Surgical Management of Norwood Procedure for Atypical Aortic Arch.

Background: Postoperative restenosis of the aortic arch after the Norwood procedure is still an important complication that significantly affects surgical outcomes. The rarity of the Norwood procedure for atypical aortic morphology means appropriate arch reconstruction methods and postoperative complications are still unknown. This study aimed to assess the rate of arch reintervention and clinical outcomes after the Norwood procedure for atypical aortic arch. Methods: This retrospective single-center study was conducted between 2001 and 2022. Sixteen patients were identified, eight with a right aortic arch, five with transposition of the great arteries, one with a right aortic arch and transposition of the great arteries, and two with a large tortuous patent ductus arteriosus connected to the opposite side of the descending aorta. We selected and performed four different surgical techniques depending on each aortic arch morphology. Results: Except for one case, autologous tissue-only arch reconstruction was possible. There was one operative death and four late deaths. Overall, no patients required any surgical or catheter-based reintervention for the aortic arch. On the other hand, left pulmonary artery stenosis due to a narrow subaortic space was found in two patients. Conclusions: The Norwood procedure for atypical aortic arch was performed with good results by choosing the appropriate technique for each morphology. On the other hand, pulmonary artery stenosis is likely to occur especially in the transposition of the great arteries group. Therefore, careful surgical method selection or further improvement of the technique that allows retroaortic space should be considered.

Lymphoscintigraphy Findings are Associated with Outcome in Children with Chylothorax After Cardiac Surgery.

Postoperative chylothorax in patients with congenital heart diseases (CHD) results in poor outcomes if anatomical and functional abnormalities of the lymphatic system are present. While these abnormalities are typically diagnosed by intranodal lymphangiography and dynamic contrast magnetic resonance lymphangiography, the usefulness of lymphoscintigraphy in these patients has not been evaluated. Between January 2019 and December 2021, 28 lymphoscintigraphies were performed in our institution for investigating prolonged pleural effusion after cardiac surgery. The images were assessed by three board-certified pediatric cardiologists retrospectively to determine the likelihood of a central lymphatic flow disorder. The likelihood was scored (range 1-3) based on structural abnormalities and congestive flow in the lymphatic system. Those scores were summed and the likelihood was categorized as low to intermediate (< 8 points) or high (8 or 9 points). Median age at lymphoscintigraphy was 129 days (IQR, 41-412 days), it was performed at a median of 22 days (IQR, 17-43) after surgery, and median score was 6 points (IQR, 4-7.5). Kendall's coefficient of concordance (0.867; p < 0.05) indicated high inter-rater reliability. Overall survival at 6 months after surgery was 92.5% in the low-to-intermediate group but 68.6% in the high group (p < 0.05), and duration of postoperative thoracic drainage was 27 and 58 days, respectively (p < 0.05). Lymphatic abnormalities detected by lymphoscintigraphy were associated with poorer outcomes. Lymphoscintigraphy was thought to be useful in assessing anatomic and functional lymphatic abnormalities, despite its minimal invasiveness.

Sutureless Repair of Extracardiac Univentricular Total Anomalous Pulmonary Venous Connection.

BACKGROUND: This study aimed to evaluate the results of sutureless repair of extracardiac total anomalous pulmonary venous connection (TAPVC) with a functional single ventricle at a single institution, including changes in the anastomotic site over time. METHODS: The database contained 98 patients with single-ventricle anatomy who underwent extracardiac TAPVC repair from 1996 to 2022. The median age and body weight at surgery were 59 days and 3.8 kg, respectively. Eighty-seven patients had heterotaxy syndrome, and 42 had preoperatively obstructed TAPVC. Primary sutureless repair was performed in 18 patients, 13 of whom were neonates. The cross-sectional area of the anastomotic site of the atrium and pericardium was divided by the body surface area, and the changes in this value over time were assessed. The median follow-up was 5.2 years (range, 0-19.4 years). RESULTS: Operative mortality and late mortality occurred in 2 (2.0%) and 38 (38.8%) patients, respectively. The actuarial survival rate at 5 years postoperatively was 56.2%. Multivariate analysis identified preoperatively obstructed TAPVC as a risk factor for mortality. Recurrent pulmonary venous stenosis (PVS) developed in 25 patients, thus giving a 5-year rate of freedom from PVS of 64.9%. Multivariate analysis revealed that sutureless repair significantly decreased the incidence of recurrent PVS. The cross-sectional anastomotic area tended to grow in accordance with the patients' growth. CONCLUSIONS: Sutureless repair of extracardiac TAPVC with univentricular anatomy achieved acceptable results. The anastomotic site tended to grow over time and contributed to a decline in the rate of recurrent PVS.

Effect of procedural volume on the outcomes of congenital heart surgery in Japan.

OBJECTIVES: The present study developed a new risk model for congenital heart surgery in Japan and determined the relationship between hospital procedural volume and mortality using the developed model. METHODS: We analyzed 47,164 operations performed between 2013 and 2018 registered in the Japan Cardiovascular Surgery Database-Congenital and created a new risk model to predict the 90-day/in-hospital mortality using the Japanese congenital heart surgery mortality categories and patient characteristics. The observed/expected ratios of mortality were compared among 4 groups based on annual hospital procedural volume (group A [5539 procedures performed in 90 hospitals]: ≤50, group B [9322 procedures in 24 hospitals]: 51-100, group C [13,331 procedures in 21 hospitals]: 101-150, group D [18,972 procedures in 15 hospitals]: ≥151). RESULTS: The overall mortality rate was 2.64%. The new risk model using the surgical mortality category, age-weight categories, urgency, and preoperative mechanical ventilation and inotropic use achieved a c-index of 0.81. The observed/expected ratios based on the new risk model were 1.37 (95% confidence interval, 1.18-1.58), 1.21 (1.08-1.33), 1.04 (0.94-1.14), and 0.78 (0.71-0.86) in groups A, B, C, and D, respectively. In the per-procedure analysis, the observed/expected ratios of the Rastelli, coarctation complex repair, and arterial switch procedures in group A were all more than 3.0. CONCLUSIONS: The risk-adjusted mortality rate for low-volume hospitals was high for not only high-risk but also medium-risk procedures. Although the overall mortality rate for congenital heart surgeries is low in Japan, the observed volume-mortality relationship suggests potential for improvement in surgical outcomes.

Case Report: Retroaortic Innominate Vein With Supracardiac Total Anomalous Pulmonary Venous Connection.

A retroaortic innominate vein (RAIV) is a rare anomaly that passes posterior to the ascending aorta to join the superior vena cava and is associated with congenital heart disease (CHD). The RAIV and normal left innominate vein (LIV) rarely duplicate. The etiology of the RAIV and its relationship with CHD remains unknown. We report a case involving a 1-month-old baby girl with RAIV and supracardiac total anomalous pulmonary venous connection (TAPVC). Transthoracic echocardiogram demonstrated a pulmonary venous confluence (CPV) posterior to the left atrium, an abnormal vertical vein (VV) that originated from the CPV, and a normally positioned LIV. Three-dimensional cardiac computed tomography revealed the VV and RAIV to which it merged. This is the first reported case of a combination of RAIV and isolated TAPVC. We speculate that the VV is connected to the CPV during fetal life, thus leaving the RAIV behind. The RAIV may be detected in various forms with the development of new diagnostic imaging methods. Although a RAIV itself does not require treatment, establishing a correct diagnosis before invasive tests and procedures are performed can help prevent unexpected complications.

[Redo Right Ventricular Outflow Tract Reconstruction].

As more patients with congenital heart disease survive the definitive surgical repair, more attention has paid to their residual defects or late sequela which required surgical re-intervention. Redo-right ventricular outflow tract reconstruction is the most frequent reoperation, mostly indicated for pulmonary regurgitation after tetralogy of Fallot repair, or conduit stenosis in post-Rastelli patients. For patients with significant chronic pulmonary regurgitation, reoperation should be considered before right ventricular geometry and function becomes irreversible. In Japan, expanded polytetrafluoroethylene (ePTFE) valved conduit with bulging sinus has been widely used and has showed excellent long-term results. However, careful monitoring must be continued for right ventricular outflow tract function in order to maintain good quality of life of these patients.

Ascending aortic extension to enlarge the retroaortic space in children after the Norwood procedure.

Left pulmonary artery stenosis is a well-recognized complication following the Norwood procedure. We herein report two cases in which ascending aortic extension was performed to enlarge the retroaortic space in children with left pulmonary artery stenosis after the Norwood procedure. We used graft interposition in the ascending aorta to increase the retroaortic apace and concomitantly performed extended left pulmonary artery reconstruction. This procedure obtains a more balanced distribution of the pulmonary blood flow, which is crucial to achieve good Fontan circulation.

The Impact of the Pulmonary Artery Index and Aortopulmonary Collateral Artery Coil Embolization on Intractable Pleural Effusions After a Fontan Surgery.

An intractable pleural effusion is a common comorbidity of a Fontan operation, occasionally leading to undesirable outcomes. The preventive effect of aortopulmonary collateral (APC) coil embolization against a pleural effusion before a Fontan operation is still controversial.This is a retrospective single-center study; among 227 Fontan cases, 57 cases with complete MRI data were analyzed at first. Factors associated with the duration of pleural drainage (median: 6 (2-41) days) and that of postoperative hospital stay (median: 25 (14-91) days) were analyzed using a multiple regression analysis. The pulmonary artery index (PAI; Nakata index) was associated with both the pleural drainage duration (P < 0.05, r2 = 0.17) and postoperative hospital stay (P < 0.05, r2 = 0.10).Thereafter, all the 227 patients were classified into the following three groups: Group A (12 patients in whom the embolization was performed within 30 days before the Fontan surgery), Group B (131 patients in whom the embolization was performed more than 30 days before the Fontan surgery), and Group C (84 patients in whom the embolization was not performed). Patients in Group A were found to be associated with the shortest length of both periods (P < 0.05).Lower PAI values were related to a prolonged pleural drainage duration and postoperative hospital stay. APC coil embolizations may reduce the risk if they are performed shortly (less than 30 days) before the operation.

Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection.

OBJECTIVES: A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG). METHODS: Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36 days and 3.4 kg, respectively. Surgical outcomes and predictors of mortality were investigated. RESULTS: Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10 months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG. CONCLUSIONS: Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial.

[Management of Functionally Univentricular Heart with Heterotaxy].

Various pathologies of heterotaxy should be diagnosed by documenting the arrangement of the cardiac structures and other organs. They carry functional derangements of cardiovascular systems and abdominal organs, such as atrioventricular valve dysfunction, pulmonary vein obstruction, arrhythmia, and intestinal malrotation. An appropriately organized management of these malformations is pivotal for improving outcomes.

Expansion of a Huge Compressive Left Atrial Appendage Aneurysm in a 29-Day-Old Infant.

We report a case of symptomatic and progressive enlargement of a huge left atrial appendage aneurysm in a 29-day-old infant. The aneurysm was detected by fetal echocardiography and exertional dyspnea developed during the neonatal period. The aneurysm was successfully resected by decompressing the aneurysm using cardiopulmonary bypass without cardiac arrest. Our findings suggest that fetal echocardiography enables early diagnosis of the rare left atrial appendage aneurysm, and early surgical resection may protect affected patients from life-threatening symptoms.

Outcomes of mitral valve replacement with bileaflet mechanical prosthetic valve in children.

OBJECTIVES: We examined the outcomes following mitral valve replacement with bileaflet mechanical prosthetic valve in children and identified the predictors for mortality and reoperation. METHODS: Medical records from 49 children who underwent mitral valve replacement between 1982 and 2015 were reviewed retrospectively. Median age and body weight at initial mitral valve replacement were 2.4 years and 9.7 kg, respectively. The median follow-up was 13 years. Surgical results and predictors for mortality and reoperation were investigated. RESULTS: There was no operative mortality; eight late deaths occurred. The actuarial survival rates were 89.5%, 84.2%, and 80.7% at 5, 10, and 15 years, respectively, after initial mitral valve replacement. The actuarial freedom rates from related complications were 89.5%, 78.3%, and 70.7% at 5, 10, and 15 years, respectively. Nineteen patients required 1st re-mitral valve replacement at a median of 5.9 years; six of these 19 required 2nd re-mitral valve replacement at a median of 8.9 years after 1st re-MVR. The actuarial freedom rates from re-mitral valve replacement were 86.0%, 56.8%, and 44.2% at 5, 10, and 15 years, respectively. No predictor for death was determined; however, the predictor for re-mitral valve replacement was initial valve diameter less than 19 mm. CONCLUSIONS: Survival outcomes among children after mitral valve replacement with bileaflet mechanical prosthetic valve in biventricular heart were satisfactory. However, complications, including re-mitral valve replacement, were frequent and the predictor was of a small prosthesis size.

[Ventricular Septal Defect Patch Closure].

Ventricular septal defect is the most frequent congenital heart disease and it exists as an isolated form or combines with other complex cardiovascular malformations. Soto's classification is based on the location and extension of the defect and it is most useful and comprehensive for a surgeon. Ventricular septal defect closure is one of the basic surgical procedures that a congenital heart surgeon must master first. In order to perform a perfect patch closure of the defect without making complications such as residual interventricular shunt, tricuspid valve regurgitation, or atrioventricular block, surgeon must acquire, ① preoperative and intraoperative accurate assessment and understanding of the anatomical features of the defect, ② effective intraoperative exposure of the defect, and ③ safe and assured stitch and ligation.

Surgical results of total anomalous pulmonary venous connection repair in 256 patients.

OBJECTIVES: This study was performed to analyse the surgical results of total anomalous pulmonary venous connection (TAPVC) repair at a single institution and to identify trends and variables associated with mortality and morbidity, particularly predictors of recurrent pulmonary venous obstruction (PVO). METHODS: Our surgical database contained 256 patients with biventricular anatomy who underwent surgical repair for TAPVC from 1981 to 2016. The anatomic TAPVC subtypes in this study were as follows: 114 supracardiac (44.5%), 56 cardiac (21.9%), 64 infracardiac (25.0%) and 22 mixed (8.6%) types. The follow-up for the entire study ranged from 1.6 months to 28.2 years (median 10.4 years). Preoperative PVO was present in 128 patients (50.0%). All patients with TAPVC with single-ventricle anatomy were excluded from the analysis. Data were retrospectively reviewed. RESULTS: Seven (2.7%) early deaths and 26 (10.1%) late deaths occurred. The actuarial survival rate at 20 years postoperatively was 85.3%. The preoperative predictors of operative mortality were younger age and the era of TAPVC repair (before 1998). In addition to these variables, associated cardiac anomalies were predictors of late mortality. Those for postoperative PVO were younger age, lower weight and being an emergency case. The actuarial survival rate at 20 years was 38.6% for patients with postoperative PVO and 92.2% for patients without postoperative PVO (P < 0.001). CONCLUSIONS: The long-term outcomes after TAPVC repair in patients with biventricular anatomy were satisfactory. Mortality was significantly associated with the rate of progression of postoperative PVO, and careful follow-up was required especially within 6 months after the primary operation.

Long-Term Growth of the Neoaortic Root After Arterial Switch Operation.

BACKGROUND: The growth of the neoaortic root after the arterial switch operation for the transposition of the great arteries remains unclear. This study aimed to investigate the growth of the neoaortic root and identify risk factors for neoaortic root dilatation. METHODS: Serial angiographic measurements of the neoaortic root for at least 10 years were evaluated in 145 patients. A total of 1,876 measurements of the sinuses of the Valsalva and the neoaortic annuli were obtained. A linear mixed effects model was used for z-score analysis, including evaluation of risk factors for neoaortic root dilatation. To assess changes in the time course of neoaortic root absolute diameters, a nonlinear mixed effects model with a growth curve model was used. RESULTS: The growth curve revealed progressive growth of the neoaortic root during somatic growth and stabilization in adulthood without normalization. The growth rates of the sinus and annulus were 0.0046 and 0.029 z-score per year, respectively. The sinus and annulus were estimated to grow up to 47 ± 1 mm and 31 ± 1 mm, respectively. Major risk factors for neoaortic root dilatation were double-outlet right ventricle (parameter estimate [PE] = 2.1, 95% confidence interval [CI] = 1.5 to 2.7, p < 0.0001 for sinus; PE = 1.2; 95% CI = 0.7 to 1.6, p < 0.0001 for annulus) and presence of neoaortic valve insufficiency (PE = 0.9; 95% CI = 0.4 to 1.5; p < 0.001 for sinus; PE = 1.6, 95% CI = 1.2 to 2.0, p < 0.0001 for annulus). CONCLUSIONS: The risk for neoaortic root dilatation was common. Long-term surveillance is mandatory, particularly in patients with double-outlet right ventricle and neoaortic valve insufficiency.

Prolonged PR Interval at Birth Predicting the High Occurrence of Fatal Atrioventricular Block in Hypoplastic Left Heart Syndrome.

Infants with hypoplastic left heart syndrome (HLHS) are at high mortality especially when they are associated with bradyarrhythmias. However, the risk factor of developing high-grade atrioventricular block (HAVB) is still unclear. Seventy-three patients with HLHS in our institutions from 2002 to 2011 were enrolled. The survival rate was assessed by the anatomical types, treatments, occurrence of HAVB, severe tricuspid regurgitation (TR), and restrictive atrial septal defect (ASD) along with electrocardiogram findings at birth. There were 23 (32%) cardiogenic and 7 (10%) non-cardiogenic deaths. The occurrence rate of HAVB but not severe TR or restrictive ASD was higher in 30 deceased patients than in 43 survived patients [7 (23%) vs. 1 (2.3%), p = 0.0038]. The overall mortality rate was higher in patients with HAVB than in those without it (p = 0.0002). Of 7 deceased patients with HAVB, 6 HAVB occurred within 10 days post-surgery, and 3 HAVB led to the early death. The mortality rate of patients with prolonged PR (≥ 0.15 s) but not wide QRS (> 0.08 s) or prolonged QTc (> 0.43 s) at birth was higher than each without it (p = 0.0106). Multivariate analysis indicated that prolonged PR but no other variables was independently associated with the mortality (hazard ratio: 2.948, p = 0.0104). Prolonged PR at birth in HLHS infants predicts the development of fatal HAVB.

Yasui Conversion for Repair After Left Ventricular Outflow Tract Obstruction.

We herein report two cases of progressive left ventricular outflow obstruction after primary repair of arch obstruction and ventricular septal defect that was successfully resolved with Yasui conversion. Patients who require surgical reintervention for progressive left ventricular outflow tract (LVOT) obstruction after primary biventricular repair of interruption of the aortic arch or coarctation of the aorta complex are occasionally experienced. The modified Konno procedure and Ross operation are well recognized as useful for these cases. However, in some patients, these procedures are difficult to perform because of anatomic restrictions or previous procedures. Although the indications are limited, the Yasui conversion is a safe, simple, and useful option for LVOT obstruction after primary biventricular repair.

Left Ventricular Outflow Tract Obstruction in Aortic Arch Anomalies With Ventricular Septal Defect.

BACKGROUND: The predictors of left ventricular outflow tract obstruction (LVOTO) after the repair of coarctation of the aorta or interruption of the aortic arch (CoA/IAA) with ventricular septal defect have been investigated. However, the predictors remain controversial. METHODS: We performed primary repair of CoA/IAA with ventricular septal defect for 75 patients from 1996 to 2005. Four of the 75 patients died within 5 years after primary repair without relation to LVOTO. The morphology of the aortic valve of 71 survivors was bicuspid in 23 patients and tricuspid in 48 patients. The mean follow-up was 9.2 ± 2.6 years after primary repair. RESULTS: There were 12 patients who showed LVOTO of 3.0 m/s or greater after primary repair. All of the 6 bicuspid patients demonstrated valvular aortic stenosis, and all of the 6 tricuspid patients showed discrete subvalvular LVOTO. In 5 of the 6 tricuspid patients, the aortic annular z-score before primary repair was -3.0 or less. A bicuspid aortic valve (p = 0.016) and the aortic annular z-score of -3.0 or less (p = 0.019) were significant risk factors for LVOTO after primary repair. At 10 years after primary repair, 82.6% and 95.6% of the bicuspid and tricuspid patients, respectively, were free from reoperation (p = 0.015). CONCLUSIONS: The presence of a bicuspid aortic valve and an aortic valve annular z-score of -3.0 or less before primary repair are risk factors for LVOTO, and stenotic bicuspid valves and discrete subvalvular LVOTO are the main causes of LVOTO after primary repair of CoA/IAA with ventricular septal defect. The bicuspid patients more frequently required reoperation than the tricuspid patients.

Results of extracardiac conduit total cavopulmonary connection in 500 patients.

OBJECTIVES: This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC). METHODS: Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed. RESULTS: There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included protein-losing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from late-occurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R(2) = 0.32) and elapsed time since operation (R(2) = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m(2) and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for ≥10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients. CONCLUSIONS: The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.