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Objectives The goals of this study were to: 1) evaluate the cardiac magnetic resonance (CMR) characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. Background After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric CMR-based diagnostic criteria. Methods CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. Results Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. Conclusions CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

Background The Long-ter M O U tcomes after the Multisystem Inflammatory S yndrome I n C hildren (MUSIC) study aims to characterize the frequency and time course of acute and long-term cardiac and non-cardiac sequelae in multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C), which are currently poorly understood. Methods This multicenter observational cohort study will enroll at least 600 patients Conclusion The MUSIC study, with the largest cohort of MIS-C patients and the longest follow-up period to date, will make an important contribution to our understanding of the acute cardiac and non-cardiac manifestations of MIS-C and the long-term effects of this public health emergency.

Background:The optimal surgical strategy for tetralogy of Fallot (TOF) repair in neonates and young infants requiring early surgical intervention remains controversial. Our surgical center follows the uniform strategy of a staged approach with initial systemic-to-pulmonary artery shunt the majority of time when early surgical intervention is required. We characterized a contemporary cohort of patients with TOF with pulmonary stenosis (PS) undergoing staged repair in order to determine the rate of pulmonary valve-sparing repair (PVSR), growth of the pulmonary valve annulus and pulmonary arteries, postoperative morbidity and mortality, and need for reintervention.Methods:We retrospectively studied patients with TOF/PS who underwent staged repair from 2000 to 2017. Surgical details, postoperative course, and reinterventions were noted. Echocardiographic measurements and Z-score values of pulmonary valve annulus, main pulmonary artery (MPA), right pulmonary artery (RPA), and left pulmonary artery (LPA) diameters were evaluated.Results:Of the 59 patients with staged TOF/PS, PVSR was performed in 25 (42%). There was a 5% incidence of postoperative arrhythmia. The Z-scores of MPA, RPA, and LPA were significantly higher before complete repair when compared to before palliative shunt. The 5 and 10-year survival rates were both 93%, and the probability of freedom from reoperation at 5 and 10 years was 87% and 82%, respectively.Conclusions:Staged repair of TOF in young symptomatic infants results in 42% receiving PVSR, pulmonary artery growth, low incidence of postoperative arrhythmia, and relatively low rate of reoperations.

Background Electrophysiology study (EPS) is an important part of the diagnosis and workup for supraventricular tachycardia (SVT). Provocative medications are used to induce arrhythmias, when they are not inducible at baseline. The most common medication is the β1-specific agonist, isoproterenol, but recent price increases have resulted in a shift toward the nonspecific agonist, epinephrine. Objective We hypothesize that isoproterenol is a better induction agent for SVT during EPS than epinephrine. Methods We created a retrospective cohort of 131 patients, who underwent EPS and required medication infusion with either isoproterenol or epinephrine for SVT induction. The primary outcome was arrhythmia induction. Results Successful induction was achieved in 71% of isoproterenol cases and 53% of epinephrine cases (P = 0.020). Isoproterenol was significantly better than epinephrine for SVT induction during EPS (odds ratio [OR], 2.35; 95% confidence interval [CI], 1.14-4.85; P = 0.021). There was no difference in baseline variables or complications between the two groups. Other variables associated with successful arrhythmia induction included a longer procedure duration and atrioventricular nodal re-entry tachycardia as the clinical arrhythmia. In a multivariable model, isoproterenol remained significantly associated with successful induction (OR, 2.57; 95% CI, 1.002-6.59; P = 0.05). Conclusions Isoproterenol was significantly better than epinephrine for SVT arrhythmia induction. However, epinephrine was safe and successfully induced arrhythmias in the majority of patients who received it. Furthermore, when atropine was added in epinephrine-refractory cases, in a post hoc analysis there was no difference in arrhythmia induction between medications. Cost savings could thus be significant without compromising safety.

We report the placement of a permanent transvenous nonselective His bundle pacing lead in conjunction with a transvenous pacemaker/implantable cardioverter-defibrillator in an adult with Levo-Transposition of the Great Arteries (L-TGA) and a stenotic coronary sinus (CS) ostium, which would not accommodate a transvenous left ventricular (LV) pacing lead. Nonselective His bundle pacing provided a nearly identical ventricular activation pattern in this previously unpaced patient. Many L-TGA patients will have an eventual need for permanent pacing and, given the challenges of CS cannulation, His bundle pacing may represent a preferred modality rather than pure morphologic LV pacing or surgical systemic ventricular lead placement to achieve optimal electrical synchrony.

In this report, a unique case of a symptomatic vascular ring formed by right aortic arch, aberrant left subclavian artery, and left ligamentum arteriosus in which there is atresia of the proximal left subclavian artery is described. Imaging modalities were non-diagnostic and the patient was sent to surgery based on strong clinical suspicion. Her anatomy was delineated in the operating room and the ring was successfully repaired.

Background— Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We assessed the prevalence of residual intramural VSDs and their effect on postoperative course. Methods and Results— Children who underwent biventricular repair of a conotruncal anomaly from January 1, 2006, to June 30, 2013, and had a postoperative transthoracic echocardiogram were included. Images were reviewed for residual intramural or nonintramural VSDs. The primary outcome was a composite of mortality, extracorporeal membrane oxygenation use, and need for subsequent catheter or surgical VSD closure. The secondary outcome was postoperative hospital length of stay. A residual VSD was present in 256 of the 442 subjects (58%), of which 231 (90%) were P P =0.0001). These associations remained significant after adjustment for known risk factors for poor outcomes, including residual VSD size and operative complexity. Conclusions— Among residual VSDs after repair of conotruncal anomalies, intramural VSDs are uniquely associated with postoperative morbidity, mortality, and longer postoperative hospital length of stay. It is important to recognize intramural VSDs in the postoperative period.

The proximity of the coronary arteries and the bundle of His to the aortic valve may contribute to the pathogenesis of arrhythmias in patients with aortic valve disease. Severe aortic valve disease may also adversely alter left ventricular hemodynamics (end-diastolic dimensions and wall stress) and thus create a substrate for ventricular arrhythmias before any intervention is performed. The severity of these arrhythmias depends on the severity of the underlying substrate (or the specific problem, such as aortic stenosis or aortic regurgitation), the age at which the aortic valve intervention was performed, the type of intervention (i.e. transcatheter aortic valve interventions or open aortic valve replacement or repair), and the reversibility of the altered hemodynamics after surgery. Both bradyarrhythmias and tachyarrhythmias are known complications of aortic valve interventions. Although data are scant, this review summarizes the incidence of arrhythmias before and after aortic valve interventions from a pediatric perspective.

Background Protein-losing enteropathy (PLE) is a challenging complication after a Fontan operation. Subclinical enteric protein loss may precede development of overt PLE. We evaluated the acute effects of Fontan circulation on enteric protein loss and mesenteric vascular resistance. Methods A prospective cohort study was performed evaluating enteric protein loss in children undergoing Fontan operations. Stool alpha-1 antitrypsin (A1AT) concentration was measured in the preoperative, early postoperative, and intermediate postoperative (3–9 months) periods. The intestinal circulation was characterized by Doppler-derived resistance indices of the superior mesenteric artery (SMA), and serum albumin and protein levels were obtained. Results We enrolled 33 participants at a median age at operation of 3.0 years (interquartile range [IQR], 2.5–3.3 years). No clinical PLE was observed. Six of the 93 stool samples obtained had elevated A1AT levels (>54 mg/dL), with 2 abnormal samples at each of the 3 time points. Two of the 5 participants with elevated stool A1AT values had significant hemodynamic disturbances requiring intervention (junctional bradycardia or tricuspid stenosis). There was no difference in SMA resistance in the preoperative versus early postoperative periods ( p = 0.9). Serum albumin levels were lower in the early postoperative period compared with the preoperative period (3.2 mg/dL [{IQR}, 2.9–3.5] versus 4.1 mg/dL; IQR, 3.4–4.5; p = 0.01) but did not correlate with abnormal stool A1AT concentration or SMA resistance indices. Conclusions The Fontan operation does not commonly result in acute development of increased enteric protein loss. However, increased enteric protein loss may occur in children before or after a Fontan operation, particularly when hemodynamic disturbances are present.

Background Intramural ventricular septal defects (VSDs), residual interventricular communications occurring after repair of conotruncal defects, are associated with poor postoperative outcomes. The ability of intraoperative transesophageal echocardiography (TEE) to identify intramural VSDs has not yet been evaluated. Methods Intraoperative TEE and postoperative transthoracic echocardiography (TTE) data in all patients undergoing all biventricular repair of conotruncal anomalies in our hospital between January 1, 2006, and June 30, 2013, were reviewed. The ability of TEE to accurately identify residual defects was assessed using postoperative TTE as the reference imaging modality. Results Intramural VSDs occurred in 34 of 337 patients evaluated; 19 were identified by both TTE and TEE, and 15 were identified by TTE only. Sensitivity was 56% and specificity was 100% for TEE to identify intramural VSDs. Peripatch VSDs were identified in 90 patients by both TTE and TEE, in 53 by TTE only, and in 15 by TEE only, yielding a sensitivity of 63% and specificity of 92%. Of the VSDs requiring catheterization or surgical reintervention, 6 of 7 intramural VSDs and all 5 peripatch VSDs were identified by intraoperative TEE. TEE guided the intraoperative decision to return to cardiopulmonary bypass (CPB) in an attempt to close residual defects in 12 patients with intramural VSDs and in 4 patients with peripatch VSDs seen after initial CPB; of these, 10 intramural VSDs and all 4 peripatch VSDs resolved or became smaller on final intraoperative TEE. Conclusions TEE has modest sensitivity but high specificity for identifying intramural VSDs and can detect most defects requiring reintervention. Repeat attempts at closure in the index operation may successfully correct intramural VSDs identified by TEE.

Background Diffuse fibrosis and strain are abnormal in adult cardiomyopathy patients and in children exposed to anthracyclines, but have not been well studied in pediatric hypertrophic (HCM) and dilated (DCM) cardiomyopathy. We hypothesized that HCM and DCM patients have different diffuse fibrosis and strain characteristics. Methods