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Background: Neonatal myocardial infarction is rare and is associated with a high mortality of 40% to 50%. We report our experience with neonatal myocardial infarction, including presentation, management, outcomes, and our current patient management algorithm. Methods: We reviewed all infants admitted with a diagnosis of coronary artery thrombosis, coronary ischemia, or myocardial infarction between January 2015 and May 2021. Results: We identified 21 patients (median age, 1 [interquartile range (IQR), 0.25–9.00] day; weight, 3.2 [IQR, 2.9–3.7] kg). Presentation included respiratory distress (16), shock (3), and murmur (2). Regional wall motion abnormalities by echocardiogram were a key criterion for diagnosis and were present in all 21 with varying degrees of depressed left ventricular function (severe [8], moderate [6], mild [2], and low normal [5]). Ejection fraction ranged from 20% to 54% (median, 43% [IQR, 34%–51%]). Mitral regurgitation was present in 19 (90%), left atrial dilation in 15 (71%), and pulmonary hypertension in 18 (86%). ECG was abnormal in 19 (90%). Median troponin I was 0.18 (IQR, 0.12–0.56) ng/mL. Median BNP (B-type natriuretic peptide) was 2100 (IQR, 924–2325) pg/mL. Seventeen had documented coronary thrombosis by cardiac catheterization. Seventeen (81%) were treated with intracoronary tPA (tissue-type plasminogen activator) followed by systemic heparin, AT (antithrombin), and intravenous nitroglycerin, and 4 (19%) were treated with systemic heparin, AT, and intravenous nitroglycerin alone. Nineteen of 21 recovered. One died (also had infradiaphragmatic total anomalous pulmonary venous return). One patient required a ventricular assist device and later underwent heart transplant; this patient was diagnosed late at 5 weeks of age and did not respond to tPA. Nineteen of 21 (90%) regained normal left ventricular function (ejection fraction, 60%–74%; mean, 65% [IQR, 61%–67%]) at latest follow-up (median, 6.8 [IQR, 3.58–14.72] months). Two of 21 (10%) had residual trivial mitral regurgitation. After analysis of these results, we present our current algorithm, which developed and matured over time, to manage neonatal myocardial infarction. Conclusions: We experienced a lower mortality rate for infants with neonatal infarction than that reported in the literature. We propose a post hoc algorithm that may lead to improvement in patient outcomes following coronary artery thrombus.

A 33-week gestation, 1.75-kg female infant with mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome and severe ventriculo-coronary connections underwent surgical septectomy and bilateral pulmonary artery banding at five weeks of age (2.10 kg). After separation from bypass, she developed hemodynamic instability requiring venoarterial extracorporeal membrane oxygenation support. She was listed for heart transplantation and transplanted after three days of support with an oversized heart (4.7:1 donor–recipient weight ratio).

Introduction Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics. Methods Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic resonance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modified Ishak congestive hepatic fibrosis score. Results Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tunnel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64). Conclusion Noninvasive tests, such as MRE, may be suitable for longitudinal follow-up in patients with single ventricle physiology. Our data suggest that there is reasonable correlation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis.

Introduction: Recent studies have suggested a relationship between mortality and center volume in pediatric heart transplantation; however, these studies utilize databases that lack granularity to assess patient risk profiles. Hypothesis: Utilizing the Pediatric Heart Transplant Society (PHTS) database, we hypothesized that there would be a difference in outcomes among centers based on yearly transplant volume, and that the patient’s clinical profiles would differ by center volume. Methods: We performed a retrospective analysis of the PHTS dataset from all pediatric (≤ 18 years of age) cases from 2009-2018. Centers were stratified into 5 groups based on annual volume: ≤ 4, 4-6, 6-9, 9-14, and > 14 transplants. We evaluated specific variables, including those identified as significant risk factors in previous PHTS studies including mechanical ventilation, PRA > 10%, high BUN, gender, and Fontan completion, within each group. We used Kaplan Meier for survival, contingency tables with Pearson Chi-square test for association, and analysis-of-variance to compare means. P< 0.05 was considered statistically significant. Results: There were 6800 cases among 55 centers. Table 1 demonstrates that there was no significant difference in one-year post transplant survival among the groups, and the patient variables showing statistically significant differences followed no specific pattern among the center volume groups. It also demonstrates that there is no clear association between cumulative risk factors within individual patients and center volume. Conclusions: One-year post transplant survival is similar within the PHTS for all centers regardless of case volume. These group differences were different for different variables and did not segregate consistently between lower volume and higher volume groups suggesting there exists a complicated relationship among risk factors and center volume.

Background Right ventricular outflow tract (RVOT) stents have been used as palliation in patients with severe tetralogy of Fallot (TOF). Radiofrequency perforation of the RVOT has also been described in patients with pulmonary atresia (PA)/ventricular septal defect (VSD). However, RVOT stenting in conjunction with radiofrequency perforation as a means for establishing reliable pulmonary blood flow in patients with PA/VSD has not previously been reported. Objectives Our aim is to report our experience with using perforation of plate-like pulmonary valve atresia combined with stenting of RVOT as an alternative and equally efficacious intervention for infants with PA/VSD, as compared to a surgical pulmonary artery shunt (SPS). Methods A retrospective review of patients with PA/VSD at our institution from Jan 2006 to October 2015 was performed. Results Twenty-seven patients received palliation for PA/VSD. Five (median 2.5 kg, 2.1-4.1 kg) underwent RVOT stenting and 22 (3.5 kg, 2.1-4.6 kg) underwent surgical SPS. Of the patients who received an RVOT stent, 3 underwent guidewire perforation and balloon dilation of the plate-like pulmonary valve, and 2 underwent radiofrequency perforation of the pulmonary plate, followed by balloon dilation. Postprocedure saturations averaged 93% in the stent group and 89% in the shunt group. One of the patients who underwent RVOT stenting required additional stenting of the PDA for isolation of the LPA, but none required re-intervention. Four patients in the SPS cohort required re-intervention (18%). Two required stenting of the shunt, and 2 required surgical revision of the shunt. There was one death in the stent group, however this was secondary to withdrawal of support given an underlying genetic diagnosis (trisomy 13). There was one fatality in the SPS cohort prior to hospital discharge. Conclusions Perforation followed by RVOT stenting may be a safe and effective alternative to surgical SPS in infants with PA/VSD.

Outcomes of ACR after pediatric HTx have been well described, but less has been reported on outcomes of AMR. We compared the clinical characteristics and cardiovascular outcomes (composite end-point of death, retransplantation, or allograft vasculopathy) of pediatric HTx recipients with AMR, ACR, and no rejection in a retrospective single-center study of 104 recipients. Twenty were treated for AMR; 15 were treated for ACR. Recipients with AMR had an increased frequency of congenital heart disease (90% vs ACR 67% vs no rejection 59%, P = .03), homograft (68% vs 7% vs 18%, P < .001), HLA sensitization (45% vs 13% vs 13%, P = .008), and positive cross-match (30% vs 7% vs 9%, P = .046). AMR caused hemodynamic compromise more often than ACR (39% vs 4%, P = .02). AMR recipients had worse cardiovascular outcome than recipients with ACR or no rejection (40% vs 20% vs 8.6%, P = .003). In bivariate Cox analysis, AMR (HR 4.1, CI 1.4-12.0, P = .009) and ischemic time (HR 1.6, CI 1.1-2.3, P = .02) were associated with worse cardiovascular outcome; ACR was not. In summary, pediatric HTx recipients who develop AMR have worse cardiovascular outcome than recipients who develop only ACR or experience no rejection at all.

Background Systemic-to-pulmonary artery shunt (SPS) dysfunction can be deleterious in shunt dependent patients and traditionally have undergone surgical revision. Data on transcatheter stenting of SPS is limited. We sought to evaluate feasibility, safety and outcomes of stenting SPS. Methods Retrospective review of all patients who underwent transcatheter SPS stenting from 1/2006 to 12/2013. Results Of 229 surgically implanted SPS, 25 transcatheter stent interventions were performed in 22 patients. The majority had pulmonary atresia (n = 9) or HLHS (n = 10). Their median age was 4 ms (range 10 days to 4 years) and median weight 4.9 kg (range 3–14). Nine had a central and 15 had a BT shunt with a median shunt size of 3.75 mm (range 3–6). The interval from shunt placement to intervention was 1.9 ms (range 4 days–3.8 years). The indication for intervention was increasing cyanosis in10 patients and delaying final repair in 9. Two patients were on ECMO at the time of intervention. The median shunt diameter increased from 2.3 to 4.1 mm and oxygen saturation from 72 to 85% (P

Objectives We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS). Background Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection. Methods A retrospective review of patients with PAIVS at our institution from Jan 2006 to Dec 2013 was performed. Demographic, echocardiographic, procedural, and follow-up data were reviewed and analyzed. Results Twenty-nine patients were identified. Thirteen underwent DS and 16 underwent SPS. In the DS group, 6 had radiofrequency perforation of the pulmonary valve, and 12 underwent balloon pulmonary valvuloplasty (BPV) prior to DS. SPS was performed after perforation of the pulmonary valve in 1 patient and after BPV in 4 patients. Procedural success was 92.8% in the DS group versus 93.3% in the SPS group. In the DS group, one patient required emergency shunting due to intra-procedural ductal spasm. In the SPS group there was one procedure related death, and there were six significant procedure-related complications. Five patients required re-interventions prior to hospital discharge. The median length of hospital stay for the DS versus SPS group was 10 versus 23 days, respectively (P

We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS).Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection.A retrospective review of patients with PAIVS at our institution from Jan 2006 to Dec 2013 was performed. Demographic, echocardiographic, procedural, and follow-up data were reviewed and analyzed.Twenty-nine patients were identified. Thirteen underwent DS and 16 underwent SPS. In the DS group, 6 had radiofrequency perforation of the pulmonary valve, and 12 underwent balloon pulmonary valvuloplasty (BPV) prior to DS. SPS was performed after perforation of the pulmonary valve in 1 patient and after BPV in 4 patients. Procedural success was 92.8% in the DS group versus 93.3% in the SPS group. In the DS group, one patient required emergency shunting due to intra-procedural ductal spasm. In the SPS group there was one procedure related death, and there were six significant procedure-related complications. Five patients required re-interventions prior to hospital discharge. The median length of hospital stay for the DS versus SPS group was 10 versus 23 days, respectively (P 0.05). Post discharge, there were seven reinterventions in the DS group and two reinterventions in the SPS group.DS may provide a safe and effective alternative to SPS in selected patients with PAIVS.