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.
ObjectiveTo analyze the Ebstein anomaly's reoperative strategy and mid- to long-term results.MethodsWe retrospectively reviewed the clinical data of 23 patients who diagnosed with Ebstein anomaly and underwent reoperation for tricuspid valve insufficiency between July 2002 and July 2017 in Fuwai Hospital. There were 9 (39.1%) males and 14 (60.9%) females, with a median age of 28.0 (19.0, 45.0) years.ResultsAmong the 23 patients, 8 (34.8%) underwent tricuspid valvuloplasty and 15 (65.2%) underwent tricuspid valve replacement. The rate of valvuloplasty was 16.7% before 2012, and 54.5% after 2012 (P=0.089) as Cone reconstruction procedure was used. In the valvuloplasty cohort, 3 (37.5%) patients were treated with Danielson or Carpentier technique, and 5 (62.5%) patients were treated with Cone reconstruction procedure. There was no operation-related death. Early complications occurred in 3 (37.5%) patients. The median follow-up was 6.9 years (range, 3.0-15.1 years), and no adverse cardiac events occurred. In the patients with valve replacement, 7 (46.7%) received mechanical prosthesis and 8 (53.3%) received bio-prosthesis. There was no operation-related death. And early complications were observed in 3 (20.0%) patients. The median follow-up was 6.5 years (range, 2.5-15.3 years). One (6.3%) patient died and 4 (26.7%) had long-term complications during the follow-up period.ConclusionThe mid- to long-term outcomes are convincing in patients who undergo the second operation due to recurrent tricuspid regurgitation of Ebstein anomaly. A low incidence of reoperation is observed. Cone reconstruction procedure provides possibilities of second tricuspid valvuloplasty, and this technique can reduce the rate of tricuspid valve replacement in the second operation. Tricuspid valve replacement is still an alternative method for the treatment of recurrent tricuspid regurgitation in patients with Ebstein anomaly. The bioprosthetic prosthesis may be a better choice than mechanical prosthesis.
We reported a case of a six-year-old boy diagnosed of single ventricle, pulmonary atresia and interrupted inferior vena cava. After modified Blalock-Taussig shunt and bidirectional Glenn procedure, he received the Fontan procedure. The Fontan procedure was done through a unilateral thoracotomy, using an autologous pericardial conduit to connect hepatic vein and azygos vein. The result of short-term follow-up was satisfactory.
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.
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.
ObjectiveTo explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease.MethodsFrom May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed.ResultsPreoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥1.2 m/s at discharge was an independent risk factor for mortality and restenosis.ConclusionThe prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.