Surgical Management of Coronary Artery Fistulas in Children.

Background: This study investigated the surgical outcomes associated with coronary artery fistulas (CAFs) in children. Methods: We retrospectively reviewed the medical records of 23 pediatric patients who underwent surgical closure of CAFs between 1995 and 2021. At presentation, 7 patients (30.4%) exhibited symptoms. Associated cardiac anomalies were present in 8 patients. Fourteen fistulas originated from the right coronary artery and 9 from the left. The most common drainage site was the right ventricle, followed by the right atrium and the left ventricle. The median follow-up duration was 9.3 years (range, 0.1-25.6 years). Results: The median age and body weight at repair were 3.1 years (range, 0-13.4 years) and 14.4 kg (range, 3.1-42.2 kg), respectively. Cardiopulmonary bypass was used in 17 cases (73.9%), while cardioplegic arrest was employed in 14 (60.9%). Epicardial CAF ligation was utilized in 10 patients (43.5%), the transcoronary approach in 9 (39.1%), the endocardial approach in 2 (8.7%), and other methods in 2 patients (8.7%). The application of cardioplegic arrest during repair did not significantly impact the duration of postoperative intensive care unit stay or overall hospital stay. One in-hospital death and 1 late death were recorded. The overall survival rate was 95.7% at 10 years and 83.7% at 15 years. A residual fistula was detected in 1 patient. During the follow-up period, no surviving patient experienced cardiovascular symptoms or coronary events. Conclusion: Surgical repair of CAF can be performed safely with or without cardioplegic arrest, and it is associated with a favorable prognosis in children.

Outcomes after Mechanical Aortic Valve Replacement in Children with Congenital Heart Disease.

Background: The optimal choice of valve substitute for aortic valve replacement (AVR) in pediatric patients remains a matter of debate. This study investigated the outcomes following AVR using mechanical prostheses in children. Methods: Forty-four patients younger than 15 years who underwent mechanical AVR from March 1990 through March 2023 were included. The outcomes of interest were death or transplantation, hemorrhagic or thromboembolic events, and reoperation after mechanical AVR. Adverse events included any death, transplant, aortic valve reoperation, and major thromboembolic or hemorrhagic event. Results: The median age and weight at AVR were 139 months and 32 kg, respectively. The median follow-up duration was 56 months. The most commonly used valve size was 21 mm (14 [31.8%]). There were 2 in-hospital deaths, 1 in-hospital transplant, and 1 late death. The overall survival rates at 1 and 10 years post-AVR were 92.9% and 90.0%, respectively. Aortic valve reoperation was required in 4 patients at a median of 70 months post-AVR. No major hemorrhagic or thromboembolic events occurred. The 5- and 10-year adverse event-free survival rates were 81.8% and 72.2%, respectively. In univariable analysis, younger age, longer cardiopulmonary bypass time, and smaller valve size were associated with adverse events. The cut-off values for age and prosthetic valve size to minimize the risk of adverse events were 71 months and 20 mm, respectively. Conclusion: Mechanical AVR could be performed safely in children. Younger age, longer cardiopulmonary bypass time and smaller valve size were associated with adverse events. Thromboembolic or hemorrhagic complications might rarely occur.

Extracorporeal Cardiopulmonary Resuscitation in Infants: Outcomes and Predictors of Mortality.

Background: Extracorporeal cardiopulmonary resuscitation (E-CPR) plays an indispensable role when resuscitation fails; however, extracorporeal life support (ECLS) in infants is different from that in adults. The objective of this study was to evaluate the outcomes of E-CPR in infants. Methods: A single-center retrospective study was conducted, analyzing 51 consecutive patients (age <1 year) who received E-CPR for in-hospital cardiac arrest between 2010 and 2021. Results: The median age and body weight was 51 days (interquartile range [IQR], 17-111 days) and 3.4 kg (IQR, 2.9-5.1 kg), respectively. The cause of arrest was cardiogenic in 45 patients (88.2%), and 48 patients (94.1%) had congenital cardiac anomalies. The median conventional cardiopulmonary resuscitation (C-CPR) time before the initiation of ECLS was 77 minutes (IQR, 61-103 minutes) and duration of ECLS was 7 days (IQR, 3-12 days). There were 36 in-hospital deaths (70.6%), and another patient survived after heart transplantation. In the multivariate analysis, single-ventricular physiology (odds ratio [OR], 5.05; p=0.048), open sternum status (OR, 8.69; p=0.013), and C-CPR time (OR, 1.47 per 10 minutes; p=0.021) were significant predictors of in-hospital mortality. In a receiver operating characteristic curve, the optimal cut-off of C-CPR time was 70.5 minutes. The subgroup with early E-CPR (C-CPR time <70.5 minutes) showed a tendency for lower in-hospital mortality tendency (54.5% vs. 82.8%, p=0.060), albeit not statistically significant. Conclusion: If resuscitation fails in an infant, E-CPR could be a life-saving option. It is crucial to improve C-CPR quality and shorten the time before ECLS initiation.

Characteristics of Patients with Surgical Closure of an Atrial Septal Defect during Infancy.

Background: Surgical closure of an atrial septal defect (ASD) is infrequently indicated during infancy. We evaluated the clinical characteristics and outcomes of patients who underwent surgical ASD closure during infancy. Methods: A single-center retrospective review was performed for 39 patients (19 males) who underwent surgical ASD closure during infancy between 1993 and 2020. The median body weight percentile at the time of operation was 9.3. Results: During a median follow-up of 60.9 months, 4 late deaths occurred due to chronic respiratory failure. A preoperative history of bronchopulmonary dysplasia (BPD) was the only risk factor for late mortality identified in Cox regression (hazard ratio, 3.54; 95% confidence interval [CI], 1.75-163.04; p=0.015). The 5-year survival rate was significantly lower in patients with preoperative history of BPD (97.0% vs. 50.0%, p<0.001) and preoperative ventilatory support (97.1% vs. 40.4%, p<0.001). There were significant postoperative increases in left ventricular end-diastolic (p=0.017), end-systolic (p=0.014), and stroke volume (p=0.013) indices. A generalized estimated equation model showed significantly better postoperative improvement in body weight percentiles in patients with lower weight percentiles at the time of operation (<10th percentile, p=0.01) and larger indexed ASD diameter (≥45 mm/m2, p=0.025). Conclusion: Patients with ASD necessitating surgical closure during infancy are extremely small preoperatively and remain small even after surgical closure. However, postoperative somatic growth was more prominent in smaller patients with larger defects, which may be attributable to an increase in postoperative cardiac output due to changes in ventricular septal configuration. The benefits of ASD closure in patients with BPD are undetermined.

Outcomes of Arterial Switch Operation With Aortic Arch Reconstruction.

BACKGROUND: This study investigated the outcomes and factors associated with reintervention or development of significant pulmonary stenosis (PS) after repair of transposition of the great arteries (TGA) or Taussig-Bing anomaly (TBA) with aortic arch obstruction. METHODS: A total of 51 patients with TGA or TBA who underwent an arterial switch operation and aortic arch reconstruction between 2004 and 2020 were included. The outcomes of interest were all-cause death, including heart transplantation, all-cause reintervention, right-sided reintervention, and development of significant PS. RESULTS: The median age and body weight at repair were 9 days and 3.2 kg, respectively. Forty-nine patients (96.1%) underwent 1-stage repair. A total of 28 patients (54.9%) had TBA, and 8 patients (15.7%) had interrupted aortic arch. There were 5 early deaths (9.8%) and 2 late deaths during a median follow-up duration of 59 months. The transplant-free survival rate 10 years after repair was 82.6%. A total of 21 reinterventions were required in 10 patients. The significant PS-free survival rate 10 years after repair was 68.8%. In univariable analysis, a higher ratio of the diameter of the main pulmonary artery to the ascending aorta was associated with all-cause reintervention (P = .007) and right-sided reintervention (P = .002). A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS (P = .049). CONCLUSIONS: The rates of overall mortality and reintervention after repair were not negligible. A higher degree of size discrepancy between the 2 great arteries was associated with all-cause or right-sided reintervention. A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS.

The Usefulness of Computed Tomography in Predicting Left Ventricular Outflow Tract Obstruction After Neonatal Arch Repair.

This study investigated the outcome after neonatal arch repair, and the usefulness of computed tomography (CT) in predicting the development of left ventricular outflow tract (LVOT) obstruction (LVOTO). A total of 150 neonates who underwent arch repair between 2008 and 2019 were included. The diameters of the aortic valve annulus (AVA) and LVOT in millimeters were measured with transthoracic echocardiography (TTE) or CT and indexed by subtracting body weight in kilograms. The outcomes of interest were the development of LVOTO (peak flow velocity > 3 m/s on TTE) and reintervention or reoperation for LVOTO. The median follow-up duration was 3.6 years. The rates of overall survival, freedom from reintervention for LVOTO, and freedom from the LVOTO development at 7 years were 93.7%, 88.2%, and 81.0%, respectively. In univariable Cox regression analysis, weight-indexed CT-measured LVOT diameter (concordance index [C-index] = 0.73, P = 0.002) and weight-indexed TTE-measured AVA diameter (C-index = 0.69, P = 0.001) were significant predictors of LVOTO. The maximal chi-square test identified the following cutoff values for predicting LVOTO: 1.4 for weight-indexed CT-measured LVOT diameter and 1.6 for weight-indexed TTE-measured AVA diameter. The high-risk group (both measures lower than the cutoff values) had a significantly lower rate of freedom from LVOTO development than the low-risk group (both measures higher than the cutoff values) (P < 0.001). The weight-indexed CT-measured LVOT diameter could be used to predict LVOTO development after neonatal arch repair, as an independent measure or complementary to traditional measures.

Long-term Surgical Outcomes of Supravalvar Aortic Stenosis: Modified Simple Sliding Aortoplasty.

This study investigated long-term outcomes and factors associated with reoperations in patients who underwent surgical repair of congenital supravalvar aortic stenosis (SVAS). A total of 39 consecutive patients who underwent congenital SVAS repair from 1999 through 2018 were included. Aortic root geometry was evaluated by measuring the ratio of the sinotubular junction diameter to the aortic annulus diameter (STJ/AVA) on echocardiography and proportion of intercommissural distance (ICD) of each sinus on computed tomography. The median age and weight at the time of operation were 4.3 years and 16.9 kg, respectively. Williams syndrome was associated in 25 patients (64.1%). Modified simple sliding aortoplasty (MSSA) was mostly used (n = 35, 89.7%). The median follow-up duration was 9.5 years. There were no early deaths and 1 late death. Overall survival rate was 97.0% at 15 years. There were 7 reoperations during follow-up. Freedom from reoperation for left ventricular outflow tract obstruction and all-cause reoperation were 91.9% and 80.4%, respectively. Age younger than 2 years at initial repair were associated with all-cause reoperation in the univariable analysis. In 35 patients who underwent MSSA, the degree of aortic regurgitation was equal to or less than mild in all patients during follow-up. Their median STJ/AVA on postoperative echocardiography was 0.95 (0.84-1.02). SVAS repair with MSSA provided excellent long-term survival with well-preserved aortic valve competence. Age younger than 2 years at initial repair might be associated with reoperation.

Outcomes of pulmonary artery sling repair without tracheoplasty.

OBJECTIVE: This study aimed to investigate surgical outcomes of pulmonary artery (PA) sling without tracheoplasty. METHODS: From 2001 through 2020, among 22 patients who underwent PA sling repair, all but 1 patient who underwent concomitant tracheal surgery were analyzed. The outcomes of interest were all-cause death, PA reintervention, tracheal intervention, and readmission for respiratory symptoms. Computed tomography was used to measure the narrowest tracheal diameter. RESULTS: The median age and weight at repair were 7.6 months and 7.7 kg, respectively. Most patients (20 out of 21, 95.2%) had preoperative respiratory symptoms. Associated airway anomalies included tracheal ring in 12 (57.1%), bridging bronchus in 8 (38.1%), and tracheal bronchus in 2 patients (9.5%). There was 1 in-hospital death (4.8%). The median ventilator time and intensive care unit stay were 23 hours and 3 days, respectively. There was neither late death nor tracheal intervention during follow-up. Five patients (25.0%) underwent reintervention for left PA stenosis. Hospital readmission for respiratory symptom was required in 7 patients and was associated with the narrowest preoperative tracheal diameter (P = .025) and cardiopulmonary bypass time (P = .040) in univariable analysis. The narrowest tracheal diameter of 3.4 mm was identified as a cutoff value for readmission for respiratory symptom. Freedom from readmission for respiratory symptom was 63.3% at 10 years. CONCLUSIONS: PA sling repair without tracheal surgery might be a reasonable surgical option with rare need for tracheal intervention. Hospital readmissions for respiratory symptoms are more frequently required in patients with smaller tracheal diameter and all readmissions were limited to within 2 years after repair.

Risk factors for early adverse outcomes after bovine jugular vein conduit implantation: influence of oversized conduit on the outcomes.

OBJECTIVES: We investigated potential risk factors for early failure of bovine jugular vein conduit (Contegra®) implantation for right ventricular outflow tract (RVOT) reconstruction. METHODS: A single-centre retrospective review of 115 consecutive patients (54 males) who underwent RVOT reconstruction with Contegra between 2016 and 2019 was performed. Overall survival, explantation-free survival and freedom from significant RVOT lesions (valve regurgitation ≥ moderate or flow velocity ≥3.5 m/s) were investigated. RESULTS: Median age, body weight and Contegra diameter were 10.3 months [interquartile range (IQR) 5.7-26.9 months], 7.8 kg (IQR 6.3-12.4 kg) and 14 mm (IQR 12-16 mm), respectively. During the median follow-up duration of 25.1 months, there were 7 deaths, 34 significant RVOT lesions, 10 endocarditis episodes and 15 explantations. Overall survival and explantation-free survival at 3 years were 94.8% and 78.4%, respectively. Significant RVOT lesions (n = 34) comprised 20 stenoses, 8 regurgitations and 6 combined lesions. Freedom from significant RVOT lesions at 3 years was 62.6%. Cox regression identified higher indexed Contegra size (Contegra diameter/body weight, mm/kg) as the only risk factor for decreased time to explantation or death (hazard ratio 2.32, P < 0.001) and time to significant RVOT lesions development (hazard ratio 2.75, P < 0.001). The cut-off value of indexed Contegra size for significant RVOT lesions at 12 months was 1.905 mm/kg (sensitivity, 0.75; specificity, 0.78; area under the curve, 0.82). CONCLUSIONS: Outcomes of RVOT reconstruction using Contegra were acceptable. However, oversized Contegra should be avoided when possible. IRB APPROVAL DATE: 26 October 2020. IRB REGISTRATION NUMBER: S2020-2505-0001.

Contemporary Outcomes After Pulmonary Artery Banding in Complete Atrioventricular Septal Defect.

BACKGROUND: This study investigated the clinical outcomes and the effect of band tightness on outcome after pulmonary artery banding (PAB) in patients with complete atrioventricular septal defect (AVSD). METHODS: From 2000 through 2019, among 133 patients with isolated complete AVSD pursuing biventricular repair, 34 patients (25.6%) who underwent PAB were included in this study. Factors associated with adverse outcome, which was defined as prolonged stay in the intensive care unit (>10 days), were analyzed using a multiple logistic regression model. Receiver operating characteristic analysis was performed to identify a threshold band tightness for adverse outcome. RESULTS: The median age and weight were 43 days and 3.6 kg, respectively. There were 4 early deaths. The median intensive care unit stay was 8 days. Twenty-eight patients (28/34; 82.4%) underwent definitive repair 10 months (interquartile range, 7-12 months) after PAB. In multivariable analysis, indexed band diameter was identified as a factor associated with adverse outcome (odds ratio, 1.60; 95% CI, 1.03-2.48; P = .035). Receiver operating characteristic analysis indicated 22.2 mm/m2 of indexed PAB diameter measured at discharge as a threshold band tightness for adverse outcome (area under curve, 0.871; P < .001). The level of B-type natriuretic peptide similarly decreased after PAB regardless of band tightness, although the probability of worsening in atrioventricular valve regurgitation was significantly decreased in patients with a tighter band (P = .027). CONCLUSIONS: PAB is a viable option for early-presenting patients with complete AVSD. Tighter PAB might be beneficial for early postoperative outcomes and preventing progression of atrioventricular valve regurgitation in complete AVSD.

The impact of a bicuspid pulmonary valve in the aortic position after arterial switch for transposition of the great arteries on neoaortic root dimension and function: a propensity score matched analysis.

OBJECTIVES: This study investigated the effect of a bicuspid pulmonary valve on neoaortic root morphology, function, and the clinical outcomes of early survivors after the arterial switch operation using propensity score matching. METHODS: From 1997 to 2018, a total of 442 patients underwent the arterial switch operation for transposition of the great arteries. After exclusion of patients who underwent a staged repair, were repaired beyond 1 year of age, died before discharge and who lacked echocardiographic data at discharge, propensity score matching was used for analysis. RESULTS: Among 352 eligible patients, 18 patients (5.1%) had a bicuspid pulmonary valve. After propensity score matching (1:4), 15 patients with bicuspid valve (bicuspid group) and 60 patients with tricuspid valve (tricuspid group) were enrolled. The median follow-up duration was 9.9 years (4 months∼22.3 years). All-cause reoperation-free survival at 10 years was 93.3% in the bicuspid group and 87.0% in the tricuspid group (P = 0.839), and reoperation for neoaortic valve or root was required in 2 patients in the bicuspid group and 1 in the tricuspid group without intergroup difference. The z-score of the neoaortic annulus did not change in either group, although there was an increasing tendency in the z-score of the neoaortic sinus without intergroup difference (P = 0.690). Deterioration in neoaortic valve function was more prominent in the bicuspid group (p = 0.028). CONCLUSIONS: The neoaortic sinus might outgrow the norm regardless of the number of neoaortic valve cusps, whereas the neoaortic annulus remained unchanged. Deterioration of valve function was more prominent in the bicuspid group, which suggests that a bicuspid valve might play a significant role in deterioration of neoaortic valve function, without an additional effect on root pathology.

Surgical Outcomes of Cor Triatriatum Sinister: A Single-Center Experience.

BACKGROUND: We investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS). METHODS: Thirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed. RESULTS: The median age and body weight at operation were 9 months (interquartile range [IQR], 3-238 months) and 7.5 kg (IQR, 5.8-49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39-195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6-112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg). CONCLUSION: Surgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.

Resolution of Postnatal Shock Before Open Heart Surgery in Neonates With Prenatal Diagnoses.

BACKGROUND: The impact of a prenatal diagnosis (PreND) for congenital heart disease on outcomes after neonatal open heart surgery is undetermined. We hypothesized that PreND has a positive impact on surgical outcomes in terms of immediate postnatal intensive care, which may lead to a decreased risk of persistent shock before surgery. METHODS: Among the 949 neonates who underwent open heart surgery between January 2002 and December 2017, 655 patients (69.0%) were diagnosed prenatally (group-PreND) and 294 patients (31.0%) were diagnosed postnatally (group-PostND). Procedural complexity, incidence of postnatal shock (serum lactate >4.0 mmol/L or pH <7.2), hospitalization length of stay, duration of shock, resolution of shock, and in-hospital mortality were compared between the 2 groups. RESULTS: In group-PreND, the procedure-dependent comprehensive Aristotle score (10.8 vs 10.0, P < .001), incidence of extracardiac anomalies (13.0% vs 7.1%, P = .008), heterotaxy syndrome (3.8% vs. 1.0%, P = .021), and postnatal shock (244 of 655 [37.3%] vs 78 of 294 [26.5%], P = .001) were higher than in group-PostND. However, patients in group-PreND were hospitalized earlier after birth (0 day vs 5 days, P < .001), experiencing shorter duration of shock (5.3 hours vs 9.0 hours, P = .01), and, consequently, showing higher incidence of shock resolution (212 of 244 [87%] vs 52 of 78 [67%], P < .001). In-hospital mortality was comparable between the 2 groups (P = .070). CONCLUSIONS: Postnatal shock is more frequently observed in group-PreND. However, prenatal awareness of the disease leads to immediate postnatal initiation of intensive care with shorter exposure to shock, leading to higher probability of shock resolution.

Paediatric heart transplantation recipients ≥7 years of age receiving donors with pre-existing coronary atherosclerosis showed progressive coronary artery disease.

BACKGROUND: This study aimed to determine the effect of donor-transmitted atherosclerosis on the late aggravation of cardiac allograft vasculopathy in paediatric heart recipients aged ≥7 years. METHODS: In total, 48 patients were included and 23 had donor-transmitted atherosclerosis (baseline maximal intimal thickness of >0.5 mm on intravascular ultrasonography). Logistic regression analyses were performed to identify risk factors for donor-transmitted atherosclerosis. Rates of survival free from the late aggravation of cardiac allograft vasculopathy (new or worsening cardiac allograft vasculopathy on following angiograms, starting 1 year after transplantation) in each patient group were estimated using the Kaplan-Meier method and compared using the log-rank test. The effect of the results of intravascular ultrasonography at 1 year after transplantation on the late aggravation of cardiac allograft vasculopathy, correcting for possible covariates including donor-transmitted atherosclerosis, was examined using the Cox proportional hazards model. RESULTS: The mean follow-up duration after transplantation was 5.97 ± 3.58 years. The log-rank test showed that patients with donor-transmitted atherosclerosis had worse survival outcomes than those without (p = 0.008). Per the multivariate model considering the difference of maximal intimal thickness between baseline and 1 year following transplantation (hazard ratio, 22.985; 95% confidence interval, 1.948-271.250; p = 0.013), donor-transmitted atherosclerosis was a significant covariate (hazard ratio, 4.013; 95% confidence interval, 1.047-15.376; p = 0.043). CONCLUSION: Paediatric heart transplantation recipients with donor-transmitted atherosclerosis aged ≥7 years had worse late cardiac allograft vasculopathy aggravation-free survival outcomes.

Development of Cardiac Events and Functional Recovery Prediction Models for Pediatric Dilated Cardiomyopathy.

Background: Since both the risk of death and the probability of spontaneous functional recovery (FR) coexist in association with pediatric dilated cardiomyopathy (DCMP), management should be based on individualized outcome predictions. Methods: A single-center retrospective review of 105 pediatric patients (age at presentation ≤ 18 years) with DCMP, managed between 1994 and 2017, was performed. Logistic regression was conducted to identify variables associated with FR and cardiac events (CEs), i.e., death or heart transplantation (HTPL), within 2 years after initial presentation. Two outcome prediction models were formulated using these variables. Results: Twenty-six (24.8%) and 51 patients (48.6%) experienced FR and CE, respectively, within 2 years after initial presentation. Predictors of mortality without HTPL were earlier era at presentation (HR: 4.13; 95% CI: 1.88-9.06; p < 0.001) and significant TR (≥moderate; HR: 4.31; 95% CI: 1.26-14.77; p = 0.020) in multivariable Cox regression model. Predictors of FR were recent era (HR: 4.49; 95% CI: 1.40-14.44; p = 0.0012), younger age at initial presentation (HR: 0.98 per 1 month increase; 95% CI: 0.97-0.99, p < 0.001), post-myocarditis DCMP (HR: 4.29; 95% CI: 1.32-13.93; p = 0.015), and arrhythmia-mediated DCMP (HR: 26.88; 95% CI: 2.61-276.70; p = 0.006). Risk factors for CEs was idiopathic DCMP (HR: 2.95; 95% CI: 1.32-6.56, p = 0.008). The low-risk group who had higher probability of FR than CE in prediction model had a slightly higher overall survival rate (71.4 vs. 52.2% at 10 years after presentation; log-rank p = 0.09) and a significantly higher HTPL-free survival rate (67.5 vs. 24.9% at 10 years after presentation; log-rank p < 0.001) than the high-risk group. Conclusions: Prognostication and management strategies for pediatric DCMP may be enhanced by risk stratification using outcome prediction modeling.

Growth of the Branch Pulmonary Arteries After Employing 'Shunt-Only' Strategy for Neonates With Pulmonary Atresia or Stenosis.

We sought to determine the impact of juxtaductal stenosis (JDS) on branch pulmonary artery (PA) growth after systemic-to-pulmonary shunt (SPS) placement without pulmonary arterioplasty (ie the 'shunt-only' strategy). This was a retrospective review of 91 patients, 54 (59.3%) with pulmonary atresia and 27 (29.7%) with a functionally single ventricle, who underwent neonatal SPS placement without pulmonary arterioplasty between 2008 and 2017. The median age and body weight at SPS procedure were 16 day's (interquartile range [IQR], 11-22) and 3.10 kg (IQR: 2.85-3.40), respectively. All patients had pre-SPS computed tomography (CT) followed by post-SPS CT at a median interval of 5.8 months' (IQR: 4.5-7.2). The ratio of the diameters of the juxtaductal PA over the non-SPS-side hilar PA (JD and/or PA) on preoperative CT-a surrogate for JDS severity-was 0.93 (IQR: 0.67-1.09). The median diameter (Z) of the SPS-side and non-SPS-side PA on postoperative CT were 1.0 (IQR: -0.07-1.73) and 0.99 (IQR: -0.45-1.70), respectively. The pulmonary artery index (Nakata index) increased significantly from 124.0 ± 50.2 mm2/m2 to 240.8 ± 88.7 mm2/m2 (P < .001). Unplanned surgical interventions on the non-SPS-side PA were performed on 7 patients. Logistic regression identified lower preoperative JD and/or PA as a risk factor for unplanned intervention on the non-SPS-side PA (odds ratio, 1.27 per 0.1 decrease; 95% confidence interval, 1.10-2.16, P = 0.025). PA growth on the non-SPS side is generally adequate without pulmonary arterioplasty among patients with JDS. However, unplanned interventions for the non-SPS-side PA are caveats for 'shunt-only' strategy in neonates with significant JDS.

Pulmonary valve replacement following repair of tetralogy of Fallot: comparison of outcomes between bio- and mechanical prostheses.

OBJECTIVES: The aims of this study were to evaluate and compare the outcomes after pulmonary valve replacement (PVR) with a mechanical prosthesis (MP) and a bioprosthesis (BP). METHODS: From 2004 through 2017, a total of 131 patients, who had already been repaired for tetralogy or Fallot or its variants, underwent their first PVR with an MP or a BP. Outcomes of interests were prosthesis failure (stenosis >3.5 m/s, regurgitation >mild or infective endocarditis) and reintervention. RESULTS: The median age at PVR was 19 years. BP and MP were used in 88 (67.2%) and 43 (32.8%) patients, respectively. The median follow-up duration was 7.4 years, and the 10-year survival rate was 96.4%. Risk factors for prosthesis failure were smaller body surface area [hazard ratio (HR) 0.23 per 1 m2, P = 0.047] and smaller prosthesis size (HR 0.73 per 1 mm, P = 0.039). Risk factors for prosthesis reintervention were smaller body surface area (HR 0.11 per 1 m2, P = 0.011) and prosthesis size (HR 0.67 per 1 mm, P = 0.044). Probability of prosthesis failure and reintervention at 10 years were 24.6% (19.5% in BP vs 34.8% in MP, P = 0.34) and 7.8% (5.6% in BP vs 11.9% in MP, P = 0.079), respectively. Anticoagulation-related major thromboembolic events were observed in 4 patients receiving an MP. CONCLUSIONS: MP might not be superior to BP in terms of prosthesis failure or reintervention. MP should be carefully considered for highly selected patients in the era of transcatheter PVR.

The impact of additional antegrade pulmonary blood flow at bidirectional Glenn shunt on long-term outcomes.

OBJECTIVES: We investigated the impact of additional antegrade pulmonary blood flow on the long-term outcomes after bidirectional Glenn shunt. METHODS: From 2001 to 2015, 279 patients underwent bidirectional Glenn shunt as an interim palliation for a functionally single ventricle. After excluding patients with a previous Kawashima or Norwood operation, 202 patients with preexisting antegrade pulmonary blood flow before bidirectional Glenn shunt were included in this study. Antegrade pulmonary blood flow was eliminated in 110 patients (no antegrade pulmonary blood flow group) and maintained in 92 patients (antegrade pulmonary blood flow group). The impact of antegrade pulmonary blood flow at bidirectional Glenn shunt on long-term outcome was analyzed using inverse probability of treatment weighting. RESULTS: Median age and body weight at bidirectional Glenn shunt were 8 months and 7.8 kg, respectively. Prolonged chest tube drainage or readmission for effusion after bidirectional Glenn shunt was more frequent in the antegrade pulmonary blood flow group (odds ratio, 3.067; 95% confidence interval, 1.036-9.073; P = .043). In the no antegrade pulmonary blood flow group, B-type natriuretic peptide level was decreased further until the Fontan operation (P = .012). In the no antegrade pulmonary blood flow group, oxygen saturation was lower just after bidirectional Glenn shunt, although it was increased further until Fontan operation (P < .001), despite still lower oxygen saturation before Fontan operation compared with antegrade pulmonary blood flow group (P < .001). The McGoon ratio was decreased in both groups without intergroup difference, although the McGoon ratio before Fontan operation was higher in the antegrade pulmonary blood flow group (2.3 ± 0.4 vs 2.1 ± 0.4, P = .008). Overall transplant-free survival was worse in the antegrade pulmonary blood flow group (hazard ratio, 2.37; confidence interval, 1.089-5.152; P = .030). CONCLUSIONS: Maintaining antegrade pulmonary blood flow at bidirectional Glenn shunt was beneficial for higher oxygen saturation and larger pulmonary artery size before Fontan operation. However, it was unfavorable for overall transplant-free survival with a sustained higher risk of death or transplant until the elimination of antegrade pulmonary blood flow.

Predictive Parameters of Decreased Left Ventricular Global Longitudinal Strain at 1 Month After Pediatric Heart Transplantation.

Previous reports indicate that the decreased left ventricular global longitudinal strain (LVGLS) seen in the early postoperative period of pediatric heart transplant patients generally recovers over the course of 1-2 years. In this study, we investigate the predictive capacity of preoperative parameters on the LVGLS decline seen at 1 month post transplant. Forty-six transplant subjects with 2D echocardiographic images sufficient for speckle tracking echocardiography were enrolled. We excluded patients diagnosed with cardiac allograft vasculopathy or with an episode of rejection 1 month before or after their echocardiographic examinations. The mean LVGLS was significantly reduced at 1 month when compared to 1 year following transplant (- 15.5% vs. - 19.4%, respectively, p < 0.001). The predictors of LVGLS that decline at 1 month were the LV mass z-score [odds ratio (OR) 1.452; 95% confidence interval (CI) 1.007-2.095, p = 0.046], recipient age (OR 1.124; 95% CI 1.015-1.245, p = 0.025), and donor age (OR 1.081; 95% CI 1.028-1.136, p = 0.002) in the univariate logistic regression analyses. Although multivariate analysis yielded no significant predictors, higher LV mass z-scores showed a trend associated with the decline of LVGLS (p = 0.087). The donor/recipient weight ratio was associated with the LV mass z-score (R2 = 0.412, p < 0.001).