Surgical management of total cavopulmonary connection procedure in functional single ventricle with separate hepatic venous drainage / 中国胸心血管外科临床杂志

@#Objective    To investigate the management of hepatic vein (HV) in patients with functional single ventricle (FSV) and separate hepatic venous drainage (SHVD) undergoing total cavopulmonary connection (TCPC) and evaluate this kind of surgery. Methods    The clinical data of 32 consecutive patients with SHVD who underwent modified TCPC operation from August 2005 to January 2017 in our center were retrospectively analyzed. There were 25 males and 7 females with an average age of 2-19 (8.0±5.0) years and body weight of 11-66 (25.4±15.8) kg. Results    There were 20 patients with heterotaxy syndrome and 12 patients with other types of FSV. SHVD was diagnosed preoperatively in 27 patients, among whom 20 patients were connected by intra-extracardiac Gore-Tex conduit, and the other 7 patients were connected by extracardiac Gore-Tex conduit. Because of the missed diagnosis of SHVD, the other 5 patients showed severe decrease of blood oxygen saturation in the early postoperative period and underwent re-operation soon. The postoperative blood oxygen saturation was 92.0% (90.0%, 96.0%), central venous pressure was 10-23 (15.5±3.5) mm Hg, mechanical ventilation assisted time was 16.0 (7.5, 24.0) h, and ICU stay time was 3.0 (2.0, 5.5) d. There were 3 early and 1 late deaths. Conclusion    Intra-extracardiac conduit is an effective and feasible modified TCPC operation for patients with FSV and SHVD, while the surgical details need to be formulated in combination with individual anatomical structure. Preoperative missed diagnosis of SHVD must be avoided. Otherwise, after TCPC, a large amount of stealing blood from HV with low circulation pressure into atrium would lead to unacceptable hypoxemia.

Surgical strategies of biventricular conversion from single-ventricle palliation for the complex congenital heart defects / 中华胸心血管外科杂志

Objective:To explore and summarize the surgical strategies of biventricular conversion from single-ventricle palliation for the complex congenital heart defects in this study.Methods:From April 2017 to December 2019, a total of 14 cases with complex complex congenital heart defects who had planned to undergo single-ventricle palliation were finally accepted biventricular conversion. According to the development and anatomical characteristics of the ventricles, they were divided into two groups: ventricular well development group(9 cases), all of which showed complex intracardiac defects, and the ventricles well developed, including 4 cases of double outlet right ventricle with remote ventricular septal defect, 2 cases of transposition of great arteries combined with ventricular septal defect and left ventricular outflow tract obstruction, one case of congenitally corrected transposition of the great arteries combined with double outlet right ventricle and interrupted aortic arch, one case of double outlet right ventricle with subpulmonary ventricular septal defect and atrioventricular connection inconsistency, one case of pulmonary atresia with ventricular septal defect. Ventricular unbalanced development group(5 cases), all of which were functional single ventricle, including 3 cases of heterotaxy and 2 cases of unbalanced complete atrioventricular septal defect. The operation time, postoperative complications, death, cardiac function and reoperation of the two groups were compared.Results:There was one early death, with a mortality rate of 7.1%. In the ventricular well development group, the average waiting time for biventricular conversion was(56.4 ± 38.9) months. One case died after the operation. The early complications included 2 case of arrhythmias, 3 cases of pleural effusions and 2 cases of pulmonary infections. In the ventricular unbalanced development group, the average waiting time for biventricular conversion was(20.4 ± 15.8) months, and the average of left ventricular end-diastolic volume was(33.6 ± 2.1) ml/m 2. The early complications included 2 cases of low cardiac outputs, one case of arrhythmia and one case of renal failure. There was no significant difference in operation time, intubation time and ICU time between the two groups. The patients were followed up for 4 months to 3 years, and there was no death. In the ventricular well development group, one case was reoperated due to the obstruction of the anastomosis between the superior vena cava and the right atrial appendage. In the ventricular unbalanced development group, one case was implanted with a permanent pacemaker due to the degree Ⅲ atrioventricular block. The NYHA grade of the whole group was Ⅰ-Ⅱ. Conclusion:Whether the complex congenital heart defects with the ventricles well developed or unbalanced, the comprehensive evaluation before the planned single-ventricle palliation will be helpful to find the appropriate cases for biventricular conversion. According to its anatomical characteristics performed the corresponding biventricular conversion can obtain a satisfied clinical outcomes, although the long-term results still need further follow-up.

Connecting hepatic vein and azygos vein by an autologous pericardial conduit to complete a Fontan procedure through a unilateral thoracotomy: A case report / 中国胸心血管外科临床杂志

@#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.

Mechanical valve replacement in complete atrioventricular septal defect—the decision making between common atrioventricular valvuloplasty and valve replacement in Fontan circulation / 中国胸心血管外科临床杂志

@#A 12-year-old girl with double outlet of right ventricle, complete atrioventricular septal defect, pulmonary valve stenosis, single atrium, inferior vena cava-hemiazygos vein drainage and left isomerism, who had undergone surgical left superior vena cava-pulmonary artery connection was referred to our hospital. Echocardiography revealed thickening, crimping and malalignment of common atrioventricular valve, moderate-to-severe regurgitation mainly from the left side and the main pulmonary artery was poorly developed. We preserved the autologous valve and used the 31# mechanical mitral valve which was sutured to mitral annulus. After the main pulmonary artery was transected, proximal end was sutured and a 20# Gore-tex artificial vessel was anastomosed to the distal end of the main pulmonary artery. We excised hepatic vein from the right atrium which was then anastomosed to another artificial vessel. Postoperative oxygen saturation was 95%-100%. Echocardiography and CT showed that the function of mechanical valve and heart was good before discharge. Common atrioventricular valvuloplasty is the preferred choice of the management of impaired valve. However, when dysfunction of valve is too severe and valvuloplasty is more likely to fail, the risk of barely performing a valvuloplasty will increase dramatically. On this condition, the mechanical valve replacement should be performed.

Surgical treatment and mid-term results of functional single ventricle associated with total anomalous pulmonary venous connection / 中国胸心血管外科临床杂志

@#Objective    To evaluate the mid-term results of surgical treatment for functional single ventricle associated with total anomalous pulmonary venous connection. Methods    We 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. Results    All 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). Conclusion    Functional single ventricle combined with complete pulmonary venous drainage is a serious clinical disease, with poor therapeutic effect and high overall mortality.

Prognosis and risk factors for mild to moderate or moderate atrioventricular valve regurgitation after Fontan operation / 中国胸心血管外科临床杂志

@#Objective    To investigate the prognosis and risk factors of mild to moderate or moderate atrioventricular valve regurgitation (AVVR) after Fontan operation. Methods    A total of 34 patients with mild to moderate or moderate AVVR who accepted Fontan operation and atrioventricular valve (AVV) repair between 2004 and 2018 in our center were selected as an AVV repair group. The patients in the same period were matched as a control group by the ratio of 1 : 1-2. Finally 99 patients were included into this study, including 64 males and 35 females, with an average age of 63.4±36.3 months and weight of 17.3±6.7 kg. Grades of AVVR decreased more than 1 was defined as significant improvement. Endpoints of the study were death, Fontan takedown, AVV replacement. Risk factors including Fontan procedures, AVV repair procedures, cardiac anatomy were analyzed. Results    Patients were followed up for 1.5 (0.3-4.0) years. Overall mortality was 15.2%. Most (82.4%) of AVV repair group accepted single AVV repair procedure while partial annuloplasty was the most common (52.9%). With the extension of follow-up, the degree of AVVR in the whole group showed a gradually increasing trend (r=0.352, P=0.000). Mild to moderate AVVR improved spontaneously after Fontan operation, while moderate AVVR did not. AVV repair could improve the degree of AVVR after moderate regurgitation, without increasing the surgical mortality, and regurgitation significantly decreased in 8.8% patients. AVV repair was not effective for mild to moderate AVVR and would increase surgical mortality. Conclusion    AVV function shows a gradual downward trend after Fontan operation. AVV repair is effective for moderate AVVR, does not increase mortality, but the degree of improvement is limited. AVV repair is not effective for mild to moderate AVVR and increases surgical mortality.

Parasitic twin with major cardiac defect: a case report

Parasitic twins are an extremely rare form of asymmetrical conjoined monochorial monoamniotic twins where one of them has a mostly intact body that is able to survive and which is referred to as 'autosite', while the counterpart, referred to as 'parasite', is only rudimentarily developed being physically attached to and nourished by the other twin. Our case is a baby boy with Single Ventricle Heart defect with a thoraco-abdominal mass (epigastric heteropagus twin) attached to the anterior abdominal wall near the umbilicus with minimal visceral sharing. The twins had two external genitalia both in host and parasite micturating separately. After high risk surgery the parasite could be separated completely from the host and postoperative recovery was uneventful.

Cardiac disease and pregnancy: hyper vigilance and extreme caution for optimal outcome

Cardiac disease in pregnancy is a leading cause of maternal death in more so high-income countries. The armamentarium for winning this difficult battle involves shared decision-making with communication across the clinical team and the patient. There is limited clinical evidence concerning effective approaches to managing such complex care and moreover involvement of different specialists makes coordinated care challenging. Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, occurring in 1-2% of the population whereas a single ventricle is a rare congenital heart disease that accounts for less than 1% of all congenital heart diseases. We had two cases of pregnancy with bicuspid aortic valve in one case and the other with single ventricle. The involvement of multidisciplinary team involving cardiologist, cardiothoracic anaesthetist and fetal maternal medicine specialist resulted in good maternal and fetal outcome in both the cases.

Outcomes of complicated congenital heart diseases following multiple re-sternotomy operations: A single-center experience / 中国胸心血管外科临床杂志

@#Objective    To analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations. Methods    We 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. Results    The 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. Conclusion    Series 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.

Strategy of surgical management for pulmonary atresia with intact ventricular septum and mid-term follow-up / 中国胸心血管外科临床杂志

@#To evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center. Methods    From January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed. Results    All patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ. Conclusion    According to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.

Trasplante cardíaco en adultos con cardiopatías congénitas / Heart Transplantation in Adult Patients with Congenital Heart Disease

RESUMEN Introducción: En las últimas décadas los pacientes con cardiopatías congénitas (CC) han presentando nuevos desafíos en el manejo de las complicaciones, tanto de la patología originaria como de las cirugías correctoras que les han permitido llegar a la adultez. Objetivo: Comunicar los resultados y la evolución de los pacientes con CC que hayan sido evaluados para trasplante en un centro de alta complejidad. Se utilizó la base de datos institucional, y se analizaron los datos de 11 pacientes evaluados para trasplante con diversas patologías congénitas. Accedieron al trasplante 5 de ellos con una sobrevida a 1,6 años del 80%. Los pacientes que se hallaban en lista y no se trasplantaron tuvieron una mortalidad del 66%, y los descartados por comorbilidades presentaron una mortalidad del 35%. Conclusión: El trasplante cardíaco en adultos con CC tiene un riesgo periprocedimiento más elevado que para las cardiopatías adquiridas (CA). No obstante, los que sobreviven el primer año postrasplante tienen una excelente evolución a largo plazo.

ABSTRACT Background: Over recent decades, congenital heart disease (CHD) patients have posed new challenges in the management of complications, both of the original condition as of the corrective surgeries that have allowed them to reach adulthood. Objective: The aim of this study was to report the outcomes and evolution of CHD patients who had been evaluated for transplantation in a tertiary care center. Using the institutional database, data from 11 patients with different congenital diseases were evaluated for transplantation. A total of 5 patients underwent transplantation, with a 1.6-year survival rate of 80%. Mortality rate was 66% for patients who were on the waiting list but were not transplanted, and 35% for those who were ruled out due to comorbidities. Conclusion: Heart transplantation in CHD adult patients present a higher periprocedural risk than in patients with acquired heart diseases. However, those who survive the first post-transplant year have an excellent long-term outcome.

Early- and midterm outcomes of pulmonary artery band as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow / 中华外科杂志

Objective@#To examine the early- and midterm outcomes of pulmonary artery banding as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow.@*Methods@#Between January 2008 and December 2017, 49 patients with single ventricle and unrestricted pulmonary blood flow underwent pulmonary artery banding at Department of Cardiac Surgery, Guangzhou Women and Children′s Medical Center, Guangzhou Medical University. There were 29 males and 20 females. The age at the time of surgery was 5.6 (11.5) months (M(QR)), and the weight was 5.2 (3.9) kg. The medical records and results after pulmonary artery banding (death/reoperation, transition to the Glenn procedure) and subsequently after the Glenn procedure (death, transition to the Fontan procedure) were reviewed retrospectively. Actuarial survivals were estimated by the Kaplan-Meier curve. Relative factors for affecting outcomes were analyzed using the Cox regression hazard model.@*Results@#There were 8 early deaths, with a mortality of 16.3%, including 4 cases who received simultaneous arch repair. There were 5 late deaths. During the follow-up of 47(62) (M(QR)) months, 11 patients (22.4%) underwent pulmonary artery banding adjustment, 29 patients (59.2%) underwent the Glenn procedure, 21 patients (42.8%) underwent the Fontan procedure. The survival of patients after the initial pulmonary artery banding were 77.4% (95%CI: 65.6% to 89.2%) and 72.6% (95%CI: 59.9% to 85.3%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that systemic ventricular outflow tract obstruction (HR=4.25, 95%CI: 1.50 to 12.03, P=0.006) and total anomalous pulmonary venous connection (HR=6.49, 95%CI: 3.24 to 12.98, P=0.000) were relative factors for death.@*Conclusions@#The early and midterm outcomes of pulmonary artery banding as an initial palliative strategy is not satisfactory. Systemic ventricular outflow tract obstruction and total anomalous pulmonary venous connection are associated with high mortality.

Value of MSCT in follow-up of functional single ventricle after Fontan palliation / 中国医学影像技术

Objective To explore the value of MSCT in follow-up of functional single ventricle (FSV) after the Fontan palliation. Methods Data of MSCT and cardioangiography (CAG), as well as clinical information of 32 FSV patients after the Fontan procedures were analyzed retrospectively. All patients underwent MSCT examinations with individually manual triggered dual-phase scanning. Qualitative assessment of Fontan conduit structures and post-operative complications were performed. The diameters of pulmonary arteries and descending aorta at the level of diaphragm were measured to calculate McGoon index, in order to evaluate pulmonary artery development. The correlation between diameters of pulmonary artery and pressure of pulmonary artery in diastole phase, as well as between McGoon index and resistent index of pulmonary artery were analyzed. Results MSCT detected 3 cases of right atrio-pulmonary connection, 10 cases of total cavopulmonary connection, 11 cases of extracardiac Fontan and 8 cases of Y-graft Fontan conduits. Moreover, 8 cases of post-operative complications were detected with MSCT, including 2 cases of pulmonary arteriovenous malformations, 3 cases of aortopulmonary collaterals, 2 cases of mural thrombus in the Fontan pathway and 1 case of ventricular aneurysm. The diameters of left and right pulmonary artery measured on MSCT were (1.36±0.22) cm and (1.40±0.15) cm, both negatively correlated with the pressure of pulmonary artery in diastole phase (r=-0.755, -0.678, both P<0.001). McGoon index showed negative correlation with the resistent index of pulmonary artery (r=-0.676, P<0.001). Conclusion With advanced imaging post-processing technology and individual scanning scheme, MSCT can supply several important indicators in the follow-up for patients with FSV after Fontan palliation, showing important clinical value.

Quantitative Evaluation of the First Order Creatine-Kinase Reaction Rate Constant in in vivo Shunted Ovine Heart Treated with Oxandrolone Using Magnetization Transfer 31P Magnetic Resonance Spectroscopy (MT-31P-MRS) and 1H/31P Double-Tuned Surface Coil: a Preliminary Study

PURPOSE: Children born with single ventricle physiology demonstrate poor growth rate and suffer from malnutrition, which lead to increased morbidity and mortality in this population. We assume that an anabolic steroid, oxandrolone, will promote growth in these infants by improving myocardial energy utilization. The purpose of this paper is to study the efficacy of oxandrolone on myocardial energy consumption in these infants. MATERIALS AND METHODS: We modeled single ventricle physiology in a lamb by prenatally shunting the aorta to the pulmonary artery and then postnatally, we monitored cardiac energy utilization by quantitatively measuring the first order reaction rate constant, kf of the creatine-kinase reaction in the heart using magnetization transfer 31P magnetic resonance spectroscopy, home built 1H/31P transmit/receive double tuned coil, and transmit/receive switch. We also performed cine MRI to study the structure and dynamic function of the myocardium and the left ventricular chamber. The spectroscopy data were processed using home-developed python software, while cine data were analyzed using Argus software. RESULTS: We quantitatively measured both the first order reaction rate constant and ejection fraction in the control, shunted, and the oxandrolone-treated lambs. Both kf and ejection fraction were found to be more significantly reduced in the shunted lambs compared to the control lambs, and they are increased in oxandrolone-treated lambs. CONCLUSION: Some improvement was observed in both the first order reaction rate constant and ejection fraction for the lamb treated with oxandrolone in our preliminary study.

Surgical treatment strategy and outcome of functional univentricular heart with total anomalous pulmonary vein drainage / 中国胸心血管外科临床杂志

@#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.

Research and application progress of mechanical circulatory support devices for univentricular circulation / 中国胸心血管外科临床杂志

@#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.

Staged surgical results of functional single ventricle anomalies with pulmonary hypertension / 中国胸心血管外科临床杂志

@#Objective    To explore current results after staged operations in patients with functional single ventricle anomalies and pulmonary hypertension. Methods    We retrospectively analyzed the clinical data of 129 patients with functional single ventricle anomalies and pulmonary hypertension undergoing pulmonary artery banding in our hospital between April 2008 and December 2015. There were 81 males and 48 females. There were 71 patients with double outlet of right ventricle, 17 patients with tricuspid atresia, 7 patients with transposition of great arteries, 33 patients with uni-ventricular heart, and one patient with complete atrio-ventricular septal defect. The surgical results, transition to Glenn procedure and subsequent transition to Fontan procedure were analyzed. Results    The 129 patients underwent 159 operations of pulmonary artery banding totally. Hospital mortality was 4.7% (6/129). Nine patients were lost to follow-up. Eighty-seven (67.4%) patients underwent the second-stage Glenn procedure, and 43 patients(33.3%) underwent third-stage Fontan procedure. Two patients died after Glenn and 3 patients died after Fontan separately. There were 32 patients who accompanied with coarctation, interruption of aortic arch, heterotaxy, total anomalous pulmonary venous connection or atrio-ventricular valve regurgitation. Fifteen (46.9%) patients succeeded in transition to Glenn, and 6 (18.8%) patients succeeded in transition to Fontan. Fourteen patients developed obstruction of left ventricular outlet tract or bulbo-ventricular foramen. Conclusion    Early pulmonary artery banding is an acceptable strategy for patients with single ventricle anomalies and pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good. Accompanied complex anomalies are risk factors for lower ratio of transition to Glenn and  Fontan procedure.

Computed Tomography-Based Ventricular Volumes and Morphometric Parameters for Deciding the Treatment Strategy in Children with a Hypoplastic Left Ventricle: Preliminary Results

OBJECTIVE: To determine the utility of computed tomography (CT) ventricular volumes and morphometric parameters for deciding the treatment strategy in children with a hypoplastic left ventricle (LV). MATERIALS AND METHODS: Ninety-four consecutive children were included in this study and divided into small LV single ventricle repair (SVR) (n = 28), small LV biventricular repair (BVR) (n = 6), disease-matched control (n = 19), and control (n = 41) groups. The CT-based indexed LV volumes, LV-to-right-ventricular (LV/RV) volume ratio, left-to-right atrioventricular valve (AVV) area ratio, left-to-right AVV diameter ratio, and LV/RV long dimension ratio were compared between groups. Proportions of preferred SVR in the small LV SVR group suggested by the parameters were evaluated. RESULTS: Indexed LV end-systolic (ES) and end-diastolic (ED) volumes in the small LV SVR group (6.3 ± 4.0 mL/m² and 14.4 ± 10.2 mL/m², respectively) were significantly smaller than those in the disease-matched control group (16.0 ± 4.7 mL/m² and 37.7 ± 12.0 mL/m², respectively; p < 0.001) and the control group (16.0 ± 5.5 mL/m² and 46.3 ± 10.8 mL/m², respectively; p < 0.001). These volumes were 8.3 ± 2.4 mL/m² and 21.4 ± 5.3 mL/m², respectively, in the small LV BVR group. ES and ED indexed LV volumes of < 7 mL/m² and < 17 mL/m², LV/RV volume ratios of < 0.22 and < 0.25, AVV area ratios of < 0.33 and < 0.24, and AVV diameter ratios of < 0.52 and < 0.46, respectively, enabled the differentiation of a subset of patients in the small LV SVR group from those in the two control groups. One patient in the small LV biventricular group died after BVR, indicating that this patient might not have been a good candidate based on the suggested cut-off values. CONCLUSION: CT-based ventricular volumes and morphometric parameters can suggest cut-off values for SVR in children with a hypoplastic LV.

Surgical management of atrioventricular valve regurgitation in functional single ventricle / 中华外科杂志

Objective@#To evaluate the early- and mid-term outcomes of surgical atrioventricular valve (AVV) intervention in patients with functional single ventricle (FSV).@*Methods@#The clinical data of 40 consecutive FSV patients who underwent surgical AVV intervention between January 2008 and December 2017 at Department of Cardiac Surgery, Guangzhou Women and Children′s Medical Center, Guangzhou Medical University were reviewed retrospectively. There were 25 male and 15 female patients. The median age at AVV intervention was 4.5 to 204.0 months (M(QR): 42.5 (59.7) months), and the median body weight was 6.0 to 55.4 kg (M(QR): 13.8 (8.1) kg). The systemic AVV morphology included common AVV in 21 patients, mitral valve in 7 patients, tricuspid valve in 3 patients, and both mitral and tricuspid valve in 9 patients. At first surgical intervention, 15 patients had severe, 25 patients had moderate AVV regurgitation. The timings of the initial surgical intervention were at the first palliative, Glenn, between Glenn and Fontan, and Fontan stage in 5, 23, 4, and 8 patients, respectively. The methods of the initial surgical intervention were AVV repair in 31 patients, AVV replacement in 9 patients. Actuarial survivals were estimated by the Kaplan-Meier method. Prognosis factors for atrioventricular valve reoperation were analyzed using the Cox regression hazard model.@*Results@#There were 6 early deaths, with a mortality of 15.0%. Thirty-four survival patients received a follow-up of 1 to 117 months (M(QR): 44 (34) months). Survival of patients after the initial surgical intervention were 85.0% (95% CI: 74.0% to 95.9%), 79.3% (95% CI: 66.6% to 92.0%), and 79.3% (95% CI: 66.6% to 92.0%) at 1 year, 5 years and 10 years, respectively. In all, only 13 patients completed the Fontan procedure. Six patients underwent 8 reoperations, including AVV replacement in 5 patients, redo AVV repair in 3 patients. Freedom from AVV reoperation at 1 and 5 years was 89.8% (95% CI: 78.8% to 100%) and 79.4% (95% CI: 64.7% to 94.1%), respectively. Multivariate Cox regression analysis revealed that common AVV (HR=3.53, 95% CI: 1.63-7.67, P=0.020) was the prognosis factors for AVV reoperation.@*Conclusions@#The early- and mid-term outcomes of surgical AVV intervention in FSV patients are not satisfactory. The mortality and reoperation rate are still high, and common AVV predicts the AVV reoperation after the initial surgical intervention.

Nutritional status and risk factors for malnutrition in children with single ventricle physiology after total cavopulmonary connection / 上海交通大学学报（医学版）

Objective: To analyse nutritional status of children underwent total cavopulmonary connection (TCPC) also called Fontan procedure, furthermore to investigate risk factors related to malnutrition including underweight, stunting, and thinness. Methods: Clinical data of 314 children underwent Fontan procedure in Shanghai Children's Medical Center from Jan. 2012 to Dec. 2015 were reviewed. WHO Z score was used to evaluate nutritional status of the children underwent Fontan procedure at the time of long-term follow-up. According to the nutritional status, children were divided into underweight and no-underweight groups, stunting and no-stunting groups, thinness and no-thinness groups. Univariate and multivariate Logistic regression analysis were used to detect risk factors for malnutrition in the children after Fontan procedure. Results: In the follow-up survey after Fontan procedure, the proportion of underweight, stunted and thin children were 11.9%, 17.8%, and 16.9%, respectively. Multivariate Logistic regression analysis showed that preoperative malnutrition, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for underweight in the children after Fontan procedure (P=0.008, P=0.000, P=0.025, P=0.001); preoperative malnutrition, elevated mean pulmonary artery pressure, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for stunting in the children after Fontan procedure (P=0.001, P=0.046, P=0.000, P=0.019, P=0.004); preoperative malnutrition, elevated mean pulmonary artery pressure, worse cardiac function, moderate or severe atrioventricular valve regurgitation and long time use of diuretics were the risk factors for thinness in the children after Fontan procedure (P=0.019, P=0.018, P=0.015, P=0.026, P=0.000). Conclusion: Malnutrition is still remained during follow-up period in the children with single ventricle physiology after Fontan procedure. Preoperative malnutrition, worse cardiac function, elevated pulmonary artery pressure, long time use of diuretics and moderate or severe atrioventricular valve regurgitation may be associated with malnutrition after Fontan procedure.