Significant atrioventricular valve (AVV) insufficiency has been associated with increased mortality and morbidity in patients with single ventricle. Although techniques such as annuloplasty, chordal elongation, and the use of pericardial patches can improve AVV function in many patients, a number of patients continue to have significant AVV insufficiency and may require AVV replacement. The present review made a comprehensive literature review concerning the outcome, optimal timing, key points of technique skills and prognosis evaluation.
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.
ObjectiveTo evaluate the mid-term results of surgical treatment for functional single ventricle associated with total anomalous pulmonary venous connection.MethodsWe reviewed the clinical data and follow-up results of 12 patients in our hospital who underwent both single ventricle series palliation and total anomalous pulmonary venous connection correction from 2008 to 2018. There were 6 males and 6 females at age of 2.3 (1-21) years. Univariable and multivariable Cox proportional hazard regression methods were performed. The Kaplan-Meier method was used to estimate the survival rate.ResultsAll patients were successfully removed from extracorporeal circulation. The cardiopulmonary bypass time was 113.8±42.5 min. The myocardial block time was 57.7±31.7 min. There were 3 in-hospital deaths and 4 late deaths. The causes of death in hospital were pulmonary hypertension, pulmonary hemorrhage, and hypoxemia. During follow-up, the cause of death was heart failure. The survival rate at 1 year and 3 years was 58.3% (95%CI 27% to 80%) and 40% (95%CI 13% to 65%), respectively. Median follow-up was 48.3 months (range: 1 to 118 months).ConclusionFunctional single ventricle combined with complete pulmonary venous drainage is a serious clinical disease, with poor therapeutic effect and high overall mortality.
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.
The single ventricular circulation is notable for coexisting systemic venous hypertension and pulmonary arterial hypotension. The use of mechanical circulatory support (MCS) devices is a viable therapeutic treatment option for patients with congestive heart failure. Ventricular assisted devices, cavopulmonary assisted devices, and total artificial heart pumps continue to gain acceptance as viable treatment strategies for single ventricular physiology patients as bridge-to transplantation, bridge-to-recovery, and longer-term circulatory support alternatives. Patients with single ventricular physiology had the lower survival rates compared with those with biventricular circulation. We present a review of the current and future MCS devices for patients with univentricular circulations.
ObjectiveTo analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations.MethodsWe retrospectively analyzed the clinical data of 146 patients undergoing multiple cardiac re-sternotomy operations between 2015 and 2019 in our center. There were 95 males and 51 females with an age of 4.3 (3.1-6.8) years and a weight of 15.3 (13.4-19.0) kg at last operation.ResultsThe top three cardiac malformations were pulmonary atresia (n=51, 34.9%), double outflow of right ventricle (n=36, 24.7%) and functional single ventricle (n=36, 24.7%). A total of 457 sternotomy procedures were performed, with 129 (88.3%) patients undergoing three times of operations and 17 (11.7%) patients undergoing more than three times. Fifty-two (35.6%) patients received bi-ventricular repair, 63 (43.1%) patients received Fontan-type procedures, and 31 (21.2%) patients underwent palliative procedures. Ten (6.8%) patients experienced major accidents during sternotomy, including 7 (4.8%) patients of urgent femoral artery and venous bypass. Eleven (7.5%) patients died with 10 (6.8%) deaths before discharge. The follow-up time was 20.0 (5.8-40.1) months, and 1 patient died during the follow-up. The number of operations was an independent risk factor for the death after operation.ConclusionSeries operations of Fontan in functional single ventricle, repeated stenosis of pulmonary artery or conduit of right ventricular outflow tract post bi-ventricular repair are the major causes for the reoperation. Multiple operations are a huge challenge for CCHD treatment, which should be avoided.
Though the 10 year survival rate of Fontan procedure has increased from 69% in 1990 to 85% in 2006. Recently researchers have strived to improve the longterm outcome of this procedure and have made great achievements. They found that among the ten items of standard, the limits associated with age, anomalous systemic or pulmonary venous drainage, sinus rhythm, ratio of pulmonary artery/aorta should be relaxed. New operative designs such as extracardiac lateral tunnel Fontan and new types of HemiFontan procedure using shortened extracorporeal circulation may help to decrease the postoperative complications. Standardized postoperative therapeutic plan after Fontan procedure may decrease the duration of chest drainage，shorten the inpatient days and also avoid longterm treatment. However, longterm followup after Fontan procedure showed that the incidence rate and risk of longterm postoperative complications were still high. This article was directed to review the researches about Fontan on operative indications, optimizing the operative strategies, modifying the postoperative monitoring techniques and longterm follow-up.
Objective Complex congenital heart defects are sometimes treated by Fontan palliation for various reasons. However, the middle- and long-term prognosis of single-ventricle repair is worse than that of two-ventricle repair. In this study we reported the results of biventricular conversion in these challenging patients initially palliated towards single-ventricle repair. Methods Eight patients underwent biventricular repair conversion from prior bidirectional Glenn shunt palliation in our hospital between October 2013 and March 2016. The median age in bidirectional Glenn shunt was 2.6 years (range, 1.0 to 5.9 years) and in biventricular repair conversion was 6.6 years (range, 4.5 to 11.1 years). Three patients suffered complete transposition of great arteries combined with ventricular septal defect and left ventricular outflow tract obstruction, three double outlet right ventricle and non-committed ventricular septal defect combined with pulmonary stenosis or atresia, one double outlet right ventricle combined with complete ativentricular septal defect and pulmonary stenosis and one Tetralogy of Fallot. Results Bidirectional Glenn shunt was taken down and superior vena cava was reconnected to the right atrium in all patients. Mean cardiopulmonary bypass and aortic cross-clamp time was 275.6±107.1 min and 165.9±63.6 min, respectively. Mean length of hospital stay and ICU stay were 33.6±23.0 d and 20.3±21.0 d, respectively. At a mean follow-up of 1.4±0.7 years, there was no mortality and reoperation. No patients presented with sinoatrial node dysfunction and superior vena cava anastomotic stenosis. According to the New York Heart Association (NYHA) Functional Classification, all patients were classified asⅠ-Ⅱ. Conclusion Biventricular repair conversion can be safely performed with favorable mortality and morbidity in specific patients palliated towards single-ventricle repair. Further follow-up is needed to investigate the long-term outcomes.