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Background: There are currently limited accurate national estimates for pediatric heart failure (HF)., Objectives: This study aims to describe the current burden of primary and comorbid pediatric HF in the United States., Methods: International Classification of Diseases, Clinical Modification codes were used to identify HF cases and comorbidities from the Kids' Inpatient Database, National Inpatient Sample, National Emergency Department (ED) Sample, and National Vital Statistics System for 2012 and 2016. To describe HF events, all visits/events among pediatric and adult subjects were included in the analysis. HF events were classified into 1 of 3 groups: 1) no HF; 2) primary HF; or 3) comorbid HF. We compared patients with and without HF and calculated unique event rates with age and sex standardization., Results: Congenital heart disease, conduction disorders/arrhythmias, and cardiomyopathy were responsible for the majority of pediatric HF-related ED visits and hospitalizations. Compared to 2012, in 2016, there was an increase in comorbid HF ED visits (rate ratio: 1.93; P < 0.001) and primary HF hospitalizations (rate ratio: 1.14; P = 0.002). Pediatric HF burden was lower compared to adult HF; however, deaths in the ED and in-hospital were significantly more likely in children presenting with HF than adults., Conclusions: The burden of pediatric HF continues to increase. Compared to adults with HF presenting to the ED and in-hospital, outcomes are inferior and per patient resource use is higher for children hospitalized with HF. National initiatives to understand risk factors for morbidity and mortality in pediatric HF and continued surveillance and mitigation of preventable risk factors may attenuate this uptrend., Competing Interests: Funding Support and Author Disclosures Dr Rossano has received personal fees from Amgen, Abiomed, Bayer, Novartis, Cytokinetics, and MyoKardia. Dr Tang is a consultant for Sequana Medical AG, Cardiol Therapeutics Inc, and Genomics plc. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associated liver disease (FALD), has emerged. FALD is an extracardiac complication that may lead to substantial comorbid disease and premature mortality. The risk factors, pathophysiology, longitudinal consequences, and therapeutic options related to FALD remain poorly defined. Although we recognize that Fontan circulatory properties are associated with extracardiac organ dysfunction, numerous gaps in our understanding of the nature of this relationship exist. Such extracardiac manifestations, in addition to other late complications of the circulation, can significantly affect quality of life and healthcare use. Therefore, to initiate a formal evaluation of FALD, the American College of Cardiology (ACC) sponsored a stakeholders meeting on October 1 to 2, 2015, in Washington, DC. The goal of the meeting was to bring together subspecialty experts in the fields of adult and pediatric hepatology, congenital cardiology (adult congenital and pediatric cardiology), heart failure/transplant, epidemiology, and cardiothoracic surgery, as well as patient advocates, patients, parents of children and young adults who have had the Fontan procedure, and research organizations and societies to discuss the current state of FALD. Topics included gaps in knowledge, optimal care, research opportunities and barriers, and sound practices to guide providers, patients, and families. This report summarizes findings from the stakeholders meeting and seeks to establish a platform for understanding and addressing FALD., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking., Objectives: This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time., Methods: Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation., Results: From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R 2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection., Conclusions: Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; NCT00132782)., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: We sought to prospectively evaluate evidence of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery with an interarterial course (AAOCA)., Background: An AAOCA is a rare anomaly associated with increased myocardial ischemia and sudden death risk in children. Data evaluating ischemia after AAOCA repair are limited., Methods: We included children who underwent AAOCA surgery between October 2001 and December 2006. They were prospectively assessed with exercise stress test (EST), stress echocardiogram (SE), and stress myocardial perfusion scan (MPS)., Results: Of 24 participating children, 15 (63%) were male, 16 (67%) had anomalous right coronary (ARCA), and 7 (29%) were asymptomatic. Median age was 12 (5 to 18) years; follow-up was 15 (2 to 48) months. All had unobstructed neo-coronary ostia by echocardiogram and were asymptomatic. One anomalous origin of left main coronary artery (ALCA) patient and 8 ARCA patients had post-operative evaluations suggestive of ischemia. The ALCA patient had reversible apical septal and mid-anteroseptal hypokinesis on SE. Of the ARCA patients, 2 had inferior ST-segment depression on EST; subsequently, 1 had normal tests, but the other developed anterolateral Q waves. Two patients had blunted blood pressure response with EST, 1 had fixed apical inferior hypokinesis on SE, 2 had reversible perfusion defects on MPS, and 1 had a fixed perfusion defect on MPS., Conclusions: Subclinical changes suggestive of ischemia might occur despite patent neo-coronary ostia, notably after ARCA repair. The implication of these results on indication for surgery and subsequent sudden death risk is unknown. Serial EST, SE, and MPS are essential in evaluating ongoing ischemia risk after AAOCA repair.

Objectives: The purpose of this study was to describe the relationship between neo-aortic root size, neo-aortic insufficiency (AI), and reintervention at mid-term follow-up., Background: Data on neo-aortic valve function and growth after the Ross procedure in children are limited., Methods: A total of 74 of 119 Ross patients from January 1995 to December 2003 had > or =2 follow-up echocardiograms at our institution and were included. Neo-aortic dimensions were converted to z-scores and modeled over time. Kaplan-Meier analysis was used to assess freedom from neo-aortic outcomes, and predictors were identified through multivariate analysis., Results: Median age at Ross was 9 years (range 3 days to 34 years). Over 4.7 years (range 3 months to 9.3 years) follow-up, there was disproportionate enlargement of the neo-aortic root (z-score increase of 0.75/year [p < 0.0001]). Neo-AI progressed > or =1 grade in 36% of patients and > or =2 grades in 15%. Nine patients (12%) had neo-aortic reintervention at 2.0 years (range 1.1 to 9.5 years) after the Ross procedure owing to severe neo-AI (n = 7), neo-aortic root dilation (n = 1), and neo-aortic pseudoaneurysm (n = 1). At 6 years after the Ross procedure, freedom from neo-aortic reintervention was 88%. Freedom from neo-aortic root z-score >4 was only 3% and from moderate or greater neo-AI was 60%. Longer follow-up time was associated with neo-aortic root dilation (p < 0.0001). Prior ventricular septal defect (VSD) repair predicted neo-AI (p = 0.02) and reintervention (p = 0.03). Prior aortic valve replacement (p = 0.002) also predicted neo-AI. Neo-aortic root dilation was not associated with neo-AI or reintervention., Conclusions: At mid-term follow-up after the Ross procedure, neo-aortic root size increases significantly out of proportion to somatic growth, and neo-AI is progressive. Prior VSD repair and aortic valve replacement were associated with neo-AI and reintervention.

Objectives: A rapid, ice-cold saline flush combined with active compression-decompression (ACD) plus an inspiratory impedance threshold device (ITD) cardiopulmonary resusitation (CPR) will cool brain tissue more effectively than with standard CPR (S-CPR) during cardiac arrest (CA)., Background: Early institution of hypothermia after CPR and return of spontaneous circulation improves survival and outcomes after CA in humans., Methods: Ventricular fibrillation (VF) was induced for 8 min in anesthetized and tracheally intubated pigs. Pigs were randomized to receive either ACD + ITD CPR (n = 8) or S-CPR (n = 8). After 2 min of CPR, 30 ml/kg ice-cold saline (3 degrees C) was infused over the next 3 min of CPR via femoral vein followed by up to three defibrillation attempts (150 J, biphasic). If VF persisted, epinephrine (40 microg/kg) and vasopressin (0.3 U/kg) were administered followed by three additional defibrillation attempts. Hemodynamic variables and temperatures were continuously recorded., Results: All ACD + ITD CPR pigs (8 of 8) survived (defined as 15 min of return of spontaneous circulation [ROSC]) versus 3 of 8 pigs with S-CPR (p < 0.05). In survivors, brain temperature (degrees C) measured at 2-cm depth in brain cortex 1 min after ROSC decreased from 37.6 +/- 0.2 to 35.8 +/- 0.3 in ACD + ITD CPR versus 37.8 +/- 0.2 to 37.3 +/- 0.3 in S-CPR (p < 0.005). Immediately before defibrillation: 1) right atrial systolic/diastolic pressures (mm Hg) were lower (85 +/- 19, 4 +/- 1) in ACD + ITD CPR than S-CPR pigs (141 +/- 12, 8 +/- 3, p < 0.01); and 2) coronary perfusion pressures (mm Hg) were higher in ACD + ITD CPR (28.3 +/- 2) than S-CPR pigs (17.4 +/- 3, p < 0.01)., Conclusions: A rapid ice-cold saline infusion combined with ACD + ITD CPR during cardiac arrest induces cerebral hypothermia more rapidly immediately after ROSC than with S-CPR.

Objectives: We sought to assess the prevalence and progression of neo-aortic root dilation and valvar regurgitation after staged reconstruction for hypoplastic left heart syndrome (HLHS)., Background: In HLHS, the pulmonary valve functions as the neo-aortic valve. Neo-aortic valve dysfunction has been observed after arterial switch operation and the Ross procedure., Methods: Patients with HLHS born before January 1995 who had the Fontan operation and had serial echocardiograms were included. Echocardiograms were reviewed preoperatively, after each surgical reconstruction, and at most recent follow-up for neo-aortic root size and severity of neo-aortic regurgitation (AR). Potential risk factors for neo-aortic valve dysfunction were assessed., Results: Fifty-three patients met inclusion criteria. Bidirectional superior cavopulmonary anastomosis as an interim procedure was performed in 39 patients (74%). Median duration of follow-up was 9.2 (range 5.1 to 21) years. During follow-up, the neo-aortic root progressively dilated out of proportion to body size over time, with 52 patients (98%) having a Z-score >2 at most recent follow-up. Neo-AR was present in 61% of patients at most recent follow-up, with progression over time in 26 patients (49%). However, neo-AR was more than mild in only three patients. Significantly larger neo-aortic root Z-scores were observed in patients with any degree of neo-AR at most recent follow-up. No other anatomic or clinical variables correlated with severity of neo-AR or root dilation., Conclusions: After staged reconstruction for HLHS, neo-aortic root dilation and neo-AR progress over time. Early volume unloading does not have a beneficial impact on dilation of the neo-aortic root. These findings raise concerns about neo-aortic valve function into adulthood.