Objective To explore the surgical management method and outcome of functional univentricular heart with total anomalous pulmonary vein drainage (UVH-TAPVD). Methods We reviewed the surgical procedures and results for 44 UVH-TAPVD patients in our hospital between the year 2010 and 2016. There were 34 males and 10 females. The age of the patients was 312 (77-4 220) d when they accepted the first surgical treatment. Results There were 8 deaths in stage Ⅰ palliation, 1 death in stage Ⅱ palliation and 5 deaths during the follow-up. The overall survival rate was 68.2% (30/44). Glenn operation was undertaken in 34 patients with 5 deaths. Fontan operation was undertaken in 9 patients with 2 deaths. Conclusion Surgical strategies for UVH-TAPVD should be planned according to different anatomical and pathophysiological conditions in different patients. Staged palliations can reduce mortality and morbidity. But pulmonary venous obstruction and heterotaxy syndrome are still risk factors for these patients.
Objective To summarize the surgical experience of supracardiac total anomalous pulmonary venous connection(S-TAPVC) and study the surgical technique and outcomes for S -TAPVC. Methods Eightysix patients with S-TAPVC underwent the surgical repair from May 1985 to December 2007. There were 49 males and 37 females. The patients aged from 7 months to 35 years (mean 9.6 years) and weighed from 4.9 kg to 68.0 kg (mean 23.8 kg). The patients were divided into three groups by the approach for the anastomosis. There were 20 patients in groupⅠthrough the right atrium incision, 49 patients in group Ⅱ through the right and left atrium incisions and 17 patients in group Ⅲ through the top of the left atrium incision. The interrupt continuous anastomosis between the common pulmonary venous and the left atrium was used in all patients. The enlarged atrial septal defect(ASD) was repaired with autopericardium. The vertical vein was ligated if the postoperative left atrial pressure was less than 15 mm Hg. But the vertical vein was opened or just partialy ligated if the postoperative left atrial pressure was more than 15 mm Hg. Results There was no early operative death. The postoperative left atrial pressure in three groups were 9.3±3.2 mm Hg, 9.9±2.9 mm Hg and 11.6±3.8 mm Hg, respectively. The cases with open or just partly ligated vertical vein in three groups were 0 case （0%）, 7 cases (14.3%) and 2 cases (11.8%), respectively. The cases of arrhythmia in three groups were 5 cases （25.0％）, 15 cases （30.6%）and 1 case （5.9%）. The severely low cardiac output syndrome occurred in 2 patients and reoperation for bleeding in 2 patients. The morbidity of arrhythmia in group Ⅲ was less than in group Ⅱ（P=0.042）. Conclusion The outcome of surgical repair for S -TAPVC is satisfactory. The anastomosis through the top of the left atrium incision has low occurrence of arrhythmia. The anastomosis through the right and left atrium incision is easy to expose and to perform surgery, especial for old children and adult patients.
ObjectiveTo analyze the surgical results of patients with supracardiac total anomalous pulmonary venous connection (TAPVC) in a single pediatric cardiac center.MethodsA retrospective study was conducted on 98 pediatric patients with supracardiac TAPVC receiving surgical repair from 2014 to 2019 in our center. There were 64 males and 34 females with a median surgical age of 3.0 (1.5, 7.0) months and a median weight of 5.0 (4.0, 6.0) kg. Twenty-three (23.5%) patients had preoperative pulmonary vein obstruction. Ninety-two (93.9%) patients received conventional surgical repair, while six (6.1%) patients were treated with the sutureless technique. The Cox regression model was used to analyze the data.ResultsThe median follow-up time was 26.50 (5.75, 44.25) months. There were 9 (9.2%) deaths. Lower weight at the time of repair (P=0.013) and prolonged cardiopulmonary bypass time (P=0.007) were associated with mortality. Postoperative pulmonary vein obstruction was observed in 8 (8.2%) patients. Associated risk factors for postoperative pulmonary vein obstruction included lower weight at the time of repair (P=0.042) and prolonged cardiopulmonary bypass time (P=0.002).ConclusionSurgical repair of supracardiac TAPVC has achieved satisfactory results in our center. Risk factors such as lower weight at the time of repair and prolonged cardiopulmonary bypass time are associated with a poor prognosis.
Objective To summarize the experience of the superior vena cava and pulmonary connection surgery for functional single ventricle (SV) with total anomalous pulmonary venous (TAPVC). Methods We retrospectively analyzed the clinical data of 10 patients with SV and TAPVC in our hospital from January 2012 through June 2014. There were 7 males and 3 females at average age of 90.33±86.53 months. The 10 patients were with right atrial isomerism, 9 with heterotary and asplenia syndrome. Five patients were anatomic single ventricle and others were with functional uni-ventricle. Nine patients were with supracardiac pattern TAPVC and one was with intracardiac TAPVC. All patients were operated unilateral or bilateral bidirectional Glenn procedure with TAPVC correction. Results The arterial oxygen saturation (SaO2) increased prominently after operation (86%±6% vs. 79%±6%, P<0.01). There were 3 patients with low cardiac output syndrome, one patient with severe arrhythmia, 4 patients with serious pleural effusion, 4 patients with hospital-acquired infection, and 3 patients with central nervous system complications (epilepsy or hemiplegia). One died because of hemorrhage and pulmonary thrombosis, and the other died of hypoxemia and mutiple organ dysfunction syndrome (MODS). Conclusion Glenn is one of palliated procedure choice for SV/TAPVC patients. The indication for surgery and perioperative management individually is crucial.
Objective To analyze risk factors for pulmonary vein obstruction (PVO) after intracardiac total anomalous pulmonary venous connection (TAPVC) repair. Methods A retrospective analysis of clinical data of the patients with intracardiac TAPVC who underwent surgery at our center from April 2009 to April 2019 was conducted. Kaplan-Meier curves were used to assess the risk of postoperative PVO. Logistic regression analysis was used to identify relevant risk factor for postoperative PVO. ResultsWe finally included 169 patients. The median age at surgery was 89.0 (41.5, 195.0) days, and the median weight at surgery was 4.8 (3.8, 6.0) kg. The preoperative PVO rate was 18.3% (31/169). Post-repair PVO occurred in 8.9% (15/169) of the patients. The atresia of common pulmonary and subtype of coronary sinus/right atrium did not significantly affect the risk of PVO (P=0.053, P=0.330). Relevant risk factors included preoperative PVO (P<0.001) and the ratio of left ventricular end-systolic diameter to right ventricular diameter (P=0.025). ConclusionSurgical repair of intracardiac TAPVC has achieved satisfactory results in our center, but the long-term risk of obstruction should not be underestimated. An increased ratio of left ventricular end-systolic diameter to right ventricular diameter and preoperative PVO are associated with post-repair PVO.
ObjectivePulmonary vein banding was used to establish a piglet model of pulmonary vein stenosis. We investigated the pathomorphological alterations of pulmonary veins in the model and compared it with the vascular tissue of recurrent stenosis after total anomalous pulmonary venous connection (TAPVC).MethodsTen pigs of 6 weeks old were selected and randomly divided into 2 groups: 5 in a sham operation group and 5 in a pulmonary vein banding group. The operation had two stages, in which thoracotomies through intercostal space were done respectively on both sides. Biocompatible materials were applied around the pulmonary veins in the experimental group. The same method was used in the sham group. But the pulmonary veins were not banded. Six weeks after the operation, the pulmonary veins of the animals were harvested for hematoxylin-eosin staining and immunofluorescence staining to observe the pathological alterations of pulmonary veins. The proliferative tissues of patients with recurrent stenosis after TAPVC repair were collected and observed by hematoxylin-eosin staining and immunofluorescence staining.ResultsBoth the sham operation group and the pulmonary vein banding group survived. But the pulmonary vein banding group had obvious clinical manifestations of pulmonary venous stenosis. Compared with the sham group, the pulmonary vein banding group showed intimal hyperplasia, decreased expression of endothelial marker and increased expression of mesenchymal markers, and co-expression of endothelial and mesenchymal markers in intimal cells. Human pathology also showed intimal hyperplasia and co-expression of endothelial and mesenchymal markers in intimal cells.ConclusionThe surgical pulmonary vein stenosis in piglets shows intimal hyperplasia and myofibroblasts, which was consistent with clinical pathology.