Long-term surgical results of anomalous origin of the left coronary artery from the pulmonary artery repair in infants and older patients.

OBJECTIVES: The objective of this study was to compare the long-term surgical results of anomalous origin of the left coronary artery (LCA) from the pulmonary artery repair between infants and older patients. METHODS: This is a retrospective study of patients undergoing anomalous origin of the LCA from the pulmonary artery repair between 1982 and 2018. RESULTS: Thirty-five patients were enrolled and divided into two groups based on their age: infants (<1 year of age: n = 11) and older patients (>1 year of age: n = 24). Median age was 5.2 months in infants and 6.3 years in older patients. The procedure included 24 LCA implantations and 11 intrapulmonary baffle repairs. Early mortality was recorded only in two infants (18%). Early complications included two low output syndrome (18%). Survival and freedom from reoperation at median follow-up of 17.2 years were not significantly different between groups (survival, 81.8 ± 11.6% vs. 94.1 ± 5.7% [p = .3846]; freedom from reoperation, 77.8 ± 13.9% vs. 90.0 ± 6.8% [p = .1175]). Mitral regurgitation (MR) developed substantially in two older patients in the long-term. In infants, significant improvements of fractional shortening (p = .0252) and left ventricular (LV) end-systolic (p = .0142) and end-diastolic diameter (p = .0157) occurred at 1 month. CONCLUSIONS: Although there was no significant difference in survival and reoperation rate, the postoperative LV remodeling occurred more evident and rapid in infants. Worsening of MR in the long-term was observed only in older patients.

Factors associated with aortic root dilatation after surgically repaired ventricular septal defect.

BACKGROUND: Aortic root dilatation (ARD) in congenital heart disease is related to aortic aneurysm, rupture, and dissection. This study aimed to investigate the factors associated with ARD in patients with surgically repaired ventricular septal defect (VSD). METHODS: This cross-sectional study included 152 patients with surgically repaired VSD. Two definitions of ARD were used: (1) observed Valsalva diameter to body surface area (BSA) ratio >2.1 cm/m2 and (2) absolute value of Valsalva diameter ≥4.0 cm. Odds ratios (ORs) and 95% confidence intervals (CIs) of ARD presence were calculated using multivariate logistic regressions. RESULTS: The prevalence of ARD ranged between 8.6% and 32.9%. Using the definition of observed aortic root diameter/BSA >2.1 cm/m2 , patients with nonsubarterial VSD type were more likely to have ARD (OR 5.65, 95% CI; 1.83-17.44, P=.003) than those with subarterial type, and patients with preoperative right- or noncoronary cusp prolapse (R/NCCP) were more likely to have ARD (OR 3.68, 95% CI; 1.20-11.23, P=.022) than patients without preoperative R/NCCP after adjustment for sex, age at repair (ie, shunt duration), VSD size, and postoperative follow-up period. Using the definition of absolute Valsalva diameter ≥4 cm, nonsubarterial VSD type and presence of R/NCCP were also significantly associated with ARD after adjustment for the same covariates. CONCLUSIONS: Anatomical and morphological features (nonsubarterial type and presence of preoperative R/NCCP) are independently correlated with ARD in patients with VSD regardless of the different definitions of ARD. Patients with surgically repaired VSD may need careful monitoring for potential ARD development.

Long-term follow-up of Fontan completion in adults and adolescents.

BACKGROUND: The Fontan procedure is rarely performed in adults and adolescents in the present era. We review our results with the Fontan procedure in adolescents and young adults. METHODS: Between 1974 and 2010, 79 consecutive patients underwent the Fontan procedure at an age ≥ 15 years (mean age at Fontan operation, 20.3 years ± 4.5 years). Forty-five patients underwent atriopulmonary connection, 11 underwent the Bjork procedure, and 23 underwent total cavopulmonary connection. RESULTS: Ten hospital deaths (HDs) and/or early Fontan takedowns (TDs) occurred. The median follow-up period was 18.2 years (range, 0.6-37.6 years). The estimated freedom from death or TD rates was 79.7% at 5 years, 77.0% at 10 years, 73.9% at 15 years, and 63.9% at 20 years. Age was not a predictor of HD and/or TD. Freedom from death or TD after 1998 was 69.1% at 5 years, 69.1% at 10 years, and 69.1% at 15 years, and before 1997 was 82.3% at 5 years, 79.0% at 10 years, 75.5% at 15 years, and 65.1% at 20 years; there were no significant differences between the two groups. In 19 late-death patients, nine (47.4%) experienced sudden death. Among these patients, five had known arrhythmias before sudden death. CONCLUSIONS: In patients who were ≥15 years old, the surgical results of the Fontan operation were acceptable. Approximately half of the late deaths were sudden deaths, mainly occurring 10-20 years postoperatively.

[Atrial Septal Hematoma after Repair of Ventricular Septal Defect;Report of a Case].

A 3-month-old female with ventricular septal defect underwent definitive repair. In the operation, a venting tube was inserted through the incised patent foramen ovale. The ventricular septal defect was closed using a patch and the incised patent foramen ovale was directly closed. A transesophageal echography showed no abnormal findings in the operating room. On 1st postoperative day, the patient's heart rate and pressure of the superior vena cava increased while blood pressure decreased. A transthoracic echocardiography revealed an atrial septal hematoma. After conservative therapy, the heart rate and pressure of the superior vena cava gradually decreased while blood pressure increased. The atrial septal hematoma disappeared spontaneously. The patient was discharged on 16th postoperative day. The atrial septal hematoma appears to have a good prognosis, but sometimes can be poor resulting and death. Meticulous and constant observation is required to detect an atrial septal hematoma.

Surgical repair of a truncus arteriosus with unilateral absence of the right proximal pulmonary artery.

The unilateral absence of a proximal pulmonary artery (UAPPA) is rare and is most frequently accompanied by cardiovascular anomalies such as tetralogy of Fallot or septal defects. We report a patient with truncus arteriosus with UAPPA in which we performed a two-stage surgical repair. During the first palliative operation, a right modified Blalock-Taussig shunt was constructed to develop the hypoplastic right pulmonary artery. At 10 months, the patient underwent patch closure of a ventricular septal defect with integration of both pulmonary arteries, and reconstruction of the right ventricular outflow tract using a conduit. Four years postoperatively, he continues to do well.

Tissue engineered vascular grafts are resistant to the formation of dystrophic calcification.

Advancements in congenital heart surgery have heightened the importance of durable biomaterials for adult survivors. Dystrophic calcification poses a significant risk to the long-term viability of prosthetic biomaterials in these procedures. Herein, we describe the natural history of calcification in the most frequently used vascular conduits, expanded polytetrafluoroethylene grafts. Through a retrospective clinical study and an ovine model, we compare the degree of calcification between tissue-engineered vascular grafts and polytetrafluoroethylene grafts. Results indicate superior durability in tissue-engineered vascular grafts, displaying reduced late-term calcification in both clinical studies (p < 0.001) and animal models (p < 0.0001). Further assessments of graft compliance reveal that tissue-engineered vascular grafts maintain greater compliance (p < 0.0001) and distensibility (p < 0.001) than polytetrafluoroethylene grafts. These properties improve graft hemodynamic performance, as validated through computational fluid dynamics simulations. We demonstrate the promise of tissue engineered vascular grafts, remaining compliant and distensible while resisting long-term calcification, to enhance the long-term success of congenital heart surgeries.

Comparison of vascular smooth muscle cells in canine great vessels.

BACKGROUND: Elucidating the histological characteristics of normal vascular smooth muscle cells (VSMCs) is important for understanding mechanisms of development, disease etiology and the remodeling and/or regeneration process of the vessel. However, knowledge regarding VSMCs is focused primarily on the artery. Although the characteristics of each great vessel are documented, few studies have examined VSMCs in parallel within each great vessel. The present study focused on comparing characteristics of canine VSMCs within the aorta (Ao), branch pulmonary artery (bPA), main pulmonary artery (mPA) and inferior vena cava (IVC), simultaneously. RESULTS: Western blot and immunohistochemistry were used to determine VSMC protein content for alpha smooth muscle actin (ASMA), calponin, myosin heavy chain (MHC) and its isozyme SM2, and non-muscle myosin heavy chain B (SMemb). Thickness and ratio of the VSMC layer were also measured. Expression levels of ASMA, calponin and SM2 significantly differed between vessels, except between mPA and either bPA, Ao and IVC vessels. Expression levels of MHC were significantly different in all vessels, whilst expression of SMemb was significantly different in the Ao compared with either bPA and mPA vessels. All vessels were significantly different with respect to total wall and VSMC layer thickness. The ratio between VSMC layer and total wall thickness was significantly different for each vessel, except between bPA and mPA vessels. Histological analysis of the IVC revealed that the VSMC layer does not line evenly and continuously through the long axis or transverse sections. With respect to the pulmonary artery, calponin was expressed to a greater extent in the mPA compared with the bPA (P < 0.01*). In contrast, MHC and SM2 were expressed to a greater extent in the bPA compared with the mPA (P < 0.01*). Differences in VSMC distribution indicate structural differences in the proximal and distal pulmonary artery bifurcation. CONCLUSION: Our results show that the VSMC expression pattern in each great vessel is unique and suggestive of the developmental differences between great vessels. We believe this study provides basic data for the pathology, etiology and regenerative capability of the vessels.

Utility and safety of nafamostat mesilate for anticoagulation in dogs.

BACKGROUND: Surgical interventions are recommended for cases of advanced mitral regurgitation, however, limited facilities are available. The most prominent complication in such procedures is heparin-derived bleeding. An alternative anticoagulant to heparin, nafamostat mesilate (NM), can reduce the occurrence of complications associated with heparin such as bleeding or shock. OBJECTIVES: This study aimed to evaluate the utility and safety of using NM during anaesthesia in canines. METHODS: Six healthy adult Beagle dogs were anaesthetised, and NM was administered intravenously as a 10 mg/kg bolus dose over 5 min, followed by a continuous infusion of 10 mg/kg/h over 20 min. Blood tests and blood pressure measurements were performed at 0, 5, 25 and 55 min after NM administration. RESULTS: Activated thromboplastin times at 0, 25 and 55 min were 13.0 ± 0.7 s, 106.7 ± 13.3 s and 28.2 ± 2.9 s, respectively, with a significant difference between 0 and 25 min (p < 0.01) only. No significant differences were observed in prothrombin time, antithrombin, fibrinogen and fibrin degradation product concentrations between timepoints. Activated clotting times (ACTs) at 0, 5, 25 and 55 min were 119.5 ± 9.6 s, 826.7 ± 78.6 s, 924.8 ± 42.4 s and 165.2 ± 13.5 s, respectively. Significant differences were observed between 0 and 5 min (p < 0.05) and between 0 and 25 min (p < 0.05). Blood pressure changes occurred in four dogs (66.7%). No other serious adverse effects were observed. CONCLUSIONS: ACT results indicated that NM use in anaesthetised healthy dogs was sufficient to obtain procedural anticoagulation with minimal adverse effects. However, these preliminary data require validation in further studies on cardiopulmonary bypass surgery.

Evaluation method for cell-free in situ tissue-engineered vasculature monitoring: Proof of growth and development in a canine IVC model.

We previously developed a non-cell-dependent biodegradable scaffold to create in situ tissue-engineered vasculature (iTEV) and tested it in a canine inferior vena cava (IVC) model. As iTEV features change dramatically during tissue generation, practical, simple, and accurate methods to evaluate iTEV are needed. The present study examined the usefulness of a novel method to evaluate iTEV growth and remodeling according to a simple formula using angiography: hepatic vein (HV) index = (IVC-HV junction angle) ÷ (π × [minimal internal iTEV diameter ÷ 2]2). HV index strongly correlated with the pressure gradient across iTEV, which tended to improve during the tissue generation period up to 12 months post-implantation. Time-course changes in HV index reflected iTEV tissue development and in-vivo characteristics, such as hemodynamic congestion. In conclusion, HV index is useful to assess iTEV graft function because it represents both the morphometrics and hemodynamics of iTEV with only diagnostic imaging data.

Long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction.

OBJECTIVE: The objective of this study was to evaluate the long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction. METHODS: We conducted a retrospective study of patients with transposition of the great arteries or double outlet right ventricle with left ventricular outflow tract obstruction undergoing biventricular repair between 1980 and 2017. RESULTS: One hundred and eleven patients were enrolled and classified into five groups: atrial switch (n = 20), arterial switch (n = 12), Nikaidoh (n = 7), Rastelli (n = 48), and REV operation groups (n = 24). Early mortality was highest in Nikaidoh group (29%). Median follow-up was 18.2 years. Long-term survival was by far lowest in Nikaidoh group and comparable among the other 4 groups. Freedom from reoperation at 20 years was lowest in Rastelli group (32.1%) due to right ventricular outflow tract-related reoperations. While having no recurrence of left ventricular outflow tract obstruction, the arterial switch operation group had a high proportion of substantial neo-aortic regurgitation (29%). CONCLUSIONS: The long-term survival was satisfactory regardless of the surgical technique except Nikaidoh group. The surgical option for transposition of the great arteries with left ventricular outflow tract obstruction should be selected based on the features of the respective procedures.

Cardiac tamponade due to perforation by an Amplatzer atrial septal occluder in a patient with Marfan syndrome.

Device closure of atrial septum defect was performed using an Amplatzer septal occluder in a 48-year-old patient with Marfan syndrome. Acute tamponade due to perforation was observed 2 months after catheter intervention. Careful consideration of the indication for device closure for atrium septal defect is necessary in patients with Marfan syndrome.

Outcome of Pulmonary Autograft After the Ross Procedure.

BACKGROUND: The purpose of this study was to assess autograft function after the Ross procedure and to review surgical outcomes associated with autograft reoperations. METHODS: This is a retrospective study of patients undergoing the Ross procedure since 1993. Autograft function and autograft reoperation were studied. Autograft failure was defined as more than moderate autograft regurgitation or autograft dilatation to more than 50 mm diameter or z-score of more than +4 in children. One hospital death was excluded from analysis as were patients with unknown late autograft status. RESULTS: Among 75 patients analyzed, preoperative diagnosis before the Ross procedure included aortic regurgitation in 26, aortic stenosis in 19, combined lesions in 28, and 2 mechanical valve malfunctions. Median age at the Ross procedure was 12.1 (0.4-43.6) years with 44 children less than 15 years old. Six patients had greater than mild autograft regurgitation at post-Ross hospital discharge. During median follow-up of 14.9 years, there were 23 autograft failures. Eighteen autograft reoperations were performed on 17 patients (13 children), including 12 aortic valve replacements, 5 aortic root replacements (including 1 valve-sparing root replacement), and 1 Konno procedure. Freedom from autograft failure and autograft reoperation at 20 years after the Ross procedure was 52.0% and 66.3%, respectively. Multivariate analysis identified greater than mild autograft regurgitation at hospital discharge from Ross procedure as a risk factor for autograft failure (P < .01). All patients who underwent autograft reoperation survived and had good health status at a median of 6.9 years after the reoperation. CONCLUSIONS: The Ross procedure is effective in delaying prosthetic aortic valve replacement, although the time-related risk of autograft failure is a real consideration.

Mid- to long-term surgical outcomes of partial anomalous pulmonary venous connection.

OBJECTIVE: To investigate mid- to long-term morbidity and mortality after the repair of partial anomalous pulmonary venous connections (PAPVCs). METHODS: We retrospectively analyzed the cases of 29 consecutive patients who underwent surgery in 1991-2010 at Tokyo Women's Medical University Hospital. Median follow-up period: 9.9 years. Median age at operation: 7.4 years. Median body weight: 22.4 kg. Right PAPVC (n = 23), left-PAPVC (n = 5), and mixed PAPVC (n = 1) were identified. RESULTS: In the 14 patients with a right upper pulmonary vein (PV) connected to the superior vena cava (SVC) and/or cavo-atrial junction, the following were performed: single-patch diversion (n = 6), double-patch diversion (n = 4), Warden procedure (n = 1), and atrial septation with the atrial wall (n = 3). In the nine patients with a right anomalous PV connected to the right atrium (RA) and/or coronary sinus, intra-atrial rerouting was performed (n = 7) and translocation of the atrial septum (n = 2). Intra-atrial rerouting of the left upper and lower PVs to the coronary sinus (n = 1) and direct anastomosis of the left upper PV to the left atrial appendage (n = 4) were performed. Double-patch diversion and direct anastomosis of the left upper PV to the left atrial appendage was performed in a patient with mixed PAPVC. There were no post-operative deaths or reoperations, although transient sinus node dysfunction occurred (n = 2 with atrial septation and intra-atrial rerouting), while mild stenosis of the SVC occurred (n = 1 with double-patch diversion). CONCLUSIONS: The various methods which we used for PAPVC repair all appear to have reasonable outcomes, although rhythm disturbance and stenotic complications were confirmed in a few patients.

Spontaneous reversal of stenosis in tissue-engineered vascular grafts.

We developed a tissue-engineered vascular graft (TEVG) for use in children and present results of a U.S. Food and Drug Administration (FDA)-approved clinical trial evaluating this graft in patients with single-ventricle cardiac anomalies. The TEVG was used as a Fontan conduit to connect the inferior vena cava and pulmonary artery, but a high incidence of graft narrowing manifested within the first 6 months, which was treated successfully with angioplasty. To elucidate mechanisms underlying this early stenosis, we used a data-informed, computational model to perform in silico parametric studies of TEVG development. The simulations predicted early stenosis as observed in our clinical trial but suggested further that such narrowing could reverse spontaneously through an inflammation-driven, mechano-mediated mechanism. We tested this unexpected, model-generated hypothesis by implanting TEVGs in an ovine inferior vena cava interposition graft model, which confirmed the prediction that TEVG stenosis resolved spontaneously and was typically well tolerated. These findings have important implications for our translational research because they suggest that angioplasty may be safely avoided in patients with asymptomatic early stenosis, although there will remain a need for appropriate medical monitoring. The simulations further predicted that the degree of reversible narrowing can be mitigated by altering the scaffold design to attenuate early inflammation and increase mechano-sensing by the synthetic cells, thus suggesting a new paradigm for optimizing next-generation TEVGs. We submit that there is considerable translational advantage to combined computational-experimental studies when designing cutting-edge technologies and their clinical management.

Local tenomodulin absence, angiogenesis, and matrix metalloproteinase activation are associated with the rupture of the chordae tendineae cordis.

BACKGROUND: Rupture of the chordae tendineae cordis (CTC) is a well-known cause of mitral regurgitation. Despite its importance, the mechanisms by which the CTC is protected and the cause of its rupture remain unknown. CTC is an avascular tissue. We investigated the molecular mechanisms underlying the avascularity of CTC and the correlation between avascularity and CTC rupture. METHODS AND RESULTS: We found that tenomodulin, which is a recently isolated antiangiogenic factor, was expressed abundantly in the elastin-rich subendothelial outer layer of normal rodent, porcine, canine, and human CTC. Conditioned medium from cultured CTC interstitial cells strongly inhibited tube formation and mobilization of endothelial cells; these effects were partially inhibited by small-interfering RNA against tenomodulin. The immunohistochemical analysis was performed on 12 normal and 16 ruptured CTC obtained from the autopsy or surgical specimen. Interestingly, tenomodulin was locally absent in the ruptured areas of CTC, where abnormal vessel formation, strong expression of vascular endothelial growth factor-A and matrix metalloproteinases, and infiltration of inflammatory cells were observed, but not in the normal or nonruptured area. In anesthetized open-chest dogs, the tenomodulin layer of tricuspid CTC was surgically filed, and immunohistological analysis was performed after several months. This intervention gradually caused angiogenesis and expression of vascular endothelial growth factor-A and matrix metalloproteinases in the core collagen layer in a time-dependent manner. CONCLUSIONS: These findings provide evidence that tenomodulin is expressed universally in normal CTC in a concentric pattern and that local absence of tenomodulin, angiogenesis, and matrix metalloproteinase activation are associated with CTC rupture.

Late Neo-Aortic Valve Regurgitation Long After Arterial Switch Operation.

BACKGROUND: The purpose of this study is to assess risk factors of neo-aortic regurgitation (AR) after an arterial switch operation and outcomes of neo-aortic valve surgery. METHODS: This is a retrospective study of 469 hospital survivors after arterial switch operation from 1982 to 2016. Preoperative diagnoses included 285 transpositions of the great arteries with intact septum, 140 transpositions with ventricular septal defect, and 44 double-outlet right ventricles. More than mild preoperative pulmonary regurgitation was found in 15 patients. Median age at the operation was 1.0 months (range, 0.1-81.5). Concomitant relief of left ventricular outflow tract obstruction was performed in 20 patients. Seventy-seven patients had more than mild neo-AR at hospital discharge. RESULTS: More than moderate neo-AR was found in 41 patients (8.6%) at a median follow-up of 19.0 years (range, 0.1-35.2). Multivariate analysis identified more than mild preoperative pulmonary regurgitation, concomitant relief of left ventricular outflow tract obstruction, and more than mild neo-AR at hospital discharge as risk factors for late neo-AR. Seventeen patients (3.6%) underwent neo-aortic valve surgeries, including 11 aortic valve replacements, 3 aortic valve plasties, 2 Konno procedures, and 1 aortic root replacement, with median age at the neo-aortic valve surgery of 16.5 years (range, 6.2-27.3). There were 2 late deaths and 5 reoperations to the neo-aortic valve in 4 patients. CONCLUSIONS: Concomitant relief of left ventricular outflow tract obstruction and preoperative more than mild pulmonary regurgitation increase the risk of neo-AR after the arterial switch operation. Reoperation for neo-AR can be done safely.

Long-term outcomes and social independence level after arterial switch operation.

OBJECTIVES: Various issues regarding the long-term survivors of arterial switch operation (ASO) have been clarified according to the improvement of surgical mortality. We reviewed the long-term results and social independence level after ASO. METHODS: Two hundred and four (204) patients who had undergone ASO more than 15 years ago were studied retrospectively. ASO was performed as a primary operation (group I, n=99) or as a secondary operation (group II, n=105). Lecompte procedure was performed in 197 patients, modified Aubert procedure in 5, and original Jatene procedure in 2. RESULTS: There were 11 late deaths. Kaplan-Meier survival rate (not including operative deaths) was 94.9% at 10 years and 94.9% at 15 years in group I, and 96.9% at 10 years and 94.4% at 15 years in group II. Forty-eight reoperations were performed (aortic valve replacement in 6, aortic valvoplasty in 2, Konno procedure in 1, double valve replacement in 1, right ventricular outflow tract reconstruction in 35). The reoperation-free rate including late death was 82.2% at 10 years and 75.7% at 15 years in group I, and 88.2% at 10 years and 78.1% at 15 years in group II. One hundred and seventy-eight patients were classified as NYHA class I and 7 patients as class II. All the patients except those with mental disorder (1) or neurodevelopmental impairment (3) were attending school or working. There was no significant difference in left ventricular function between group I and II, both showing values within the normal range. CONCLUSIONS: The long-term (>15 years) outcome of ASO survivors was satisfactory. Most patients showed excellent cardiac function and were socially independent.

Long-term results of Konno procedure for complex left ventricular outflow tract obstruction.

OBJECTIVE: The current study aims to evaluate the long-term outcomes of the Konno procedure. METHODS: The clinical records of 63 patients who had undergone the Konno procedure between February 1984 and March 2007 were reviewed. During this period, the Ross procedure was introduced in 1996. Of the 63 patients, 38 were male and 25, female. Their ages at the time of operation ranged from 1 year 9 months to 37 years, and their body weights ranged from 8.1 to 63 kg. Valves larger than 23 mm were used in 57 patients. RESULTS: There was one hospital death (myocardial infarction) and six late deaths (sudden death, 2; congestive heart failure, 2; infectious endocarditis, 1; traffic accident, 1). The Kaplan-Meier survival rates including hospital mortality and late mortality were 91.9% at 10 years and 87.7% at 15 years. There were 20 significant complications in 16 patients: thromboembolism was noted in 1 patient; reoperations (Konno procedure (aortic valve replacement), 5 (thrombosed valve, 3; pannus formation, 1; IE, 1); mitral valve replacement, 3; coronary artery bypass grafting, 2; grafting of the descending aorta, 1), balloon dilatation for recoarctation, and 7 catheter interventions were required in 9, 1, and 5 patients, respectively. The event-free rates including all events were 75.2% at 10 years and 67.2% at 15 years. In the long-term period, the results of echocardiography revealed good cardiac function. CONCLUSIONS: The Konno procedure is effective and safe for the treatment of complex left ventricular outflow tract obstruction and for the preservation of ventricular function. Since some issues concerning anticoagulation-related complications and infectious endocarditis remain, careful observation is mandatory.

Tissue-engineered Vascular Grafts in Children With Congenital Heart Disease: Intermediate Term Follow-up.

Tissue engineering holds great promise for the advancement of cardiovascular surgery as well as other medical fields. Tissue-engineered vascular grafts have the ability to grow and remodel and could therefore make great advances for pediatric cardiovascular surgery. In 2001, we began a human clinical trial evaluating these grafts in patients with a univentricular physiology. Herein, we report the long-term results of patients who underwent implantation of tissue-engineered vascular grafts as extracardiac total cavopulmonary conduits. Tissue-engineered vascular grafts seeded with autologous bone marrow mononuclear cells were implanted in 25 patients with univentricular physiology. The graft is composed of a woven fabric of poly-l-lactide acid or polyglycolic acid and a 50:50 poly (l-lactic-co-ε-caprolactone) copolymer. Patients were followed up with postoperatively in a multidisciplinary clinic. Median patient age at operation was 5.5 years and the mean follow-up period was 11.1 years. There was no graft-related mortality during the follow-up period. There was also no evidence of aneurysmal formation, graft rupture, graft infection, or calcification. Seven (28%) patients had asymptomatic graft stenosis and underwent successful balloon angioplasty. Stenosis is the primary complication of the tissue-engineered vascular graft. Avoidance of anticoagulation therapy would improve patients' quality of life. Tissue-engineered vascular grafts have feasibility in pediatric cardiovascular surgery.

Verification of Data Accuracy in Japan Congenital Cardiovascular Surgery Database Including Its Postprocedural Complication Reports.

BACKGROUND: Japan Congenital Cardiovascluar Surgical Database (JCCVSD) is a nationwide registry whose data are used for health quality assessment and clinical research in Japan. We evaluated the completeness of case registration and the accuracy of recorded data components including postprocedural mortality and complications in the database via on-site data adjudication. METHODS: We validated the records from JCCVSD 2010 to 2012 containing congenital cardiovascular surgery data performed in 111 facilities throughout Japan. We randomly chose nine facilities for site visit by the auditor team and conducted on-site data adjudication. We assessed whether the records in JCCVSD matched the data in the source materials. RESULTS: We identified 1,928 cases of eligible surgeries performed at the facilities, of which 1,910 were registered (99.1% completeness), with 6 cases of duplication and 1 inappropriate case registration. Data components including gender, age, and surgery time (hours) were highly accurate with 98% to 100% concordance. Mortality at discharge and at 30 and 90 postoperative days was 100% accurate. Among the five complications studied, reoperation was the most frequently observed, with 16 and 21 cases recorded in the database and source materials, respectively, having a sensitivity of 0.67 and a specificity of 0.99. CONCLUSIONS: Validation of JCCVSD database showed high registration completeness and high accuracy especially in the categorical data components. Adjudicated mortality was 100% accurate. While limited in numbers, the recorded cases of postoperative complications all had high specificities but had lower sensitivity (0.67-1.00). Continued activities for data quality improvement and assessment are necessary for optimizing the utility of these registries.