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Objective:To discuss the treatment strategies and experiences of total anomalous pulmonary venous connection (TAPVC) and study the risk factors for postoperative pulmonary venous obstruction (PVO).Methods:Clinical data of 125 children with TAPVC in the Children Heart Center of Fuwai Central China Cardiovascular Hospital from January 2018 to December 2020 were included in this study and analyzed retrospectively.Of the 125 cases, 116 were treated surgically.They were divided into 2 groups according to whether PVO appeared after repair of TAPVC.The t-test or chi- square test was used to compare the clinical data of the 2 groups, and Logistic regression analysis was adopted to analyze the risk factors for postoperative PVO. Results:Among the 116 patients undergoing surgery, including 73 males (62.9%) and 43 females (37.1%), and according to Darling′s classification, there were 58 cases (50.0%) of supracardiac, 35 cases(30.2%)of intracardiac, 15 cases(12.9%) of infracardiac and 8 cases (6.9%) of mixed.The median age and body weight during surgery were 3.0 (1.2, 7.0) months and 5.0 (4.0, 6.8) kg.There were 13 deaths (11.2%), and recurrent PVO was observed in 18 patients (15.9%). Statistically significant diffe-rences were observed in terms of Darling′s classification (supracardiac 27.8% vs.56.6%, intracardiac 27.8% vs.31.6%, infracardiac 27.8% vs.10.5%, mixed 16.6% vs.5.3%, χ2=8.571, P=0.036) of PVO group and non-PVO group after operation preoperative PVO (83.3% vs.21.1%, χ2=25.293, P<0.01), with confluence (55.6% vs.81.1%, χ2=6.049, P=0.014), preoperative critical state (83.3% vs.31.6%, χ2=12.938, P<0.01), cardiopulmonary bypass time[ (128.800±47.254) min vs.(106.700±37.288) min, t=-2.094, P=0.039 ], delayed thoracic closure(27.8% vs.5.3%, χ2=6.227, P=0.013), and anastomotic velocity at 1 day after operation[1.0(0.9, 1.4) m/s vs.0.9(0.8, 1.1) m/s, Z=-2.004, P=0.045], anastomotic velocity at 1 week after operation[1.4 (1.3, 1.8) m/s vs.0.9 (0.7, 1.1) m/s, Z=-4.446, P<0.001], and mechanical ventilation time[121.0 (76.8, 246.9) h vs.91.5 (60.4, 135.9) h, Z=-1.989, P=0.047]. All of these data were included in the Logistic regression analysis.The results showed that preoperative PVO ( OR=797.179, 95% CI: 8.074-78 712.270, P=0.004) and increased anastomotic flow velocity at 1 week after operation ( OR=11 848.376, 95% CI: 23.746-5 912 017.803, P=0.003) were associated with postoperative PVO. Conclusions:Surgical correction in patients with TAPVC with a biventricular anatomy in this center is satisfactory.Preoperative PVO and early anastomotic flow velocity increase after operation are the high risk factors for postoperative PVO.
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@#Objective To analyze the surgical results of patients with supracardiac total anomalous pulmonary venous connection (TAPVC) in a single pediatric cardiac center. Methods A 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. Results The 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). Conclusion Surgical 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.
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Objective:To analyze the risk factors for postoperative pulmonary venous obstruction after correction of total anomalous pulmonary venous connection(TAPVC).Methods:We retrospectively reviewed all patients undergoing operative repair of TAPVC in our institution from December 2013 to January 2018. Patients with functionally univentricular circulations or atrial isomerism were excluded. Patients were divided into two groups according to whether there was pulmonary vein obstruction. The clinical variables of the two groups were compared. Variables for the multivariable analysis were chosen if there was statistical significance on univariable analysis.Results:145 patients were included, 91(63%) males, aged 4(2, 8)months and weight 5.5(4.5, 7.5)kg. Mean follow-up interval was(51±23) months. Postoperative obstruction developed in 27 patients(18.6%). The differences of anatomic type[supracardiac 18(67%) vs.59(50%), cardiac 4(15%) vs. 50(42%), infracardiac 3(11%) vs. 1(1%), mixed 2(7%) vs. 8(7%), P=0.003], preoperative obstruction[yes 19(70%) vs. 37(31%), no 8(30%) vs. 81(69%), P<0.001], associated cardiac lesions[yes 13(48%) vs. 27(23%), no 14(52%) vs. 91(77%), P=0.008] and bypass time[109(89, 129)min vs. 88(70, 110)min, P=0.002] between two groups were statistical significant. A multivariable model showed preoperative obstruction( P<0.001) and bypass time( P=0.009) were associated with postoperative obstruction. Conclusion:The incidence of pulmonary vein obstruction after correction of TAPVC was still high. If there was preoperative obstruction, or the bypass time was too long during operation, the surveillance of pulmonary vein obstruction should be strengthened after operation.
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@#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.
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We report four cases of unilateral pulmonary vein atresia without associated congenital intracardiac anomalies to illustrate a part of the clinical and radiological characteristics and its diagnosis. Pulmonary vein atresia was in right side in all cases. Narrowing of contralateral pulmonary vein and pure red cell anemia were combined in one cases. In four cases, initial presentations were hemoptysis and lower respiratory tract infection. The simple chest X-ray revealed loss of volume, increased reticular interstitial markings of the involved lung and the shift of mediastinal structures to the involved side, and hilar prominency in the contralateral side. Lung perfusion scan revealed no visualization of involved lung. Cardiac Catheterization revealed pulmonary artery hypertension 2/4 and pulmonary artery wedge pressure 1/2. On pulmonary angiogram, nonvisualiaztion of pulmonary vein in the involved side in 3: partial visualization of pulmonary vein not connected to atrium in 1: dysmorphic pulmonary artery on the contralateral side in 1. Right pneumonectomy was done without specific problems in two cases. In conclusion, Unilateral pulmonary vein atresia should be suggested when the clinical and radiological characteristics are present.