Objective To comprehensively analyze the clinical outcomes of total cavopulmonary connection (TCPC) in the treatment of functional single ventricle combined with heterotaxy syndrome (HS). MethodsA retrospective analysis was conducted on the patients with functional single ventricle and HS who underwent TCPC (a HS group) in Guangdong Provincial People's Hospital between 2004 and 2021. The analysis focused on postoperative complications, long-term survival rates, and identifying factors associated with patient survival. Early and late postoperative outcomes were compared with matched non-HS patients (a non-HS group). Results Before propensity score matching, 55 patients were collected in the HS group, including 42 males and 13 females, with a median age of 6.0 (4.2, 11.8) years and a median weight of 17.0 (14.2, 28.8) kg. Among the patients, there were 53 patients of right atrial isomerism and 2 patients of left atrial isomerism. Eight patients underwent TCPC in one stage. TCPC procedures included extracardiac conduit (n=39), intracardiac-extracardiac conduit (n=14), and direct cavopulmonary connection (n=2). Postoperative complications included infections in 27 patients, liver function damage in 19 patients, and acute kidney injury in 11 patients. There were 5 early deaths. The median follow-up time was 94.7 (64.3, 129.8) months. The 1-year, 5-year, and 10-year survival rates were 87.2%, 85.3%, and 74.3%, respectively. After propensity score matching, there were 45 patients in the HS group and 81 patients in the non-HS group. Compared to the non-HS group, those with HS had longer surgical and mechanical ventilation time, higher infection rates (P<0.05), and a 12.9% lower 10-year survival rate. Multivariate Cox regression analysis identified asplenia was a risk factor for mortality (HR=8.98, 95%CI 1.86-43.34, P=0.006). ConclusionCompared to non-HS patients, patients with HS have lower survival rates after TCPC, and asplenia is an independent risk factor for the survival of these patients.
Abstract: Objective To summarize the clinical experience for complex congenital heart disease treated with modified Fontan operation. Methods From November 1996 to May 2005,124 patients (male 83,female 41; including tricuspid atresia, single ventricle, double outlet of right ventricle, malposition of great arteries, pulmonary atresia, corrected transposition of great arteries, hypoplastic rightheart syndrome, etc.) underwent modified Fontan operation at age 7.6±5.5 years. Noncardiopulmonary bypass was used in 19 patients, 105 patients with cardiopulmonary bypass. Right atria-pulmonary artery connection were performed in 17 patients, right atria-ventricular connection were performed in 19 patients, and total cavopulmonary connection (TCPC) were performed in 88 patients. Staged operation were performed in 23 patients. Results The hospital mortality (30 days postoperative) was 13.7% (17/124). The hospital mortality of patients undergone right atria-pulmonary artery connection was 23.5%(4/17), patients undergone right atria-ventricular connection was 15.8%(3/19), patients undergone TCPC was 11.4%(10/88), patients undergone operation with fenestration was 14.6%(6/41), and the patients undergone staged operation was 8.7%(2/23). Low cardiac output syndrome, multiple organ failure, and ventricular fibrillation were the cause of death. Morbidity of complications was 16.9％(21/124) in early period. Complications consisted of pleural effusion, arrhythmia, pericardial effusion and low cardiac output syndrome, etc. Eightynine patients were followed up, followup time was from postoperative 6 months to 65 months. Re-hospitalization rate was 6.5%, and re-operation rate was 0.9%. There were pleural effusion in 3 patients, pericardial effusion in 3 patients, and obstruction of inferior vena cava in 1 patient. All patients recovered. Conclusion Modified Fontan operation is an optimal procedure for functional single ventricle, fenestration seems to decrease postoperative pleural effusions.
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 summerize the early results of total cavopulmonary connection (TCPC) procedure with an extracardiac conduit in adults with congenital heart disease, and assess risk factors for postoperative delayed recovery in ICU. MethodsWe retrospectively analyzed the clinical data of 20 adult patients underwent TCPC operation with extracardiac conduit in Fu Wai Hospital between January 2012 and December 2014. There were 14 female and 6 male patients at age of 16 to 33 (20.45±4.33) years. ResultsThere was no hospital mortality. The time of ICU stay was 4.4±1.7 days. And time of hospital stay was 32.5±21.6 days. Morbidities included prolonged pleural effusion lasting more than 7 days in 12 patients (60.0%), new arrhythmias in 3 patients (15.0%), reexploration for bleeding in 3 patients (15.0%), surgical wound poor healing in 1 patient (5.0%). Dopamine and calcium were used in all the patients in the ICU, epinephrine in 18 patients, milrinone in 11 patients. Risk factors for postoperative delayed recovery in ICU were preoperative arrhythmias (P=0.02), cardiopulmonary bypass time longer than 120 min (P=0.04), plasma applications more than 2 000 ml (P=0.01), absence of fenestration (P=0.04), and pleural effusion lasting longer than 7 days (P=0.04). ConclusionThe TCPC procedure with an extracardiac conduit can be performed in adults with encouraged early results. Actively vasoactive drugs to maintain circulation early in ICU has good results for the patient's recovery.
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.
ObjectiveTo analyze the outcomes of patients with one-stage Fontan procedure and short-term prognosis at a single institute. Method We retrospectively analyzed clinical records of 116 patients with one-stage Fontan procedure in our hospital from January 2008 through September 2013. There were 77 males and 39 females (36%), with median age 6.27 years (ranged 3.15 to 17.47 years) at the time of surgery and the median weight at 17.5 kg (ranged 10.0 to 80.0 kg). There were 55 patients with standard extracardiac conduit, 13 patients with direct extracardiac connection, 17 patients with intra-atrial or intra/extracardiac conduit, and 31 patients with lateral tunnel. ResultsMedian cardiopulmonary bypass time was 124 (61-256) minutes. Median cross-clamp time was 60 (19-152) minutes. There were six early deaths (5.1%). The overall median time of the cardiac intensive care unit stay was 4 (1-17) days, with a median ventilator support of 7.3 (1.0-181.0) hours. The mean room air saturation was 90.00%±4.68% before discharge. Median length of chest tube drainage was 10 (4-45) days. Multiple logistic analysis confirmed that heterotaxy syndrome was the only independent predictor for postoperative renal insufficiency. Operations with aortic cross-clamping (OR=26.184, 95% CI 1.712-400.451), preoperative sinus mode dysfunction (OR=6.777, 95% CI 1.495-30.721) and cross-clamp time over 60 minutes (OR=1.036, 95% CI 1.002-1.076) were predictors for prolonged chest tube drainage. A total of 110 patients were followed up for 17 (8-47) months with 2 deaths and 1 with thrombosis. No reoperation occurred. ConclusionThe one-stage Fontan procedure can be performed with satisfactory outcomes. Staged strategies for operations may be appropriately loosen for selected elder children.
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 prevalence, severity and consequences of acute kidney injury (AKI) in the patients who underwent total cavopulmonary connection (TCPC).MethodsThe clinical data of TCPC patients in our center from January 1, 2010 to December 31, 2014 were collected and retrospectively analyzed. The patients with renal replacement therapy, missing serum creatinine data before operation or combined with valve procedures were excluded. We identified whether AKI was associated with hospital length of stay, ICU duration, mechanical ventilation duration, hospital acquired infection, and early mortality by univariable and multivariable analyses.ResultsA total of 163 patients were included. AKI occurred in 57% of patients (n=93), mild AKI in 26.4% (n=43), moderate AKI in 12.3% (n=20) and severe AKI in 18.4% (n=30). Compared with the no AKI group, the AKI group had higher hospital acquired infection rate (15.1% vs. 0.0%, P<0.001). AKI was independently associated with hospital length of stay (median, 10 d, 95%CI 3.9-16.0, P=0.001), ICU duration (median, 103.9, 95%CI 48.6-159.2, P<0.001) , but not associated with mechanical ventilation duration (median, 8 h vs. 7 h, P=0.529).ConclusionPostoperative AKI in the patients undergoing TCPC is common. AKI is associated with higher hospital acquired infection rate, longer hospital length of stay and ICU duration, but not associated with mechanical ventilation duration.
Objective To investigate clinical features and risk factors of prolonged postoperative recovery of pediatric patients in ICU after total cavopulmonary connection（TCPC），provide evidence for risk stratification management strategy， and enhance their postoperative recovery. Methods We conducted a retrospective analysis of clinical data of 81 patients undergoing TCPC in Fu Wai Hospital from January 2010 to July 2012. Three patients who died postoperatively were excluded from analysis. Prolonged postoperative recovery was defined as patients whose postoperative mechanical ventilation time was longer than that of 75% of all the patients. A total of 78 patients were divided into normal recovery group and prolonged recovery group. There were 59 patients in the normal recovery group including 34 male and 25 female patients with their age of 62.5±20.7 months，and 19 patients in the prolonged recovery group including 11 male and 8 female patients with their age of 64.8±29.8 months. Perioperative variables were compared between the two groups. Results The average cardiopulmonary bypass time of all the 81 patients was 107.6±54.1 （33-350） minutes. The average aortic cross-clamping time of 17 patients was 46.4±31.5 （22-143） minutes. Three patients （3.7%） died postoperatively because of severe low cardiac output syndrome and thrombosis in the extracardiac conduit. The mechanical ventilation time and ICU stay were 7.5 hours and 1.6 days respectively in the normal recovery group，which were both significantly prolonged in the prolonged recovery group. Preoperative high hemoglobin level，coexistence of intracardiac anomalies，longer cardiopulmonary bypass time，and non-fenestrated procedure were the main risk factors of prolonged postoperative recovery. Conclusion Early extubation and fast track recovery can be achieved in most of TCPC patients. Risk stratification management strategies may contribute to successful postoperative recovery of critical patients after TCPC.
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.