ventilation time (p < 0.001). MI during RS occurred in 7 of the 195 patients (3.6 %), while operative mortality was 1.0 % (2/195). However, in the RS patients, mortality and morbidity rates were not significantly different between the MI subgroup and the non-MI subgroup (p = 1.000 and 0.556, respectively). Additionally, no significant difference was found between the RS and PS groups in terms of mortality (p = 1.000) and morbidity (p = 0.125).Conclusions: Both RS and MI are not associated with increased risk of operative mortality and morbidity. Outcomes for reoperative pediatric operations in contemporary practice are similar with those for primary operations.

Objective To explore the changes in pulmonary surfactant (PS) and to analyze the clinical data of the complex congenital heart disease(CHD) infants who got acute respiratory distress syndrome (ARDS) after surgery and those of the simple CHD infants who recovered smoothly after surgery.Methods Fifteen infants less than 3 months with congenital cardiac defects undergoing cardiovascular surgery and developed ARDS after operation were chosen as ARDS group,other 15 infants without ARDS were chosen as a control group.The data of mean mechanical ventilation time,the intensive care unit treatment time,mortality and the incidence of postoperative complications were collected.And saturated phosphatidylcholine (SatPC),total phospholipids (TPL) and total protein (TP) in sequential airway aspirates and surfactant protein-A (SP-A) in the blood after operation were determined.Results The mean mechanical ventilation time of ARDS group [(6.69 ± 2.35) d] was longer than that of the control group [(0.90 ±0.84) d] (t =8.986,P ＜ 0.01),and the mean ICU treatment time of ARDS group [(7.20 ± 3.39) d] was longer than that of the control group [(1.56 ± 0.77) d] (t =6.283,P ＜ 0.01).ARDS group had a higher rate of mortality (6.67％) and the incidence of postoperative complications (46.67％) than that of the control group (0) (F =10.562 5,2.227 3,all P ＜ 0.05).Plasma SP-A level in ARDS group [(148.18 ± 19.18) mg/L] was higher than that in the control group [(93.06 ± 12.39) mg/L] (t =9.349,P ＜ 0.01).The SatPC/TPL decreased in ARDS group [(0.602 9 ± 0.045 1) ％] compared with that in the control group [(0.819 9 ± 0.074 1) ％] (t =-9.689,P ＜0.01).The SatPC/TP was decreased in ARDS group[(0.085 6-± 0.069 5) mg/g] compared with that in the control group [(0.485 5-± 0.039 5) mg/g] (t =-19.374,P ＜ 0.01).Conclusions ARDS after the surgery of CHD may significantly reduces PS activity in the lungs and its loss into blood.Their recovery process may be longer than that of the simple simple CHD infants with unstable conditions.