[Anatomic variations and corresponding surgical techniques of total anomalous pulmonary venous connection].

OBJECTIVE: To describe the anatomic variations of total anomalous pulmonary venous connection (TAPVC) and its corresponding surgical techniques. METHODS: A total of 143 TAPVC subjects were hospitalized from April 1981 to July 2010. Those patients with other complex congenital heart diseases, such as transposition of great artery and single ventricle, were excluded. A pathological diagnosis was made by echocardiography, magnetic resonance imaging, computed tomography, catheterization and intra-operative findings. The specific types of TAPVC were as follows:supra-cardiac (49.7%, 71/143), cardiac (40.6%, 58/143), infra-cardiac (4.2%, 6/143) and mixed (5.6%, 8/143). The subtypes were classified by the pathway of common confluence, distribution of pulmonary vein and their orifice site. The techniques of surgical repairs included modified Warden procedure and pulmonary vein transplantation. RESULTS: The patients with supra-cardiac type were further divided into 4 subtypes according to the course of vertical veins and their orifice site: right and left veins forming a common confluence, then draining into vertical and innominate veins (n = 65); common confluence of pulmonary vein drainage into superior vena cava through a short vertical vein at the right pulmonary hilus (n = 3); right and left pulmonary veins separately draining into superior vena cava (n = 2); common confluence draining into innominate vein through a right path beside trachea (n = 1). Cardiac types were further divided into 3 subtypes: coronary sinus (n = 20), right atrium (n = 37) and right atrium & sinus (n = 1). Infra-cardiac type had no subtype. Mixed type was more complex and it was further divided into 3 subtypes: bilateral & symmetrical connection (right 2 + left 2, n = 5); bilateral & asymmetrical connection (3 + 1, n = 3). Surgical repairs were performed on 135 patients. The surgical mortality of TAPVC was 5.9% (8/135). And there was no late death. The major causes of death were pulmonary infection and low cardiac output syndrome. CONCLUSION: A detailed classification of TAPVC is of great importance for surgical approaches and methodological designs. And an individualized surgical plan yields excellent patient outcomes.

Experience in minimally invasive Nuss operation for 406 children with pectus excavatum.

BACKGROUND: This study was to investigate the advantages of thoracoscopy-assisted minimally invasive Nuss operation for the treatment of pectus excavatum (PE) in children. METHODS: A total of 406 patients with PE (female: 93; male: 313) with an average age of 6.8 years (range: 3.5-17.5 years) were included in this study. Associated diseases included congenital heart disease in 9 patients and congenital pulmonary cyst in 2. The Haller index of the patients ranged from 3.35 to 7.23, with an average of 5.17±1.64. Minimally invasive Nuss operation was performed for all the patients. RESULTS: The operations were performed successfully and no operative mortality occurred. The average blood loss during the operation was less than 10 mL and the operating time ranged from 30 to 85 minutes with an average of 45 minutes. The length of hospital stay ranged from 5 to 9 days with an average of 7 days. Struts were implanted in 12 (3.0%) of the 406 patients. Injury of the pericardium occurred in 1 patient during the operation. Early post-operative complications occurred in 9 patients with pneumothorax and 6 patients with pleural effusion, which were cured by puncture or drainage. Poor wound healing occurred in 4 patients (1.0%) and was cured by nutritional support. During a 3-month to 6-year follow-up, 2 patients had scoliosis and 3 patients had displacement of the strut, which was cured by a second Nuss operation. Allergy occurred in 2 patients: the symptoms were improved in 1 patient after conservative treatment, but the strut was removed in advance due to allergy in the other patient. Totally 154 patients (40.0%) underwent operation for strut removal. Excellent repair results were achieved in 387 (95.3%) patients, good repair results in 12 (3.0%), and fair results in 7 (1.7%). CONCLUSIONS: Thoracoscopy-assisted Nuss operation has many advantages including small and masked incision, short operative time, minimal blood loss, fast recovery, less trauma, and satisfactory outcomes of repair. Nuss is a safe and reliable technique for repair of PE.

[The relationship between postoperative complication and pathological features of 324 patients with neuronal intestinal malformations].

OBJECTIVE: To investigate the relationship between the morphological features of different types of neuronal intestinal malformations (NIM) and their postoperative complications. METHODS: The data of morphological and clinical features of 324 cases with NIM were analyzed retrospectively. RESULTS: In all 324 patients, 210 cases were Hirschsprung's disease (HD), 38 intestinal neuronal dysplasia (IND), 45 mixed HD/IND, 8 hypoganglionosis, 22 combined HD/hypoganglionosis and 1 immaturity of ganglion cells. The percentages of normal neuron in bowel of different NIM were 88.1%, 24.4%, 18.4%, 4/8, 27.7% and 0/1 in HD, HD/IND, IND, hypoganglionosis, HD/hypoganglionosis and immaturity of ganglion cells respectively. There were totally 46 cases complicated with recurrent postoperative enterocolitis (EC). Incidence of recurrent postoperative EC in HD patients was 6.7% while in IND/HD and IND patients was 35.6% and 28.9%, respectively. Incidences of EC in cases with the residual IND margins and with the normal margins were 38.2% and 8.7%, respectively. Incidence of EC in cases with transanal endorectal pull-through procedure and with transabdominal procedure was 18.0% and 8.3%, respectively. Nine cases underwent another procedure because of severe persistent constipation or EC after operation, including 4 cases HD/IND, 1 case IND, 3 cases HD and 1 case HD/hypoganglionosis. CONCLUSIONS: Neuron distribution is inconsistent with pathology of NIM. Postoperative EC are rare in the patients only with isolated HD. Furthermore, margins with residual IND and transanal endorectal pull-through procedure are risk factors to recurrent EC. However, the extension of excision about IND is uncertain and need further study.

Mutation of RET proto-oncogene in Hirschsprung's disease and intestinal neuronal dysplasia.

AIM: To investigate the genetic relationship between Hirschsprung's disease (HD) and intestinal neuronal dysplasia (IND) in Chinese population. METHODS: Peripheral blood samples were obtained from 30 HD patients, 20 IND patients, 18 HD/IND combined patients and 20 normal individuals as control. Genomic DNA was extracted according to standard procedure. Exons 11,13,15,17 of RET proto-oncogene were amplified by polymerase chain reaction (PCR). The mutations of RET proto-oncogene were analyzed by single strand conformational polymorphism (SSCP) and sequencing of the positive amplified products was performed. RESULTS: Eight germline sequence variants were detected. In HD patients, 2 missense mutations in exon 11 at nucleotide 15165 G-->A (G667S), 2 frameshift mutations in exon 13 at nucleotide 18974 (18974insG), 1 missense mutation in exon 13 at nucleotide 18919 A-->G (K756E) and 1 silent mutation in exon 15 at nucleotide 20692 G-->A(Q916Q) were detected. In HD/IND combined patients, 1 missense mutation in exon 11 at nucleotide 15165 G-->A and 1 silent mutation in exon 13 at nucleotide 18888 T-->G (L745L) were detected. No mutation was found in IND patients and controls. CONCLUSION: Mutation of RET proto-oncogene is involved in the etiopathogenesis of HD. The frequency of RET proto-oncogene mutation is quite different between IND and HD in Chinese population. IND is a distinct clinical entity genetically different from HD.

[The risk factors of failed extubation after cardiac surgery in infants].

OBJECTIVE: To investigate the risk factors of failed extubation (FE) after cardiac surgery in infants. METHODS: 227 infants (< 1 year of age) who underwent congenital heart surgery in the period between January 2001 and December 2002 were included in this study. Logistic regression analysis was used to assess the risk factors of failed extubation. Odds Ratio was used to suppose the degree of relationship between FE and risk factors. RESULTS: Out of the 227 infants undergoing congenital heart operations, 30 (13.22%) cases failed at the extubation. Risk factors for failed extubation included postoperative duration of ventilation (EOR = 12.0; 95% CI = 4.04 - 35.71; P = 0.000 9), postoperative pneumonia (EOR = 5.33; 95% CI = 1.81 - 15.68; P = 0.002) and preoperative pulmonary hypertension (EOR = 2.80; 95% CI = 1.21 - 10.45; P = 0.041). Postoperative pneumonia and preoperative pulmonary hypertension were the 2 independent risk factors for failed extubation (P < 0.05). CONCLUSIONS: Postoperative pneumonia and preoperative pulmonary hypertension are major risk factors for failed extubation after congenital heart surgery in infants. To prevent and to treat postoperative pneumonia and pulmonary hypertensive crises will be beneficial to the successful extubation.

[Postoperative hemodynamic parameter follow-up in children with severe pulmonary resistance hypertension due to ventricular septal detect].

OBJECTIVE: To study the relationship between the age at operation and prognosis in children with severe pulmonary hypertension (PH) due to ventricular septal defect (VSD). METHODS: Forty children with severe PH (increased total pulmonary circulation resistance)due to VSD were divided into two groups based on age at operation(Group I aged less than 2 years and group II more than 2 years). The hemodynamic parameter follow-up was measured by cardiac catheterization at presurgery, one week after surgery and 5-7 years postoperatively. RESULTS: The ratio of pulmonary arterial pressure and systemic arterial pressure (pp/ps),pulmonary resistance and systemic resistance (R(p)/R(s)), and pulmonary vascular resistance (PVR) and small pulmonary arterial resistance (PAR) were significantly different in two groups (P<0.01). During follow-up in the group less than 2 years, all the hemodynamic parameters were at normal level, while in the group more than 2 years, only p(p)/p(s) and R(p)/R(s) were close to normal level. The pulmonary arterial resistance was still abnormal. CONCLUSION: An early operation may be the only way to gain optimal long term result of surgery and decrease the incidence of pulmonary vascular disease in children with PH due to VSD.