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.
【摘要】 目的 探讨正常成人肺静脉开口径线（pulmonary vein ostia，PVO）的测量方法，并获得我院PVO径线值的95%参考值范围。 方法 2005年6月-2006年1月收集无明显心肺疾病的正常成人236例，对其进行多层螺旋CT扫描，利用斜冠面重建和三维标定的方法测量PVO径线。 结果 四条PVO径线的95%参考值范围如下：右上肺静脉长径线为男性1.49~2.63 cm，女性1.31~2.37 cm，短径线为男性0.87~2.05 cm，女性0.91~1.81 cm；右下肺静脉长径线为男性1.12~2.06 cm，女性1.05~1.95 cm，短径线为男性0.77~1.83 cm，女性0.79~1.69 cm；左上肺静脉长径线为男性1.18~2.20 cm，女性1.09~2.11 cm，短径线为男性0.82~1.72 cm，女性为0.80~1.62 cm；左下肺静脉长径线为男性0.96~1.98 cm，女性1.03~1.81 cm，短径线为男性0.56~1.26 cm，女性0.51~1.33 cm。除左下肺静脉短径男女差异无统计学意义外，其余径线值男性均大于女性。 结论 采用CT三维重建像改进测量方法测量PVO径线值既简便又准确。【Abstract】 Objective To measure and calculate the diameters of pulmonary vein ostia (PVO) by multi-slice spiral computed tomography (MSCT) in healthy people. Methods From June 2005 to January 2006, 236 healthy people were adopted to undergo MSCT. Their diameters of PVO were measured and calculated by oblique coronal planes reconstruction and 3D position-setting. Results Right superior vein: the 95% reference ranges of the long diameters were 1.49-2.63 cm in the male and 1.31-2.37 cm in the female;the ranges of the short diameters were 0.87-2.05 cm in the male and 0.91-1.81 cm in the female. Right inferior vein: the ranges of the long diameters were 1.12-2.06 cm in the male and 1.05-1.95 cm in the female;the ranges of the short diameters were 0.77-1.83 cm in the male and 0.79-1.69 cm in the female. Left superior vein: the ranges of the long diameters were 1.18-2.20 cm in the male and 1.09-2.11 cm in the female;the ranges of the short diameters were 0.82-1.72 cm in the male and 0.80-1.62 cm in the female. Left inferior vein: the ranges of the long diameters were 0.96-1.98 cm in the male and 1.03-1.81 cm in the female;the ranges of the short diameters were 0.56-1.26 cm in the male and 0.51-1.33 cm in the female. The diameters in the male were all longer than those in the female, except the short diameter of left inferior vein. Conclusions It is a convenient and accurate method to measure the diameters of PVO by blique coronal planes reconstruction and 3D position-setting.
ObjectiveTo review our experience of reoperations for pulmonary venous stenosis (PVS) after total anomalous pulmonary venous connection (TAPVC) repair for the past decade in Fuwai Hospital.MethodsNine patients underwent reoperation for PVS between 2009 and 2019 in Fuwai Hospital, including 4 males and 5 females with an average age of 5.10±5.00 years. The patients were divided into a sutureless group (n=3) and a non-sutureless group (n=6). Clinical data were reviewed and analyzed.ResultsFor primary TAPVC type, 4 patients were supracardiac, 2 patients were cardiac, 1 patient was infracardiac, and 2 patients were mixed-type anomaly. The median cardiopulmonary bypass time was 95 (63, 208) min, aortic clamping time was 58 (30, 110) min, ICU stay was 24 (24, 2 136) h. Early hospital death occured in 1 (11.1%) patient. One (11.1%) patient with single ventricle physiology had hospital comorbidity, who underwent hemofitration therapy. The follow-up time was 11.9 (2.2, 18.0) months, during which 1 patient died of restenosis of pulmonary vein and another patient died of stroke. No statistically significant difference was found between the sutureless group and non-sutureless group in postoperative or follow-up results (P>0.05). ConclusionSurgery is effective for treatment of PVS after repair of TAPVC, yet with a realatively high morbidity and mortality. The advantage of sutureless repair over conventional repair for this particular group of patients is yet to be verified.
Objective To summarize the experience of the surgical treatment of total anomalous pulmonary venous connection (TAPVC) in 31 cases，so as to elevate the operative effect. Methods The total corrected operation under moderate hypothermic cardiopulmonary bypass were performed in 31 cases with TAPVC. Site of drainage was supracardiac (n=16),cardiac(n=13)and mixed(n=2). Other accompanying congenital cardiac malformations were diagnosed such as atrial septal defect(n=31), patent ductus arteriosus(n=4), pulmonic stenosis(n=1), mitral regurgitation(n=1), tricuspid regurgitation (n=15)，etc. Results No hospital death was observed in the early period of operation (30 d). Complication occurred in 8 cases (25.8%), such as paroxysm of nodal rhythm(n=1), Ⅰ° atrial ventricular block(n=1), frequently atrial premature beats and paroxysm of auricular tachycardia(n=1),pulmonary infection(n=2),atelectasis(n=1), pneumatothorax (n=1), left diaphragmatic paralysis and pulmonary infection with tracheal reintubation (n=1),etc. All of them discharged after active therapy. All of 31 cases was followed up, followup time was 2.8±1.5 years. 30 cases were living (96.7%) and 1 case died of heart failure 8 months after operation. 2 cases had auricular arrhythmia with a bad medical effect. 28 cases(93.3%) had normal heart function and they could work and live normally after discharged. Conclusion Surgical procedure should be based on individual abnormality for TAPVC.Surgeons should take care not only of the stenosis of anastomotic stoma,but also of the arrhythmia after operation.Satisfied correction of the abnormality is important and the curative effect of those cases is approving.
ObjectiveTo investigate the risk factors for prolonged postoperative mechanical ventilation patients with total anomalous pulmonary venous connection (TAPVC). MethodsWe retrospectively analyzed the clinical data of 97 survived TAPVC patients in our hospital between June 2011 and December 2013. There were 55 males and 42 females with age of 4.4 (2, 12) months. The patients ventilated longer than mean time were as a prolonged ventilated group (n=50) and the others as a normal group (n=47). Perioperative variables between the two groups were compared and selected, then put into logistic regression analysis. ResultsFor the 97 survived patients, the mean ventilation time is 49 (25, 90) hours. Age, weight, pre-operative left ventricular end-diastolic dimension, atrial septal defect (ASD) caliber, inotropic drug dosage, postoperative left ventricular end-diastolic dimension, maximum pulmonary venous velocity (P < 0.01), and cardio-pulmonary bypass (CPB) time (P < 0.05) were statistically different between the two groups. In logistic regress analysis, age (OR=0.804 with 95%CI 0.71 to 0.91) and maximum pulmonary venous velocity (OR=1.016 with 95%CI 1.00 to 1.03) were risk factors for prolonged postoperative mechanical ventilation. ConclusionAge and maximum pulmonary venous velocity are the risk factors associated with prolonged postoperative mechanical ventilation in patients with TAPVC.
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.
ObjectiveTo compare the outcomes of sutureless technique and conventional technique in the surgical repair for infracardiac total anomalous pulmonary venous connection (TAPVC).MethodsThe clinical data of 46 consecutive patients with infracardiac TAPVC undergoing surgical repair in our hospital between June 2014 and April 2019 were retrospectively analyzed. Patients with combined congenital cardiac anomalies such as single ventricle and tetralogy of Fallot were excluded. Patients were divided into a conventional technique group and a sutureless technique group according to the surgical techniques. There were 35 patients in the conventional technique group, including 28 males (80.0%) and 7 females (20.0%) with a median age of 21 (8, 42) d and a median weight of 3.6 (3.0, 4.0) kg. There were 11 patients in the sutureless technique group, including 8 males (72.7%) and 3 females (27.3%) with a median age of 14 (6, 22) d and a median weight of 3.5 (2.9, 3.6) kg. The curative effect of the two groups was compared.ResultsThere were 5 deaths (10.9%) in the conventional technique group, including 4 in-hospital deaths (8.7%) and 1 late death (2.2%). Overall mortality of the conventional technique group (14.3%, 5/35) was higher than that of the sutureless technique group (0.0%, 0/11), although the difference was not statistically significant (P=0.317). Cox regression analysis showed that sex (P=0.042), age at repair (P=0.028), cardiopulmonary bypass time (P=0.007), aortic cross-clamping time (P=0.018) and duration of ventilation (P=0.042) were risk factors for postoperative mortality. The median follow-up was 18.00 (5.00, 37.75) months. Postoperative pulmonary venous obstruction occurred in 22 patients of the conventional technique group, which was significantly more than that of the sutureless technique group (P=0.000).ConclusionFor infracardiac TAPVC, sutureless technique can reduce the incidence of postoperative pulmonary venous obstruction compared with conventional technique.
ObjectiveTo introduce a modified sutureless technique and its surgical results in the treatment of total anomalous pulmonary venous connection (TAPVC).MethodsClinical data of 11 patients with TAPVC who underwent the modified sutureless technique treatment from 2014 to 2019 in our center were retrospectively analyzed, including 4 males and 7 females. The median surgical age was 1.4 (0.3, 27.0) months. The median weight was 4.3 (3.5, 8.5) kg.Six (54.5%) patients were of supracardiac subtype, and five (45.5%) patients were of infracardiac subtype. Five (45.5%) patients had preoperative severe pulmonary hypertension, and three (27.3%) patients had preoperative pulmonary vein obstruction. The surgical results were compared with those of 10 patients treated with conventional surgical technique.ResultsThe median follow-up was 12 (range, 1-65) months. During the follow-up, no death or postoperative pulmonary vein obstruction occurred in the modified sutureless technique group. The perioperative data and relief of re-obstruction were superior in the modified sutureless technique group, but the difference was not statistically significant (P>0.05). The postoperative survival of the the modified sutureless technique group was better than that of the traditional surgery group (P=0.049).ConclusionThe modified sutureless technique which includes partial suture and then incising, and eversion of pulmonary vein incision, is a safe and reliable method for the treatment of TAPVC with satisfactory short-term results.
Objective To analyze the sutureless technique use in the surgical repair for total anomalous pulmonary venous connection in neonates. Methods Between September 2002 and December 2015, 71 consecutive neonates with supracardiac or infracardiac type total anomalous pulmonary venous connection who underwent surgical repair in our institute were included in this study. There were 57 males and 14 females at median age of 8 (1, 29) d. And the median body weight was 3.3 (2.1, 4.7) kg. There were 45 patients (63.4%) with supracardiac, 26 patients (36.6%) with infracardiac. Patients were divided into two groups: a conventional technique group (29 patients) and a sutureless technique group (42 patients). To control for potential differences in the characteristics of patients between the sutureless technique group and the conventional technique group, the method of propensity-score matching was used. Results Basic characteristics of patients after propensity-score were not different.There were 11 operative deaths (15.5%), 7 late deaths (96.%), total 18 deaths (25.4%). The mortality was 58.6% (17/29) in the conventional technique group, 2.4% (1/42) in the sutureless technique group (P=0.000). Kaplan-Meier survival curve showed a difference in mortality between the two groups (P=0.005). The patients were followed up for 25.5 (1.0-13.0) months.Postoperative anastomotic or pulmonary vein stenosis occurred in 12 patients, 8 of them died at a higher mortality than that of the patients with smooth anastomosis [66.7% (8/12) vs. 16.9% (10/59), P=0.001]. Conclusion The patients who have postoperative anastomotic or pulmonary vein stenosis have higher mortality. Compared with conventional technique, sutureless technique can dramatically decrease the incidence of postoperative anastomotic or pulmonary vein stenosis and the mortality of surgical repair for total anomalous pulmonary venous connection in neonates.