Abstract： Objective To analyze the manifestation, treatment，outcome and potentially predisposing risk factors of perioperative thromboembolism after cavo-pulmonary connection, and to assess the effects of our prophylactic antithrombotic strategy. Methods Clinical data of 264 cases of bidirectional Glenn and 224 cases of total cavopulmonary connection （TCPC） operations undertaken from September 2005 to December 2010 in Fu wai hospital were retrospectively analyzed. Perioperative thromboembolic complications occurred in 9 patients. 5 of them were after bidirectional Glenn, 4 were after TCPC. Most of the 9 patients had at least one thrombi-related risk factor. The routine prophylactic antithrombotic therapy after these operations were intravenous infusion of heparin 10 U/（kg·h） initially, followed with oral aspirin 1-3 mg/（kg·d）. Results Thromboembolic events occurred within 7 days after the operation in 6 cases, appeared 2 to 4 weeks after the surgery in 3 cases. All 9 patients received anticoagulation with heparin. One patient also undertook take-down operation. 3 （33%） patients died, accounting for 33% （1/3）, and 18% （2/11） of the early deaths after bidirectional Glenn and TCPC operations. Clinical symptoms improved or disappeared in the remaining 6 patients. Conclusion The mortality of perioperative thromboembolic complications after cavo-pulmonary connection is high, consisting of the important cause of early deaths after these procedures. Our prophylactic anticoagulation strategy proved to be effective, though some thrombi-relatedrisk factors still need to be avoided.
Objective To investigate the value and significance of the changes of plasma level of brain natriuretic peptide（BNP） in evaluating ventricle performance of functional single ventricle after total cavopulmonary connection （TCPC）. Methods We studied 11 patients with functional single ventricle undergone TCPC procedure after 2.1 years, who were followed-up at our ward between April 2004 and November 2004, 7 of them were males and 4 of them were females （TCPC group）. The clinical heart function of patients was scored according to the modified scoring system described by Ross. We obtained 3ml blood samples from the extremital vein of all subjects. Blood was collected into chilled tubes containing EDTA and aprotinin （4.5mg and 1 500u/ml blood, respectively）. The blood samples were promptly centrifuged （-4℃, 3 000r/min for 10 min） and the plasma was separated. BNP concentration was determined using immunoradiometric assay kits. Magnetic resonance imaging （MRI） examination was undertaken in 6 patients of TCPC group to analyse the relative factors with the change of BNP. Control group included 9 healthy children. Results （1） Median plasma BNP level for TCPC group and control group was 400pg/ ml （IQR200-690） and 110 pg/ml （IQR90-190）, respectively. There was a significant difference in plasma BNP between them （P=0. 003）. （2） The results of the index of heart function of TCPC group determined by MRI were 65. 76±8. 65 ml/m2 in end-diastolic volume index, 31. 90±6. 36ml/m2 in end-systolic volume index, 39.09±11.76ml/m2 in stroke volume index, 0. 52± 0. 06 in ejection fraction（EF）, 2.38±0.58L/min·m2 in cardiac index （CI）, 103.49±21.57g/m2 in mass index and 1.57±0.24 in mass/EDV. （3） The plasma BNP level for TCPC group was significant correlation with operation ages（r=0.632, P=0.041 ）. There was no correlation between plasma BNP level with EF, CI, score of Ross, gender, ages, percutaneous oxygen saturation（SpO2） and the type of dominant ventricle, respectively. Conclusions Raised concentration of plasma BNP in patients 2 years after undertaken TCPC procedure indicates that nervous-endosecretory system is still under stress condition. This pattern suggests that neurohormonal activation is primarily related to the altered postoperative physiology. the significance of BNP in patients of functional single ventricle after undertaken TCPC is different from tat in biventricular physiology patiens. The plasma BNP level could not be correctly evaluated the cardiac function after TCPC operation.
Objective To evaluate the clinical outcomes of total cavopulmonary connection (TCPC) and bidirectional Glenn shunt for treating complex congenital heart diseases with single functional ventricles. Methods From January 2002 to May 2004, twelve children, who had complex congenital heart diseases with single functional ventricles, underwent TCPC and bidirectional Glenn shunt. Among them, male was 3 and female was 9. Ages were from 4 to 13 years and body weights were from 14 to 34 kilograms. The diseases included mitral atresia 1 case, tricuspid atresia 3 cases, right ectopic heart with transposition of great arteries 3 cases, D-transposition of great arteries 3 cases, and single ventricle 2 cases. Results Eleven children survived and one child died in acute renal failure 19 hours after operation. The hospital mortality was 8.3%. Four children had chyle-thorax postoperatively, and eight children had uneventful recovery. In the follow-up period, one child died 12 months postoperatively for pulmomary arteriovenous fistula, and there were no complications like severe arrhythmia, thrombosis and cerebral problems. Conclusions TCPC and bidirectional Glenn shunt are safe and effective techniques for treating complex congenital heart diseases with single functional ventricles, and the clinical outcomes are satisfactory. The key points for the successful operation are big enough cava-pulmonary anastomosis as well as aggressive perioperative management.
Objective To summary the experience of extracardiac conduit total cavopulmonary connection (TCPC) and study the operative indication, design, method, and therapeutic efficacy. Methods 29 patients of extracardiac conduit TCPC were reviewed:the average age was 10 years. Of them, there were 9 cases of tricuspid atresia, 9 double inlet ventricle with left ventricular type, 3 mitral atresia, 3 corrected transposition of the great arteries with anatomically right ventricular hypoplasia and 5 double outlet of right ventricle with left ventricular hypoplasia. All patients underwent cardiopulmonary bypass, 12 cases with heart arrested, and 17 without heart arrested. In them, 20 cases’ superior vena cava were anastomosed directly to the upper margin of right pulmonary artery, 9 cases deviated to the left side of right pulmonary artery to enlarge the stoma. For the inferior vena cava stoma, 22 cases’ anterior walls of right atrium were partially incised, and sutured to the posterior wall, then anastomosed with Gore-Tex blood vessel prostheses and connected to pulmonary trunk, and the other 7 cases’ bottom of right atrium was totally incised, the proximal was closed, and the distal was anastomosised with Gore-Tex blood vessel prostheses and connected to the lower margin of right pulmonary artery, deviated to the right sidedness. Results 5 died in the first 22 cases, and the next 7 cases all survive. All patients were followed up for 3 months to 10 years with no late death. Of them, 12 cases had low cardiac output syndrome, and 11 cases of chylothorax. Conclusions Compared with other types of Fontan operation, the extracardiac conduit TCPC has better long-term effects in older or grown-up children. Nevertheless, strict operative adoption, reasonable operative design, refined procedures, carefully observation and treatment are the key points of improving therapeutic efficacy.
ObjectiveTo summarize clinical experience and outcomes of extracardiac conduit total cavopulmonary connection (TCPC)for surgical treatment of complex congenital heart diseases. MethodsClinical data of 52 patients who underwent extracardiac conduit TCPC from September 2006 to December 2012 in Department of Cardiac Surgery, Guangzhou General Hospital of Guangzhou Military Command were retrospectively analyzed. There were 12 patients who received one-stage extracardiac conduit TCPC. There were 40 patients who received two-staged extracardiac conduit TCPC after bidirectional Glenn procedure. Clinical data of all the patients were analyzed. Mortality, morbidity, length of hospital stay and intensive care unit (ICU)stay, mechanical ventilation time, change of arterial oxygen saturation (SaO2)were compared between the 2 groups. ResultsTwo patients (3.8%)died postoperatively including 1 patient with severe low cardiac output syndrome and another patient with multiple organ dysfunction syndrome. Fifty patients were discharged successfully. Mechanical ventilation time, length of ICU stay and hospital stay of the 40 patients who received two-stage extracardiac conduit TCPC were significantly shorter than those of the 12 patients who received one-stage extracardiac conduit TCPC. There was no statistical difference in postoperative morbidity, SaO2 (two-staged vs. one-staged:93%±3% vs. 94%±3%)or mortality (two-staged vs. one-staged:2.5% vs.8.3%)between the 2 groups (P > 0.05). Forty-five patients (90%)were followed up for 6-52 months, and there was no death during follow-up. At 3 months after TCPC, all the patients had heart function of class I or II, and echocardiography showed patent cavopulmonary anastomosis. ConclusionExtracardiac conduit TCPC is a simple procedure, can produce more physiological hemodynamic results, and can be performed for patients who cannot undergo biventricle procedure. Compared with one-stage extracardiac conduit TCPC, two-staged extracardiac conduit TCPC has wider surgical indications, can produce better postoperative recovery, and is easier to perform.
ObjectiveThe total cavopulmonary connection (TCPC) offers a palliation for the hemodynamic derangements associated with congenital heart lesions characterized by a single functional ventricle, but it may cause acute hepatic injury because of the special physiology. The objective of this study was to characterize hepatic function and its relationship to cardiac function in children who had undergone the Fontan procedure. MethodsWe retrospectively analyzed 114 children who had undergone TCPC operation in Shanghai Children's Medical Center between January 2013 and March 2014. There were 65 males and 49 females with a median age of 3.8 years (range 2.5 to 13.2) and a median weight of 14.8 kg (range 12.0 to 33.0). The study cohort was further divided into three groups according to the Child-Pugh classification. The total scores were calculated regarding to ascite, bilirubin, albumin, and international normalized ratio (INR). The scores from 4 to 5 were classified in Child A group, from 6 to 8 classified in Child B group, from 9 to 11 classified in Child C group. Thirty-four patients met criteria for Child Class A, 53 patients for Child Class B, and 27 patients for Child Class C. The univariate analysis and multivariable logistic regression model were used to compare demographic, anatomic, and physiological variables among the three groups. ResultsWithin the study population, 80 patients of Child B group and Child C group met criteria for acute hepatic injury. Univariate risk factors for acute hepatic injury included longer total bypass time (P=0.044), longer aortic cross-clamping time (P=0.005), longer ventilation time (P=0.000), higher postoperative mean pulmonary arterial pressure (P=0.000), elevated N-terminal pro-brain natriuretic peptide (P=0.001), higher vasoactive inotropic score (P=0.000), lower mixed venous oxygen saturation (SvO2, P=0.000) and arterial oxygen saturation (P=0.001), higher incidence of arrhythmia (P=0.000), and low cardiac output syndrome (P=0.003), the need of peritoneal dialysis (P=0.000). In the multivariable logistic model, the need for peritoneal dialysis (OR=17.018, 95%CI 5.117-56.602) and the lower postoperative SvO2 (OR=0.922, 95%CI 0.871-0.976) were two independent risk factors for acute hepatic injury after the TCPC. ConclusionThe need for peritoneal dialysis and lower postoperative SvO2 may represent the compound effects of multiple risk factors including preoperative hemodynamic and a marked hepatic vascular inflammatory response to surgery and cardiopulmonary bypass, which in turn may mediate acute hepatic injury.
ObjectiveTo investigate the effect of fenestration on total cavopulmonary connection (TCPC) in the treatment of complex congenital heart disease. MethodsWe retrospectively analyzed the clinical data of 142 patients undergoing TCPC in Fu Wai Hospital between January 2010 and December 2013. The patients were divided into 2 groups depending on with fenestration or not. There were 71 patients including 44 males and 27 females at age of 65.7+24.5 months in the fenestration group. There were also 71 patients with 42 males and 29 females at age of 60.7+20.8 months in the no fenestration group. Perioperative variables were compared between the two groups. ResultsFour patients (2.82%) died postoperatively. The fenestration significantly increased in the patients with atrioventricular valve regurgitation (AVVI). There were no statistical differences in duration of mechanical ventilation, ICU hospitalized time, early mortality and complications between the two groups (P>0.05). But there were statistical differences in the postoperative pleural effusion duration and 24 h capacity requirement (9.1 d versus 13.1 d, 4.19 ml/(kg · h) versus 5.48 ml/(kg · h)) between the two groups. In the patients whose preoperative mPAP was more than 12 mm Hg, postoperative CVP was lower (P=0.046), maintaining the same blood pressure (SBP=80-90 mm Hg) of vasoactive drugs (P=0.019) and 24 h capacity requirement (P=0.041) were lower, pleural effusion duration was shorter (9.8 d versus 17.8 d, P=0.000) in the fenestration children. 113 patients were followed up for 1.1+1.2 years. SpO2 was 92.1%+3.5% in the fenestration children. Spontaneous closure occured in 8.5% of the patients. No severe cyanosis (SpO2<85%), limb embolism, or stroke. ConclusionFenestration should not be a routine in children of TCPC. Patients with fenestration or not can obtained satisfactory early clinical efficacy. Atrial septal fenestration should be considered in high-risk children with mPAP higher than 12 mm Hg or serious AVVI and be corrected at the same time. Fenestration contributes to stable circulation after TCPC surgery. It can shorten the duration of pleural effusion.
Objective To explore the effects of intravenous treprostinil in different doses on the hemodynamics and postoperative outcomes after high-risk total cavo-pulmonary connection (TCPC). MethodsFrom 2018 to 2021, among 189 patients who underwent TCPC in the Department of Pediatric Cardiac Surgery of Fuwai Hospital, 26 high-risk patients who received the intravenous treprostinil therapy were retrospectively analyzed. There were 12 males and 14 females, with an age of 4 (3, 6) years and a weight of 17.6±6.2 kg. The patients were divided into two groups: a high-dose group [15 patients, maintaining dose>10 ng/(kg·min)] and a low-dose group [11 patients, maintaining dose≤10 ng/(kg·min)]. The hemodynamics before treprostinil using and during the first 24 hours after reaching the maintaining dose of treprostinil, and postoperative outcomes of the two groups were investigated. ResultsThe incidence of heterotaxia was higher in the high-dose group (66.7% vs. 18.2%, P=0.021). During the observation period, the mean pulmonary artery pressure decreased from 11.9±3.6 mm Hg to 11.0±3.3 mm Hg in the low-dose group (P=0.013), and from 12.9±4.7 mm Hg to 10.2±3.4 mm Hg in the high-dose group (P=0.001). The decreasing effect in the high-dose group was better than that in the low-dose group (P=0.010). There was no statistical difference in the postoperative outcomes between the two groups (P>0.05). In terms of side effects, patients needed temporarily increased dosage of vasoactive drugs to maintain stable blood pressure during 6-12 h after treprostinil therapy in the high-dose group. ConclusionIn patients after high-risk TCPC, intravenous high-dose treprostinil has a better therapeutic effect on reducing pulmonary artery pressure. However, it should be noted that increased dosage of vasoactive agents may be required to maintain blood pressure stability in patients with high-dose treprostinil.