Preoperative evaluation of children with anomalous origin of left coronary artery from the pulmonary artery and the follow-up after surgery / 中华实用儿科临床杂志

Objective To retrospectively study 50 children with anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA) and find the simple and practical indexes that may predict myocardial viability and the possible risk factors that may affect the choice of operation and the recovery after surgery.Methods A retrospective study was performed in 50 children with ALCAPA [29 male,21 female,aged from 4 months to 18 years,average (4.49 ±4.30) years] between Apr.1999 and Mar.2013.The preoperative examination included electrocardiogram (ECG),echocardiography,chest X-ray,mutislice spiral computerized tomography (MSCT),angiocardiography et al.Follow-up data were obtained by reviewing the records made in out-patient and recent telephone call.According to myocardial 18F-FDG imaging,the extent of myocardial viability of 15 patients with ALCAPA was classified into 4 grades.0 normal myocardial; 1 viable myocardial ;2 partial viable myocardial;3 myocardial infarction.Meanwhile,the global clinical scores were calculated and divided into 10 levels according to the deviations of clinical manifestations which included abnormal Q waves,left ventricular ejection fraction (LVEF),left ventricular end-diastolic dimension (LVED),cardiothoracic ratio(CTR),intercoronary collaterals(ICC),mitral regurgitation(MR) and aneurysm.The relationship of the extent of myocardial viability and clinical features were studied.All patients with ALCAPA were classified into groups by the global clinical scores and the preoperative,perioperative and post operative clinical manifestations were compared among groups.Results The extent of myocardial viability was related well to the global clinical scores (r =0.936,P ＜0.001),LVEF(r =0.783,P ＜ 0.001),CTR (r =0.770,P ＜ 0.002),abnormal Q waves (r =0.667,P ＜ 0.01) and LVED (r =0.637,P ＜ 0.02),but was not related to age,MR and ICC (r =-0.206,-0.268,-0.342,all P ＞ 0.05).The results showed that grade 0-1 equaled scores 0-3,grade 2 equaled scores 3-5,grade 3 equaled scores ＞ 5.Chil-dren with less viable myocardium had more severe clinical symptoms.Among abnormal Q waves,LVEF ＜ 50％,CTR ＞0.65,ICC dysplasia,median to severe MR and the global clinical scores ＞ 3,the global clinical scores ＞ 3 and LVEF ＜50％ showed a good predict of myocardial infarction.The preoperative,perioperative and post operative clinical manifestations were compared between 2 groups divided by the global clinical scores (group A,scores ≤ 3 and group B,scores ＞3) in all patients.More infants and toddlers,abnormal Q waves,ICC dysplasia,RCA/AO ＜0.2,larger CTR and lower LVEF were found in group B than those in group A.The time of cross-clamp and CPB was not different between the 2 groups,but the postoperative ventilation time and postoperative ICU stay were longer in group B than in group A.Fortyseven patients underwent operation and there were no operative deaths.One infant with scores 9 died while waiting heart transplantation.Follow-ups from 1 to 168 months were conducted in 38 patients(80.9％,38/47 cases) and 1 patient with ligation of the left coronary artery (LCA) had a sudden death after 8 months of surgery.All patients had gotten smaller LVED after surgery.Nineteen patients had mild MR and 3 patients had mild supravalvular pulmonary stenosis in following-up.Of the 16 patients with LVEF ＜ 50％,14 had a recovery of LVEF,2 patients still had LVEF ＜ 50％.Conclusions In children with ALCAPA,the extent of myocardial viability evaluated by myocardial 18 F-FDG imaging is related closely to the preoperative clinical manifestations.The global clinical scores ＞ 3 and LVEF ＜ 50％ have a good predict of myocardial infarction.Even in young children with severely depressed left ventricular function,higher global clinical scores and more myocardial infarction,median and long-term follow-ups showed satisfactory recovery of cardiac function after successful restoration of a dual coronary arterial system.

The analysis of the recent effectiveness of the total anomalous pulmonary venous connection repairs on infants / 中华外科杂志

<p><b>OBJECTIVE</b>To review the efficacy of total anomalous pulmonary venous connection (TAPVC) repair and to conclude the factors impacting the peri-operative death rate.</p><p><b>METHODS</b>The clinical data of 145 infants under 1 year old who underwent the TAPVC repair from January 2001 to July 2008 was analyzed. There were 94 male and 51 female patients. The mean age when the repair was performed was (7 ± 3) months, and the average weight was (6.3 ± 1.6) kg. As to the pulmonary connection type, 77 patients were supracardiac (53.1%), 47 patients were cardiac (32.4%), 9 patients were intracardiac (6.2%), and the remaining 12 patients were mixed (8.3%). Pre-surgery echocardiography showed that 21 patients had pulmonary venous obstruction (12 patients were supracardiac type, 3 patients were cardiac type, 3 patients were intracardiac type, and 3 patients were mixed type).</p><p><b>RESULTS</b>All patients underwent two-ventricle anatomy correction (the cases of complex malformations had been excluded). Peri-operative mortality was 11.7% (17/145). Because of the significant improvement in the surgical techniques, anesthesiology, cardiopulmonary bypass and the management of ICU in January 2006, the population was divided into two groups: A (before January 2006) and B (after January 2006). Peri-operative mortality decreased from 19.0% in group A to 6.2% in group B(P = 0.020). After analysis, it was determined that the factors impacting mortality were which group the patient belongs to, whether he/she had preoperative pulmonary vein obstruction and how big the atril septel connection was. The operative technique to keep the anastomotic aperture adequate and prophylaxis pulmonary hypertensive episodes contributed to the improvement on the mortality rate. There had been no case of repeating the surgery because of pulmonary venous obstruction during peri-operative care period.</p><p><b>CONCLUSIONS</b>Improvements of the surgical technique as well as the treatment in preoperative and postoperative have led to the reduction of the mortality. Preoperative pulmonary vein obstruction is still an important factor that contributes to early mortality.</p>

One-stop hybrid cardiac surgery for neonates and young children with congenital heart disease / 中华心血管病杂志

<p><b>OBJECTIVE</b>To summarize our 5 years experiences of one-stop hybrid procedure (OHP) for the management of congenital heart disease (CHD) in neonates and young children (< 2 years old).</p><p><b>METHODS</b>Clinical data derived from consecutive 152 young children and neonates with CHD underwent OHP between March 2004 to March 2009 were analyzed. Patients were divided into 3 groups: Balloon plasty group (n = 72), device closure group (n = 43) and collateral arteries occlusion group (n = 37). All procedures were image-guided and performed in a specially designed hybrid operation room. Incidence of major adverse cardiovascular events was obtained.</p><p><b>RESULTS</b>Patients received successful per-ventricular valvuloplasty or per-aortic balloon angioplasty in balloon plasty group. Two patients in this group with severe right ventricle outflow obstruction received regular open-heart outflow tract reconstruction immediately (n = 1) or selective conventional open-heart operation after discharge (n = 1). One neonate with pulmonary atresia with intact ventricular septum died from liver failure 6 month after OHP. In device closure group, device closure was failed in 3 cases (2 with atrial and 1 with ventricular septum defects), 1 young child with ventricular septum defects died from pneumonia after successful device closure. No device malposition was observed in device closure group during the follow-up. All patients received major collateral arteries occlusion and open-heart correction were discharged without complication.</p><p><b>CONCLUSION</b>OHP could avoid or shorten the application of cardiopulmonary bypass and reduce the surgical trauma in selected young children with CHD. Although OHP was feasible and safe, the image outfits, image-guided technology and OHP-related device should be further developed and improved for better procedure outcome.</p>

Outcome of surgery for interrupted aortic arch combined cardiac anomaly / 中华外科杂志

<p><b>OBJECTIVE</b>To retrospectively review the experience in repair of interrupted aortic arch (IAA) and associated cardiac anomaly.</p><p><b>METHODS</b>From January 1997 to January 2008, 36 patients with interrupted aortic arch and associated cardiac anomaly underwent surgical treatment. There were 22 male and 14 female. Mean age of the 35 children patients was 2.8 years, with a range from 2 months to 7 years. There was a 31 years old adult patient. Types of interrupted aortic arch include 30 cases of type A and 6 cases of type B. In all 36 patients, 33 cases had patent ductus arteriosus (PDA) and intracardiac abnormality, including 28 cases of simple anomaly as ventricular septal defect and 5 cases of complex anomaly, two cases were single IAA arch without PDA and other cardiac defect, one case had no intracardiac anomaly but PDA. For 33 patients with PDA and intracardiac anomaly, median sternotomy was used to simultaneously repair interrupted aortic arch and intracardiac defect in 31 cases, left thoracotomy and median sternotomy were applied to repair IAA and intracardiac anomaly respectively in one case, one patient had palliative repair. For three patients without intracardiac anomaly, left thoracotomy was applied in two cases, median sternotomy and abdominotomy were used in one adult patient. Techniques of operation for interrupted aortic arch include 16 cases of conduit connection, 9 cases of direct anastomosis, 9 cases of direct anastomosis with patch augmentation, 1 case of subclavian flap aortoplasty. In all 31 cases of one-stage operation through median sternotomy, selective cerebral perfusion was used in 17 patients, deep hypothermia and low flow were applied in 8 cases, deep hypothermia circulatory arrest was performed in 6 patients.</p><p><b>RESULTS</b>There were 5 hospital deaths. Three cases died of pulmonary infection, 1 case died of of pulmonary hypertension crisis, and another case died of postoperative low cardiac output, which was misdiagnosed before operation. Seven cases had other main postoperative complications. Thirty-one survivors were followed up from 3 months to 5 years, there was no late death and reoperation.</p><p><b>CONCLUSION</b>One-stage repair through median sternotomy using selective cerebral perfusion or deep hypothermia and low flow can be applied to most of the cases with associated cardiac anomaly.</p>

Results of surgical treatment of 1,387 infants under 6 months of age with congenital heart disease / 中华儿科杂志

<p><b>OBJECTIVE</b>To summarize and review the result of surgical repair of congenital heart disease in infants under 6 months of age.</p><p><b>METHODS</b>Between January 1997 and December 2007, 1387 infants under 6 months of age with congenital heart disease were operated on. There were 675 cases with ventricular septal defect with pulmonary hypertension (VSD/PH), 138 with complete transposition of the great arteries (TGA), 155 with tetralogy of Fallot (TOF), 111 with totally abnormal pulmonary venous connection (TAPVC), 54 with coarctation of aorta or interrupted aortic arch with ventricular septal defect [CoA(IAA)/VSD], 46 with double outlet right ventricle (DORV), 25 with pulmonary atresia with ventricular septal defect (PA/VSD), 24 with pulmonary atresia with intact interventricular septum (PA/IVS) and so on. The operative procedure was dependent on different diseases. Follow-up has been conducted in patients with some complex congenital heart diseases.</p><p><b>RESULTS</b>In the recent 11 years, the number of surgical repair in infants under 6 months of age, including neonates, with congenital heart disease has been increased. In contrast to the early phase when ventricular septal defect was the major disease treated with surgery, infants with complex congenital heart disease account for half of all cases treated with surgery now. In the meantime, the surgical mortality has been decreased year after year. There were 110 deaths in our group and the total mortality was 7.9% (110/1387). With improvement of surgical procedure, the mortality was decreased from 11.5% - 14.4% in 1997 - 2003 to 8.6% - 8.9% in 2004 - 2005 to 3.3% - 3.8% in 2006 - 2007. Follow-up data were available for 98 patients in TGA (83.8%, 98/117), 79 in TAPVC (87.8%, 79/90), 68 in TOF (48.2%, 68/141), 13 in PA/VSD (65%, 13/20) and 19 in PAA/IVS (95%, 19/20). The duration of follow-up ranged from 3 to 86 months. There were 16 late deaths, 4 in TGA, 10 in TAPVC and 2 in PA/VSD patients. The majority were asymptomatic on follow-up. Mild residual obstruction was seen in 4 cases with TAPVC. Pulmonary hypertension was seen in 5 cases with TAPVC. Mild aortal valve regurgitation, pulmonary valve regurgitation and tricuspid valve regurgitation were seen in 23 cases with TGA (23.5%, 23/98). Some PA/VSD patients had second operation.</p><p><b>CONCLUSIONS</b>Most symptomatic neonates and infants younger than 6 months with critical congenital heart defects can undergo corrective operation under acceptable risk. Due to improvements in perioperative, anaesthetic, surgical, and postoperative care, contemporary hospital mortality can be reduced to 3.3% - 3.8%. Palliative procedures still play an important role in the staged treatment of severe complex heart defects in neonates and infants younger than 6 months of age.</p>

Surgical treatment of aortic coarctation with intracardiac anomaly in infants and toddlers / 中华外科杂志

<p><b>OBJECTIVE</b>To review the experience in repair of aortic coarctation with intracardiac anomaly in infants and toddlers.</p><p><b>METHODS</b>From January 2000 to December 2006, 84 infants and children diagnosed as aortic coarctation with intracardiac anomaly underwent surgical treatment. Mean age of the patients was 13.5 months, with a range from 1 month to 3 years. Mean body weight was 7.3 kg, with a range from 3.3 to 15 kg. Twelve patients complicated with complex intracardiac anomaly. Seventy-two patients complicated with ventricular septal defect and other simple anomaly. Twenty-one patients had hypoplasia of the aortic arch. Sixty-two patients had one-stage repair. Median sternotomy was used to simultaneously repair coarctation and intracardiac defect in 49 patients. Left thoracotomy and median sternotomy were applied to repair aortic coarctation and intracardiac anomaly respectively in 13 patients. Twenty-two patients had staged repair. Operational techniques for aortic coarctation include 42 patients of patch aortoplasty, 30 patients of resection and end-to-end anastomosis, 6 patients of subclavian flap aortoplasty, 3 patients of vascular bypass, and 1 patient of balloon dilation. In all 49 patients of one-stage operation through median sternotomy, selective cerebral perfusion was used in 43 patients, deep hypothermia low flow was applied in 4 patients, deep hypothermia circulatory arrest was performed in 2 patients.</p><p><b>RESULTS</b>There were 8 hospital deaths. The mortality is 9.5%. Among 8 deaths, 3 patients were misdiagnosed.</p><p><b>CONCLUSIONS</b>Surgeries for aortic coarctation with intracardiac anomaly have satisfactory short-term results in infants and toddlers. One-stage repair through median sternotomy can be applied to most of the patients. Selective cerebral perfusion with deep hypothermia and circulatory arrest in lower body can protect the brain and other vital organs.</p>

Surgical treatment of flail leaflet of tricuspid valve / 中华外科杂志

<p><b>OBJECTIVE</b>To explore the approach and the result in tricuspid valve insufficiency treatment by a cusp remodeling technique.</p><p><b>METHODS</b>Nine patients with severe tricuspid regurgitation, congenital lack of chordae in 6 cases and traumatic rupture of chordae in 3 cases, underwent surgical repair between April 1997 and March 2006. There were six male and three female. Their ages ranged from 8 years to 57 years. One or two segments of flail leaflets were presented in these patients. Valve repair was performed by suture of the free edge of the affected cusp segment, plication of the segment of annulus devoid of leaflet, and fixation of the neo-annulus with a flexible annuloplasty ring.</p><p><b>RESULTS</b>All patients survived and recovered after the operation. Echocardiography showed good coaptation with no regurgitation of the tricuspid valve in six patients and a mild residual tricuspid regurgitation in three. A remarkable decrease in the diameter of the right ventricle (anterior to posterior) was observed: from mean (43.6 +/- 4.2) mm (range 29 mm to 64 mm) preoperatively reducing to mean (24.0 +/- 1.8) mm (range 16 mm to 32 mm) postoperatively. All patients are doing well in 1 month to 109 months follow up.</p><p><b>CONCLUSION</b>The procedure provided a simple and valuable option for repair of flail leaflet of tricuspid valve caused by congenital lack of chordae or traumatic rupture of chordae.</p>

Effects of intraoperative hybrid procedures for the treatment of congenital heart disease / 中华心血管病杂志

<p><b>OBJECTIVE</b>A hybrid operation is a joint procedure involving the interventional cardiologist and the cardiac surgeon concomitantly to optimize surgical management for complex congenital heart disease. We analyzed the feasibility and efficacy of this procedure in 20 patients with congenital heart disease.</p><p><b>METHODS</b>Data from 20 patients who underwent intraoperative hybrid procedures from March to October 2005 were analyzed. Two groups were identified: (1) Balloon valvuloplasty group: pulmonary atresia with intact ventricular septum in neonate (n = 3) and severe pulmonary stenosis in infants (n = 4); (2) Device closure group: atrial septum defects (ASD) in child (n = 7), adult ASD (n = 3) and multiple ventricular septum defect (VSD, n = 3). Pulmonary balloon dilation under the guidance of echocardiography together with modified Blalock-Taussig shunt procedure and PDA ligation were applied to patients in balloon valvuloplasty group. For ASD patients, the occluder was placed via right atria after opening the chest either with median sternotomy (4 case) or with submaxillary minimally invasive incision (6 cases), PDA ligation was performed in 1 patient and 2 coronary artery bypass surgeries were performed in 2 adult ASD patients complicating CHD. For multiple VSD, perimembranous VSD was closed surgically and muscular VSD closed by occluder via tricuspid valve approach under cardiopulmonary bypass.</p><p><b>RESULTS</b>All operations were successful and 1 infant received another conventional valvuloplasty due to the pulmonary restenosis after initial balloon valvuloplasty and 1 patient's muscular VSD was closed by conventional procedure after unsuccessful device closure. No device-related complications were found during follow-up (1-6 months).</p><p><b>CONCLUSION</b>Our results showed that intraoperative hybrid procedure was feasible and effective in selected patients with congenital heart disease.</p>