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>

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>

Arterial switch operation for transposition of the great arteries and double outlet of right ventricle with subpulmonary ventricular septum defect / 中华外科杂志

<p><b>OBJECTIVE</b>To summarize the clinical experience on the arterial switch operation for complex congenital heart disease in recent 3 years in our hospital.</p><p><b>METHODS</b>Sixty-one patients with complex congenital heart disease received by arterial switch operation from Jan 2000 to May 2003. There were 26 patients with transposition of the great arteries and intact ventricular septum (TGA-IVS), 19 patients with transposition of the great arteries and ventricular septal defect (TGA-VSD) and 16 patients with double outlet of right ventricle with subpulmonary ventricular septum defect (Taussig-Bing).</p><p><b>RESULTS</b>There were 2 death among TGA-IVS cases, 4 deaths among TGA-VSD cases and 4 deaths among Taussig-Bing cases. The total operative mortality was 16%. All patients were followed up 3 months to 3 years. In all patients, the cyanosis disappeared and the physical activities increased obviously. One patients with Taussig-Bing keep moderate mitral valve reflux unchanged, 2 patients with TGA had pulmonary and aortic supra-valve stenosis with 40 mmHg pressure gradient. One patient with subpulmonary stenosis and residual VSD was re-operated 3 months later. All survivor had good heart function.</p><p><b>CONCLUSIONS</b>The effect of arterial switch operation on the treatment of TGA was well accepted in this study. The procedure used in treatment on Taussig-Bing can prevent pulmonary obstruction and avoid the complication of left ventricular outflow tract obstruction caused by intraventricular repair.</p>