Medical treatment following successful separation of thoracopagus conjoined twins / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze and summarize the medical treatment experience following separation of thoracopagus conjoined twins.</p><p><b>METHOD</b>The clinical manifestations and the medical therapy of a pair of thoracopagus conjoined twins were analyzed. The conjunction of the female twins was from 5 cm above the nipple to the umbilicus. They also suffered from complex congenital heart diseases. At the 17th day of their lives, they were surgically separated. One girl died after the operation, the other survived but experienced heart failure, sepsis and multiple organ dysfunction (including liver, blood and kidney et al). In order to protect or maintain the main organ function, the vital signs and the objective indexes were monitored continually, such as blood routine test, C reactive protein, hepatorenal function, bacterial culture, and galactomannan test, blood gas analysis and chest radiogram.</p><p><b>CONCLUSION</b>It is important to protect the main organ function and prevent or control infection. The early surgical correction of congenital heart disease may contribute to recovery of the children.</p>

Surgical repair of truncus arteriosus in children: early results and long-term outcomes / 中华外科杂志

<p><b>OBJECTIVE</b>To recite early results and long-term outcomes after surgical repair of persistent truncus arteriosus (PTA).</p><p><b>METHODS</b>The clinic data of 54 patients underwent surgical repair for PTA from January 1999 to December 2009 was analyzed retrospectively. There were 36 male and 18 female patients, with a mean age of (9 ± 10) months (range, 1 to 38 months; median, 5 months). Preoperative mechanical ventilation was required in 5 patients. The surgical procedures were closure of ventricular septal defect and re-establishment of continuity between right ventricle and pulmonary artery. The right ventricular outflow tract (RVOT) was reconstructed by direct anastomosis pulmonary artery to right ventriculotomy with anterior wall patch enlargement (28 cases), or by inserting conduits (26 cases). Valvuloplasty were performed in 4 patients with truncal valves moderate to severe insufficiency and aortoplasty in 3 patients with interrupted aortic arch (IAA).</p><p><b>RESULTS</b>There were 3 patients (5.6%) died of pulmonary hypertensive crisis in hospital. The mean duration of ventilation was 6.8 days in 5 patients who were intubated before operation, while the others were 3.6 days. Forty-seven (92.2%) patients were followed-up for mean (6.8 ± 2.5) years (from 2.5 to 11.0 years). There were 2 patients with mild to moderate aortic regurgitation. One patient with aortic arch obstruction underwent balloon dilatation 2 years postoperatively. Among those patients who underwent direct anastomoses, 8 (32.0%) patients had pulmonary branch stenosis at 7 months to 1.5 years postoperatively, 12 (48.0%) patients were freedom from surgical reintervention 5.0 to 11.0 years postoperatively. Among those inserting conduits, 7 patients (31.8%) had conduit stenosis at 2.8 to 7.0 years after operation. Reoperations were performed for RVOT in 15 patients and there was no mortality.</p><p><b>CONCLUSIONS</b>It is difficult to treat the PTA patients with IAA, intra-mural coronary artery or mechanical ventilation support before operation. The technique of direct anastomosis between pulmonary artery and right ventricle offers the potential growth for RVOT, but bilateral pulmonary branch stenosis may be occurred at earlier period of postoperation in some patients.</p>

Judgement of the left ventricle retraining in the rapid two stage-switch operation / 中华外科杂志

<p><b>OBJECTIVES</b>To Summarize the results of left ventricle retraining in rapid two-stage switch operation and to determine the estimating index of left ventricle retraining and the best time of the second stage operation.</p><p><b>METHODS</b>From September 2002 to September 2007, 21 patients underwent rapid two stage switch operation. There were 13 male and 8 female patients, ageing from 29 to 250 d [mean (103 ± 69) d, median 75 d], weighting from 3.5 to 7.0 kg [mean (5.0 ± 1.2) kg, median 5.0 kg]. After pulmonary band, bedside echocardiography was regularly done every other day. Paired t-test was used to analyze the changes of left ventricular end-diastolic dimension (LVDd), left ventricular posterior wall dimensions (LVPWd), diastolic intra-ventricular septal dimensions (IVSd), left ventricular (LV) mass and LV mass indexed for body surface area.</p><p><b>RESULTS</b>The mean interval was (9 ± 5) d. After the left ventricle preparative operation, the left ventricular to right ventricular pressure ratio (pLV/RV) raised from 0.47 ± 0.15 to 0.91 ± 0.20 (P < 0.01). LV mass indexed for body surface area raised from (30 ± 11) g/m(2) to (60 ± 20) g/m(2) (P < 0.01). Extremely significant difference of LV mass existed between pre-arterial switch operation and pre-left ventricle preparative operation, and significant difference existed in LVDd, LVDd(3), LVPWd and IVSd between the two operative timing points.</p><p><b>CONCLUSIONS</b>The left ventricular function of the transposition of the great arteries can be retraining by the left ventricle preparative operation. The interval of left ventricle retraining should be controlled in 7 to 10 d, and the pLV/RV reach 0.65 and the LV mass index over 50 g/m(2) are two important indicators of the second stage operation of arterial switch operation.</p>

Treatment strategies for pediatric patients with primary cardiac tumors / 中华外科杂志

<p><b>OBJECTIVE</b>To analyze the experience of treatment strategies for pediatric patients with primary cardiac tumors.</p><p><b>METHODS</b>The clinical data of 27 patients with primary cardiac tumors which detected by echocardiography from May 1999 to May 2009 was analyzed retrospectively. There were 20 male and 7 female patients, aged from 24 d to 12.6 years. There were 59.2% less than 1 year old at the time of diagnosis. A single tumor were present in 22 cases and multiple in 5 cases. Surgery was performed for 22 patients due to the varied significant symptoms such as arrhythmia, pericardial effusion, swoon and congestive heart failure with dyspnoea. Five patients were discharged hospital without surgical treatment. The surgical approaches were adopted according to tumor location. Complete surgical resection was performed in 14 patients and partial resection in 8 patients. Seven patients were underwent valve reconstruction, 5 involving the mitral valve and 2 involving the tricuspid valve.</p><p><b>RESULTS</b>Histologic examination of the surgically resected tumors showed rhabdomyomas in 8 cases, fibromas in 5 cases, hemangiomas 3 cases, myxomas in 4 cases, fibrosarcoma in 1 case and yolk sac sarcoma in 1 case. Sixteen cases revealed stable haemodynamic status postoperative. Two cases occurred apparent symptoms of low cardiac output and significant arrhythmias, finally recovery after comprehensive treatment of restoration the heart function. There was a total of 4 patients in-hospital death following surgery due to multiorgan system failure. Of the 18 patients who survived after the surgery were followed up from 1 to 10 years, echocardiography showed the residual mass of the tumor with partial resection, rhabdomyoma diminishing in 2 patients and almost vanishing in 1 patient. The residual mass of one fibrosarcoma patient and one hemangioma patient were not increased. Patients with myxomas had no recur or systemic embolisation after the initial surgery. Five nonsurgical patients were followed up from 1 to 3 years, 2 patients without haemodynamic alterations, 1 patients with giant tumor of left ventricular free wall was died of arrhythmia, the other one was alive; the patient of multiple cardiac tumor with low cardiac output was died of heart failure.</p><p><b>CONCLUSIONS</b>Despite the benign histology of most paediatric primary cardiac tumours, there may be significant associated with morbidity and occasional mortality. Therapy strategies should be individualised: surgery is indicated in cases with significant clinical symptoms and close follow-up is necessary for asymptomatic patients. Total resection is not the only therapeutic aim. Most important is the restoration of the normal haemodynamic heart function.</p>

Evaluation on the early hemodynamic changes after cardiac surgery for congenital heart diseases in neonates / 中华儿科杂志

<p><b>OBJECTIVE</b>To accurately evaluate the early hemodynamic status of neonates who undergo complex neonatal cardiac surgery, through monitoring the cardiac index (CI), serum lactate (Lac), mixed venous oxygen saturation (SvO(2)).</p><p><b>METHODS</b>From January to November 2007, haemodynamic data of 80 patients who had open heart surgery for congenital heart disease were analyzed within 48 hours after operation. Of the 80 patients, 47 were neonates, their age ranged from 3 days to 29 days [mean (21.98 + or - 8.15) days] and weight ranged from 2.6 kg to 4.2 kg [mean (3.51 + or - 0.39) kg]. As the control group, 33 young infants at the age of 30 days to 180 days [mean (76.36 + or - 24.79) days] with body weight ranged from 3.1 kg to 6.0 kg [mean (4.59 + or - 0.59) kg] were also enrolled. The value of CI derived from pulse contour and was calculated by using the PiCCO system. Meanwhile, measurements of serum lactate level and SvO(2) were recorded. Serial measurements of the cardiac output were performed for the neonates.</p><p><b>RESULTS</b>CI in survivors of neonates (2.01 + or - 0.35) L/(min x m(2)) was lower than that of the infants (2.26 + or - 0.39) L/(min x m(2)) after cardiac surgery (P < 0.05) at 2 h, 6 h postoperatively. However, urine output remained normal. The value of pulse pressure in neonates was less than that in young infants. Serum lactate level in neonates was significantly higher than that of young infants during cardiac surgical procedures (P < 0.01) at 12 h postoperatively; the SvO(2) was more than 60% postoperatively in survived neonates, there was no significant difference (P > 0.05) in SvO(2) between neonates and young infants during preoperative and postoperative periods. There was a positive correlation between CI and SvO(2). Four neonates and 1 young infant died after surgical treatment, surgical mortality was 8.5% and 3.0%, respectively. The deaths of the neonates were related to the cardiocirculatory function decompensation, unrelieved severe acidosis preoperatively, and the transposition of great artery with coronary artery malformation and longer cardiopulmonary bypass. The patients with significantly high arterial blood lactate levels during the first 6 - 12 hours postoperatively had poor outcome. Lactate levels were higher than 10 mmol/L and SvO(2) less than 50% in neonates who developed multiple organ system failure. One young infant died of sudden arrhythmia after surgical treatment, whose death may be related the surgical procedure itself with pulmonary artery banding and blalock-taussig shunt leading to increased preload and afterload of the heart.</p><p><b>CONCLUSIONS</b>Elevated serum lactate level postoperatively may reflect intraoperative tissue hypoperfusion. Serial blood lactate level measurements may be an accurate predictor of clinical outcomes in children after pediatric open heart surgery. Mixed venous oxygen saturation changes more rapidly than other standard hemodynamic variables. The higher mortality of neonates with congenital heart disease is related to the malformation complexity itself and illness severity.</p>

Application of neurally adjusted ventilatory assist in infants who underwent cardiac surgery for congenital heart disease / 中国当代儿科杂志

<p><b>OBJECTIVE</b>Neurally adjusted ventilatory assist (NAVA) is a new mode of mechanical ventilation that delivers ventilatory assist in proportion to neural effort. This study aimed to compare the hemodynamic safety, oxygenation and gas exchange effects ventilated with NAVA and with pressure support ventilation (PSV) in infants who underwent open-heart surgery.</p><p><b>METHODS</b>Twenty-one infants who underwent open-heart surgery for congenital heart disease (mean age 2.9+/- 2.1 months and mean weight 4.2+/- 1.4 kg) were enrolled. They were ventilated with PSV and NAVA for 60 minutes respectively in a randomized order. The hemodynamic, oxygenation and gas exchange effects produced by the two ventilation modes were compared.</p><p><b>RESULTS</b>Three cases failed to shift to NAVA because of the bilateral diaphragmatic paralysis after operation. In the other 18 cases, there were no significant differences in the heart rate (HR), systolic blood pressure (BPs) and central venous pressure (CVP) in the two ventilation modes. The PaO2/FiO2 (P/F) ratio in NAVA was slightly higher than in PSV, but there was no statistical difference. PaCO2 did not show significant differences in the two modes. The peak inspiratory pressure (PIP) and electrical activity of the diaphragm (EAdi) in NAVA were significantly lower than in PSV. The EAdi signal after extubation was higher in infants who needed reintubation or intervention of noninvasive mechanical ventilation than in those who were extubated successfully (30.0+/- 8.4 microV vs 11.1+/- 3.6 microV; P<0.01).</p><p><b>CONCLUSIONS</b>As the first study of application of NAVA in infants in China, this study shows that NAVA has the same homodynamic effects as PSV. However the PIP for maintaining the same level of PaCO2 in NAVA is significantly lower than that in the traditional PSV. Monitoring the EAdi signal after extubation may show the risks of reintubation or intervention of noninvasive mechanical ventilation.</p>

Use of the lecompte procedure for ventriculoarterial connection abnormalities in infants and children with congenital heart diseases / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The Lecompte (REV) procedure is used to correct abnormal ventriculoarterial connections in patients with congenital heart diseases; it avoids the need for an extracardiac conduit for pulmonary outflow tract reconstruction. The present study aimed to investigate effectiveness and criteria of the REV procedure in children with abnormal ventriculoarterial connections.</p><p><b>METHODS</b>Thirty-eight children (mean age, (2.2 +/- 1.7) years; mean weight, (11.5 +/- 3.8) kg) with abnormal ventriculoarterial connections who had an REV procedure in our hospital from January 1998 to May 2006 were studied. Only 10 patients had the usual anteroposterior relationship of the two great arteries. The infundibular septum between the two semilunar valves was aggressively resected to enlarge it and construct a straighter left ventricular outflow tract and a wide tunnel between the ventricular septal defect (VSD) and the aorta. Eighteen cases had the original REV procedure; 20 had a modified REV procedure.</p><p><b>RESULTS</b>All patients are alive; none developed severe complications. The postoperative right ventricular (RV) to left ventricular (LV) pressure ratio was 0.20-0.45. Five patients had RV dysfunction; 2 patients had a pressure gradient in the RV ventricular outlet of 30.0-34.5 mmHg; 3 cases had a 37.5-47.3 mmHg pressure difference in the RPA. All patients had an RV pressure less than half the systemic pressure. These gradients' magnitudes in all patients were consistent with the post-operative RV to LV pressure ratio (P < 0.05). During the follow-up (mean, (4.2 +/- 0.6) years), 2 patients had an RPA pressure gradient of 24.0-29.3 mmHg which abated to less than 10 mmHg after two years.</p><p><b>CONCLUSIONS</b>The REV procedure provides satisfactory short- to medium-term results. It may be superior to the Rastelli procedure for treating ventriculoarterial connection abnormalities; it allows early, complete anatomic repair and reduces the need for late re-operation, since no extracardiac conduit is needed. Longer follow-up is needed to determine long-term outcomes.</p>

Analysis surgical results of 113 patients with complete transposition of the great arteries / 中华外科杂志

<p><b>OBJECTIVE</b>To review and analysis the surgical results of 113 arteries Switch operations.</p><p><b>METHODS</b>One hundred and thirteen patients had been repaired by arterial Switch operation from January 2001 to December 2005. There were 60 patients with transposition of the great arteries and intact ventricular septum (TGA/IVS), 53 patients with transposition of great arteries and ventricular septal defect (TGA/VSD). The lowest body weight was 2.3 kg, and the youngest operative age was 6 h. The arteries Switch operation was performed underwent deep hypothermic circulation arrest and low-flow perfusion.</p><p><b>RESULTS</b>The total mortality was 9.7%. There were 5 deaths among TGA/IVS (8.3%), 6 deaths among TGA/VSD (11.3%). Following improvement of surgical technique, post-operative management and cardiopulmonary bypass, the operative mortality was decreased from 16.6% to 5.5%.</p><p><b>CONCLUSIONS</b>The main reason for operative mortality was abnormal coronary arteries. The incidence of abnormal coronary arteries was high at TGA/VSD. The surgical results was not infected by the position of great arteries. The low cardiac output was appeared if the ratio of left ventricular pressure and right ventricular pressure less than 0.6.</p>

Single stage corrections of Taussig-Bing anomaly associated with aortic arch obstruction / 中华胸心血管外科杂志

Objective Aortic arch obstruction is a commonly associated malformation in the patients with Taussig-Bing anoma- ly.Herein,we reported our outcomes of single stage corrections in patients with the Taussig-Bing anomaly associated with aortic arch obstruction.Methods Between May,2000 and Dec,2006,12 patients with Taussig-Bing anomaly associated with aortic arch obstr- uction (5 patients associated with interrupted aortic arch) underwent arterial switch operation with baffling of the left ventricle to neo- aorta.The corrections of aortic arch obstruction included extended resection combined with end to end anastomosis to aortic arch or end to side anastomosis to ascending aorta.Results The hospital mortality rate was 25% (3/12).The ventilating time and ICU stay were (7.4?2.1) days and (11.7?4.6) days,respectively.No reoperation because of residual anomalies was required.Conclusion Tanssig-Bing anomaly,especially associated with aortic arch obstruction,is different from transposition of great artery.The opti- mized operative indications,techniques and the managements of aortic arch obstruction are discussed in the article.