Morphologic and functional changes of ventricles between pre- and post- cone reconstruction surgery in children with Ebstein’s anomaly / 中华胸心血管外科杂志

Objective:To understand the morphologic and functional changes of ventricles between pre- and post- cone reconstruction(CR) surgery in children with Ebstein’s anomaly(EA).Methods:The clinical data of children with EA who underwent CR and cardiac magnetic resonance(CMR) in Shanghai Children’s Medical Center between July 2011 to April 2019 were collected and analyzed. Ventricular functions were assessed with the use of ejection fraction(EF), stroke volume index(SVI), cardiac output(CO), and cardiac index(CI). Ventricular morphologies were assessed with the use of end-diastolic ventricular volume(EDV), end-diastolic ventricular volume index(EDVI) and ventricular cine images. Paired student t tests and Wilcoxon rank sum tests were used for statistical analysis. Results:There were a total of 32 children with EA who underwent CR and CMR, with 13 males and 19 females, a median operative age of 2.9 years old(0.6-15.5 years old), and a mean follow-up time of(4.4±1.9) years. Seven patients had both preoperative and postoperative CMR, with a mean follow-up time of(3.3±1.4) years; Eleven patients had two or more postoperative CMR, with a mean interval time of(1.9±1.0) years. After the surgery, the median tricuspid-regurgitation grade decreased from 3 to 2, and the median New York Heart Association functional class improved from 2.5 to 1, the left ventricle(LV)-SVI, LV-EDV and LV-EDVI increased from 29.8 ml/m 2 to 43.2 ml/m 2( P=0.039), from 56.4 ml to 86.9 ml( P=0.004), from 50.5 ml/m 2 to 68.4 ml/m 2( P=0.022), respectively. And the long-term LV-EDV increased from 56.6 ml to 74.7 ml( P=0.002) when compared to that of early postoperative. There was no significant differences in right ventricle(RV)-EF, RV-SVI, RV-CO, RV-CI, RV-EDV and RV-EDVI between pre- and post- CR( P>0.05); but the long-term postoperative RV-CO and RV-EDV increased from 3.1 L/min to 4.1 L/min( P=0.008), from 67.5 ml to 96.5 ml( P<0.001), respectively, when compared with those of early postoperative. Conclusion:CR improves the function and morphology of both ventricles in children with EA. And although postoperative ventricles grow well, RV dysfunction persists.

Ten-year experience of mitral valve surgery in infants / 中华胸心血管外科杂志

Objective:To summarize the experience and effect of mitral valvuloplasty in the treatment of mitral valve disease in infants.Methods:The clinical data of 140 infants with mitral valve disease from June 2010 to June 2020 were retrospectively analyzed. There were 62 males and 78 females, with body weight of(6.4±1.4)kg and age of(196.6±80.1)days. Among them, 131 cases were moderately or above mitral insufficiency, and 9 cases were mitral stenosis. The perioperative and follow-up clinical data were recorded, and the therapeutic effect and prognostic factors of mitral valve plastic surgery were analyzed.Results:All 140 children were received surgery of mitral valve repair under cardiopulmonary bypass. Cardiopulmonary bypass time was(79.1±41.9)min, aortic cross clamp time was(46.8±20.0)min, 7(5%)early death. Mechanical ventilation time was(74.2±149.8)h. After surgery, mild mitral regurgitation was found in 48 cases, mild-moderate regurgitation in 53 cases, moderate regurgitation in 32 cases, moderate-severe regurgitation in 6 cases, severe regurgitation in 1 case, with no mitral stenosis. During the follow-up period of 6-126 months, 3 cases died in the long term, and 11 cases were reoperated. Freedom from >moderate mitral regurgitation at 5 years after surgery was 81.2%. Longer cardiopulmonary bypass time and postoperative mitral regurgitation were risk factors for death and moderate or above regurgitation at the follow-up.Conclusion:Mitral valvuloplasty can effectively treat the mitral regurgitation and stenosis in infants. The prognosis of children with short cardiopulmonary bypass time and good valve plastic effect is better. For infants with Carpentier type I, good shaping effect can be achieved by mitral annuloplasty alone, while for type Ⅱ and type Ⅲ, treatment should be tailored to deal with the problems at all levels of the valve, and additional annuloplasty should be performed.

Surgical experience of pediatric cardiac fibromas / 中华胸心血管外科杂志

Objective:To summarize single-center experience and short-term outcomes of surgical treatment of pediatric cardiac fibromas.Methods:There was a retrospective study of 10 patients who underwent surgical treatment of cardiac fibromas between January 2018 and October 2021. Fibromas were located in the left ventricle in 7 cases, and in the right ventricle in 3 cases. Mean tumor diameter was(5.6±2.0) cm.Results:Median age at surgery was 3.1 years old(5 months-9 years old). Nine patients received complete resection, and the other one received partial resection. One patient with giant left ventricular fibroma required extracorporeal membrane oxygenation support for ventricular fibrillation after weaning from cardiopulmonary bypass, and was successfully weaned on the third postoperative day. There was no early mortality. Median follow-up time was 6 months(1 month-3 years). No tumor recurrence nor ventricular arrhythmia was documented during the follow-up period.Conclusion:Pediatric cardiac fibromas can be usually treated by complete resection, with relief of cardiac dysfunction and ventricular arrythmia after surgery, and with satisfactory results.

A single-institution systematic review of 135 patients with pediatric primary cardiac tumors / 中华实用儿科临床杂志

Objective:To investigate the pathology characteristics, so as to provide treatment experience of primary cardiac tumors for pediatric patients.Methods:A retrospective study was conducted for 135 patients with primary cardiac tumor between January 2004 and December 2017 in Shanghai Children′s Medical Center.The median age was 0.54 years (range, 0-14.36 years). Single tumor was discovered in 61 cases and multiple rumors were found in 74 cases.Forty-five patients presented obvious clinical symptoms, 4 patients with mild symptoms and 86 patients without symptoms.Forty-one patients underwent surgical treatment, including complete resection of the tumor in 24 cases, partial resection in 16 cases and heart transplantation in 1 case.Concomitant valvuloplasty was required in 13 patients.Ninety-four patients did not receive surgical treatment.Results:A total of 40 patients underwent surgical treatment in Shanghai Children′s Medical Center.Three patients died of low cardiac output (in-hospital mortality: 7.5%), and 2 patients recovered from postoperative low cardiac output.The hemodynamic status was stable in the remaining 35 cases.One patient who received heart transplantation in another hospital survived and had good cardiac function during 24 months follow-ups.There were no significant differences in the survival rate between partial resection and complete resection of benign tumors.One late death was observed in patients with malignant tumors for 2 years after operation.Surveillance was kept in 94 non-surgical patients, among whom 1 case died for the abandon of surgery and 1 case died when waiting for heart transplantation.Other 2 patients were waiting for heart transplantation and other 90 patients had no hemodynamic disorder.Conclusion:Most of primary cardiac tumors are benign and long time follow-up should be able to get on for pediatric patients.When patients develop hemodynamical obstruction, arrhythmia or malignant tumor, surgical treatment is necessary.The principle of surgical therapy in these patients is to restore normal hemodynamic status, instead of completely removing tumor.Heart transplantation is potentially the only way for patients whose tumor cannot be resected.

Surgical treatment of infracardiac total anomalous pulmonary venous connection: A retrospective cohort study / 中国胸心血管外科临床杂志

@#Objective    To compare the outcomes of sutureless technique and conventional technique in the surgical repair for infracardiac total anomalous pulmonary venous connection (TAPVC). Methods    The clinical data of 46 consecutive patients with infracardiac TAPVC undergoing surgical repair in our hospital between June 2014 and April 2019 were retrospectively analyzed. Patients with combined congenital cardiac anomalies such as single ventricle and tetralogy of Fallot were excluded. Patients were divided into a conventional technique group and a sutureless technique group according to the surgical techniques. There were 35 patients in the conventional technique group, including 28 males (80.0%) and 7 females (20.0%) with a median age of 21 (8, 42) d and a median weight of 3.6 (3.0, 4.0) kg. There were 11 patients in the sutureless technique group, including 8 males (72.7%) and 3 females (27.3%) with a median age of 14 (6, 22) d and a median weight of 3.5 (2.9, 3.6) kg. The curative effect of the two groups was compared. Results    There were 5 deaths (10.9%) in the conventional technique group, including 4 in-hospital deaths (8.7%) and 1 late death (2.2%). Overall mortality of the conventional technique group (14.3%, 5/35) was higher than that of the sutureless technique group (0.0%, 0/11), although the difference was not statistically significant (P=0.317). Cox regression analysis showed that sex (P=0.042), age at repair (P=0.028), cardiopulmonary bypass time (P=0.007), aortic cross-clamping time (P=0.018) and duration of ventilation (P=0.042) were risk factors for postoperative mortality. The median follow-up was 18.00 (5.00, 37.75) months. Postoperative pulmonary venous obstruction occurred in 22 patients of the conventional technique group, which was significantly more than that of the sutureless technique group (P=0.000). Conclusion    For infracardiac TAPVC, sutureless technique can reduce the incidence of postoperative pulmonary venous obstruction compared with conventional technique.

The experience of surgical treatment for Ebstein anomaly in 141 children / 中华外科杂志

Objective@#To explore the surgical strategy for Ebstein anomaly in children.@*Methods@#From January 2003 to December 2015, a total of 141 cases of Ebstein anomaly were treated at Department of Pediatric Cardiothoracic Surgery, Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiaotong University. There were 65 male and 76 female patients, with age of (6.9±1.6) years (ranging from 10 months to 15 years), weight of (19.6±4.7) kg (ranging from 6.5 to 59.0 kg). All patients were diagnosed by 2 dimensional Doppler echocardiography and the septal leaflet and posterior leaflet displaced downward from 1.0 to 5.0 cm. The tricuspid valve regurgitation (TR) were mild in 26 cases, moderate in 46 cases and severe in 69 cases. Tricuspid valvuloplasty were performed in 131 cases (94 cone reconstruction, 37 valve hoist), tricuspid valve replacement in 2 cases and tricuspid valve closed in 8 cases. Surgical strategy were divided into biventricular heart function in 77 cases, one and a half ventricular heart function in 56 cases, and single ventricular heart function in 8 cases.@*Results@#Three patients were changed to one and a half ventricular repair from biventricular repair due to unstable hemodynamics in the early postoperative period. One case died in biventricular group. The complete atrioventricular block were occurred in 3 patients and pacemaker were applied. One hundred and forty cases discharged from hospital. There were mild TR in 118 cases, moderate in 14 cases and closed in 8 cases. One hundred and thirty-seven cases were followed up regularly in 18 to 172 months. Ninety-one cases were treated by cone reconstruction (mild TR in 75 cases, moderate in 15 cases and severe in 1 case). Thirty-six cases were operated by tricuspid valve hoist (mild TR in 21 cases, moderate in 12 cases and severe in 3 cases). In the patients with severe TR (4 cases), 3 cases were reoperated by cone reconstruction. One case′s valve was closed because of the dysplasia of the anterior valve and then from one and a half ventricular heart function to single ventricular function, the oxygen saturation was increased. Two patients underwent tricuspid valve replacement, 1 died and the other′s mechanical valve was removed, and changed to single ventricular function repair.@*Conclusions@#Although tricuspid cone reconstruction can achieve good results, the stable hemodynamic of early postoperative can be effectively maintained by using the surgical strategy of one and a half ventricular repair. To the patients with severe tricuspid regurgitation and hypoxemia due to severe tricuspid valve dysplasia, transforming to a functional single ventricle may be the only choice when there comes to the unstable hemodynamic.

The left ventricular assistance device was used for anomalous origin of the left coronary artery from the pulmonary artery in perioperative period / 中华外科杂志

<p><b>OBJECTIVE</b>To review the experience of left ventricular assistance device (LVAD) using for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in perioperative period.</p><p><b>METHODS</b>There were 29 patients with ALCAPA underwent surgical repair from May 2006 to May 2013. The mean age was 6.5 months (ranging from 3.3 to 12.1 months). The mean weight was 6.2 kg (ranging from 4.1 to 9.5 kg). Diagnosis was established by echocardiography in all patients. There were clinical symptomatic of the severe heart dysfunction and ejection fraction were 23% to 45%. Mitral insufficiency was moderate to severe in 8 patients and less than moderate in others. Surgical methods included the intrapulmonary tunnel (Takeuchi procedure) of 4 cases, direct reimplantation of the left coronary artery onto the aorta of 6 cases and reimplantation by pericardiac patch enlarge of 19 cases. Valvuloplasty were performed in 5 patients with mitral severe insufficiency. Twenty-two patients were treated only by medicine therapy. LVAD was used in 7 patients: there were 3 patients with low blood pressure at the end of surgical repair and 4 patients with low cardio output within 24 hours postoperatively.</p><p><b>RESULTS</b>Postoperatively, transesophageal echocardiography demonstrated that blood flow of the left coronary artery is fluently but left ventricular is also largement. The hemodynamic of 18 patients was stable in medicine group but 3 patients were sudden died of low cardiao output and ventricular fibrillation respectively. One patient was died of diffuse intravascular coagulation at the time of 72 hours after operation. The hemodynamic was stable in 6 patients in LVAD group and the devices after using time from 72 to 108 hours was taken down except one patient died of multi-organ dysfunction. The hospital mortality was 5/29 (17.2%). Nineteen survival (19/24) was followed up of 3.5 years (ranging from 1 to 7 years). Reoperations was performed for one patient with the supravalvar pulmonary stenosis due to the Takeuchi procedure 4 years postoperatively. Echocardiographic demonstrated that the blood flow of the left coronary artery are fluently. Mitral insufficiency was moderate in 2 cases, mild to moderate in 9 cases and mild in 8 cases. The ejection fraction value were 43% to 55% and apparent arrhythmia didn't occur.</p><p><b>CONCLUSIONS</b>Although late results are satisfactory and left ventricular function always recovery, early mortality is higher even though the protective methods are carried out during the whole cardiopulmonary bypass procedure. In order to decrease the early mortality, heart function evaluation and LVAD should be used as an effective cardiac support technique to prevent heart failure in time.</p>

Mitral valve replacement in the young children / 中华胸心血管外科杂志

Objective To review and summarize the experiences of mitral valve replacement in the children aged less than five years.Methods Twelve patients with moderate to severe mitral valve regurgitation (MR) or/and severe mitral valve stenosis (MS) were retrospectively study from January 2008 to December 2011,all of them suffered from severe heart failure and underwent mechanical mitral valve replacement.There were 9 males and 3 females.They aged from 4 to 58 months [mean (26.2 ± 18.1) months] and weighted from 5.6 to 13.0 kg [mean (9.6 ± 3.8) kg].Three patients underwent unsuccessful mitral valve repair before mitral valve replacement.17 to 23 mm aortic bileaflet mechanical prosthesis were reverse directional implanted in nine patients and 25 to 27 mm mitral bileaflet mechanical prosthesis were implanted in three patients.Results One patient died,the operative mortality rate was 8.3％.2 patients were complicated by cardiac arrhythmia and 2 patients complicated by mild hemolysis,all recovered after symptomatic treatment.The heart function of servived 11 patients improved significantly,none of them were complicated by haemorrhage or thromboembolic.Conclusion Severe mitral valve lesion injure heart function significantly for the young children and timely surgical intervention is the only option.Mitral valve replacement is the ultimate option for the patients failed in mitral valve annuloplasty.The introduction of aortic bileaflet mechanical prosthesis which smaller size is available and reverse directional implantation resolve the difficulty of prosthesis-patient mismatch essentially,most children with a small prosthesis has to redo mitral valve replacement with a larger size prosthesis.The compliance of permanent anticoagulation of young children is favourable and more intensively follow-up is essential to avoid the incidence of bleeding and thromboembolic.

Early assessment of post-operative cardiac output and causes of death in the neonates with congenital heart diseases / 中华胸心血管外科杂志

Objective To evaluate accurately the cardiac output, an early post-operative indicator for the cardiac function, after cardiac surgery in the neonates with congenital heart diseases and assess the risk factors for surgery, with an aim at exploring the early strategy for decressing the mortality. Methods From January 2007 to November 2007, forty-seven consec-utive neonates with complex congenital heart diseases underwent surgical correction at Shanghai Children's medical center.There were 38 boys and 9 girls with age from 3 days to 29 days [mean age (21.98 +8.15) days] and weight from 2.6 kg to 4.2 kg [ mean (3.49±0.51 ) kg]. 15 patients had delayed sternal clousure. The operations were performed with hypothermia, deep hypothermia low flow, or deep hypothermia circulatory arrest techniques. Cardiac index (CI) was derived from pulse contour analysis and calculated with the PiCCO plus system. Meanwhile, serum cardiac troponin I ( cTnl), mixed venous oxygen saturation ( SvO_2 ) and cardiopulmonary bypass (CPB) time were measured. Finally, the risk factors for surgical treatment in survivors and that associated with an increased mortality were analyzed. The association between post-operative cardiac output and the death after surgery was examined. Results Four neonates died after surgery, with a surgical mortality of 8.5%. CIvalue in the neonates [ (2.0±0.3 ) liters per minute per square meter of body surface was less than normal 2.5±0. 3. CI was associated inversely with CPB time but had a positive correlation with SvO_2. No significant difference association between CI and cTnI was observed. The value of cTnl was associated with the type of surgical procedures. Cases for which DHCA and low flow cerebral pefusion technique were used were free from neurological complications, ischemia in the lower extremities and oli-guria. The duration of cardiopulmonary bypass, urgent state of the operation and the abnormal coronary artery were associated with high mortality in the neonates after cardiac surgery. Conclusion The cardiac output of the neonates in whom cardiac pro-cedures were performed is adequate for the the oxygen supply required by the whole body though it is slightly lower than nomad after cardiac surgery. Higher mortality in the neonates during early postoperative period may be due to the complexity of the primary diseases, prolonged cardiopulmonary bypass time, residual abnormalities and severe acidosis before operation. Various techniques for CPB and that for the myocardial protection are safe and can provide an appropriate operative field.

Modified extracardiac conduit Fontan procedure for heterotaxia syndrome with complex congenital heart disease / 中华胸心血管外科杂志

Objective To review and summarize the experiences of modified extracardiac conduit Fontan operation for heterotaxia syndrome with complex congenital heart disease. Methods There were 11 patients with cynosis complex congenital heart disease, 9 were aspleenia syndrome (right atrium isomerism, including 6 single ventricle with common atrioventricular valve, 2 double outlet right ventricle with atrioventricular discordance, and 1 corrected transposition of the great arteries), and 2 were polyspleenia (left atrium isomerism, double outlet right ventricle with common atrioventricular valve). The mean age was (6.3?3.7)year-old, the mean body weight was (21.0?5.5)kg. 3 patients underwent one-stage modified extracardiac conduit Fontan procedure, 8 patients after bi-directional Glenn operation underwent two-stage procedure. Results During early postoperative period, two patients had low cardiac output syndrome, 1 renal dysfunction; and 1 supraventricle tachycardiac. The time of chest drainage (120*!ml/d) was more than 10 days in 2 patients. 2D-echo showed that superior vena cava blood flow rate was 0.6 to 0.8 m/s, inferior vena cava flow rate was 0.3 to 0.4 m/s. Oxygen saturation were from 0.92 to 0.95 in room air in 9 patients, 2 patients were under 0.86. The exercise capacity was significantly improved. All patients were survived and no early death. At follow-up ranging from 6 months to 2 years, supra vena cava blood flow rate was 0.8*!m/s, inferior vena cava flow rate is 0.4 to 0.7*!m/s. There was no thrombus formation in the conduit. The diameter of fenestration was 0.34*!cm and blood flow from right to left. No pulmonary vein drainage obstruction. Heart function was normal. No atrial arrhythemia were detected. The oxygen saturation in the room air was more than 0.90. No chronic effusion and protein-losing enteropathy, no mid-term death. Conclusion Modified extracardiac conduit Fontan operation is suited for heterotaxia syndrome with complex congenital heart disease. The incidence of arrhythemia is low.