Ductal stenting with bilateral pulmonary artery banding as a life-saving management for hypoplastic left heart syndrome with congenital esophageal atresia: A case series.

We present three cases of hypoplastic left heart syndrome (HLHS) complicated by congenital esophageal atresia and trachea-esophageal fistula (EA/TEF). The standard treatment for HLHS involves a staged surgical approach, eventually reaching Fontan completion. There is no report of patients with both HLHS and EA/TEF, and no established treatment strategy exists for such cases. Given the significant risk of simultaneously operating on HLHS and EA/TEF, we elected to pursue staged repair for each condition separately. Initially, soon after birth, we performed gastrostomy to secure the nutritional pathway for EA/TEF and stabilize breathing. Subsequently, we conducted bilateral pulmonary artery banding (bil-PAB) and ductal stenting for HLHS, as the Norwood operation carried an unacceptably high risk in these patients. Two of these patients were able to transition to home care, while the other patient died during hospitalization due to complications after EA repair. A combination of bil-PAB with ductal stenting for HLHS and staged repair for EA/TEF may provide effective management for patients with both conditions. Learning objective: Hypoplastic left heart syndrome (HLHS) and congenital esophageal atresia (EA) are both life-threatening conditions that require early intervention after birth. There are few reports of patients with both conditions, and no treatment strategy is established. Although the procedure carries a high risk, we successfully performed ductal stenting with bilateral pulmonary artery banding for HLHS, as well as staged repair procedures for EA. Our approach may be a viable strategy for these conditions.

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.

Ductus Arteriosus Aneurysm and Pulmonary Artery Thromboses in a Protein S-Deficient Newborn.

Ductus arteriosus aneurysm (DAA) asymptomatically occurs in newborn infants and resolves spontaneously. High-risk DAA with compression, rupture, and thrombosis requires early surgical intervention. Newborn infants have the highest risk of thrombosis among pediatric patients, but the genetic predisposition is difficult to determine in infancy. We herein report a neonatal case of massive thromboses in DAA and pulmonary artery. Desaturation occurred in an active full-term infant 2 days after birth. Echocardiography and contrast-enhanced computed tomography indicated thrombotic occlusion of the DAA and pulmonary artery thrombus. Urgent thrombectomy and ductus resection were successfully performed. After 6 months of anticoagulant therapy, the dissociated low plasma activity levels of protein S from protein C suggested protein S deficiency. A genetic study of PROS1 identified a heterozygous variant of protein S K196E, a low-risk variant of thrombophilia in Japanese populations. There have been seven reported cases with neonatal-onset symptomatic thromboses of DAA involving the pulmonary artery. All survived without recurrence after surgical intervention in five and anticoagulant therapy alone in two. Two newborns had a heterozygous methylenetetrahydrofolate reductase ( MTHFR ) variant, but information on thrombophilia was not available for any other cases. A genetic predisposition may raise the risk of DAA thrombosis, leading to rapid progression.

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.

Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.

Recently, the outcome of severe Ebstein anomaly (EA) has improved with the prevalence of the Starnes procedure. However, time-dependent changes in the size of the central pulmonary artery (PA) have not been fully understood. A retrospective chart review of patients with EA who underwent the Starnes procedure during the neonatal period and those with pulmonary atresia with intact ventricular septum (PAIVS) was performed at Fukuoka Children's Hospital. There were 14 patients in the severe EA group and 36 in the PAIVS group, with mean observational periods of 3.8 and 4.2 years, respectively. No significant difference in survival was observed between the groups. However, the mean size of the central PA was smaller in the severe EA group at each surgical stage (after systemic-to-pulmonary shunt, after the bidirectional Glenn procedure, and after the Fontan procedure). A significantly larger ventricular volume was observed in the severe EA group after the Fontan procedure. The growth of the central PA was poor in patients with severe EA. Patients with severe EA should be carefully monitored in this regard both before and after undergoing the Fontan procedure. Further studies regarding long-term prognosis are expected.

Hemodynamic Characteristics After Fontan Procedure in Patients with Down's Syndrome.

Patients with Down's syndrome (DS) are generally regarded as not being good candidates for the Fontan procedure. However, detailed hemodynamic changes over time are not fully clarified. A retrospective chart review of all patients with DS who underwent the Fontan procedure and 5 times that number of Fontan patients without DS performed in Fukuoka Children's Hospital and Kyushu University Hospital. Seven Fontan patients with DS were identified, and 35 Fontan patients without DS were recruited. During the mean observational periods of 14.7 years and 15.0 years (DS and non-DS, respectively) after the Fontan procedure, only one DS patient died. Central venous pressure (CVP) and transpulmonary pressure gradient significantly increased, and arterial oxygen saturation significantly decreased over time in DS patients after the Fontan procedure compared with those without DS. CVP in DS patients after the Fontan procedure increased over time compared with non-DS patients. Better management including the efficacy of Pulmonary arterial hypertension-specific therapy should be clarified in further studies.

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.

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.

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.

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.

Systemic-to-Pulmonary Collateral Flow Correlates with Clinical Condition Late After the Fontan Procedure.

In the Fontan circulation, there is a substantial degree of systemic-to-pulmonary collateral flow (SPCF), which can be measured by cardiac magnetic resonance (CMR). However, the correlation between the degree of SPCF and long-term outcomes is not fully understood. We retrospectively studied 321 patients who underwent the Fontan procedure and CMR at a single center. Using CMR, we calculated SPCF as pulmonary blood flow - systemic blood flow. %SPCF was defined as SPCF ÷ pulmonary blood flow. The mean age of patients at CMR was 14.3 ± 7.5 years. The average %SPCF was 13.0% ± 11.0%. With a multivariate analysis, %SPCF was significantly correlated with time (i.e., the longer the time period since the Fontan procedure, the lower the %SPCF) (p = 0.006), previous total anomalous pulmonary vein drainage (p = 0.007), a low pulmonary artery index (Nakata index) before the Fontan procedure (p = 0.04), and older age at the time of the Fontan procedure (p = 0.002). Regarding the findings after the Fontan procedure, %SPCF was significantly correlated with ventricular end-diastolic volume (p < 0.001), ventricular end-systolic volume (p < 0.001), central venous pressure (p < 0.001), plasma brain natriuretic peptide concentration (p < 0.001), hemoptysis (p = 0.009), and poor New York Heart Association functional class (p = 0.007). SPCF was correlated with clinical condition after the Fontan procedure. The importance of sufficient growth of the pulmonary vascular bed should be emphasized because the development of SPCF is believed to result from the poor condition of the pulmonary circulation.

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.

Impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after Fontan operation.

BackgroundsThe aim of this study was to assess the impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after total cavopulmonary connection. METHODS: We conducted a retrospective analysis of 100 consecutive patients who underwent total cavopulmonary connection from 2010 to 2014. All patients had previously undergone bidirectional cavopulmonary anastomosis. These patients were classified into two groups according to age at bidirectional cavopulmonary anastomosis: younger group, 6 months (n=67). RESULTS: The proportion of hypoplastic left heart syndrome was higher in the younger group (48 versus 4%). After total cavopulmonary connection, the chest tube period was longer in the younger group (10.1±6.6 versus 6.7±4.5 days; p=0.009). Catheterisation 6 months after total cavopulmonary connection revealed that pulmonary artery pressure was higher (11.5±1.9 versus 10.4±2.1 mmHg; p=0.017) and Nakata index was lower (219±79 versus 256±70 mm2/m2; p=0.024) in the younger group. In patients with a non-hypoplastic left heart syndrome, there was no difference in post-operative haemodynamics between two groups, but the total amount of chest drainage after total cavopulmonary connection was larger in the younger group (109±95 versus 55±40 ml/kg; p=0.044). CONCLUSIONS: Early bidirectional cavopulmonary anastomosis did not affect the outcome of total cavopulmonary connection. Longer chest tube period, smaller pulmonary artery, and higher pulmonary artery pressure after total cavopulmonary connection were recognised in early bidirectional cavopulmonary anastomosis patients, especially in hypoplastic left heart syndrome.