Amplatzer septal occluder-associated CP uniquely involves the anterosuperior atrial walls and adjacent aorta. Pathophysiology remains poorly understood.

CT-derived PBV heterogeneity is a suitable surrogate for CT-derived PBF heterogeneity.

Objectives: We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. Background: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. Methods: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. Results: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. Conclusion: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures. V C 2015 Wiley Periodicals, Inc.

PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc.

Guidelines for the treatment of active right-sided endocarditis are not well defined. We report the first novel use of an aspiration catheter system for transcatheter therapy of refractory active right sided endocarditis on a bioprosthetic valve in a high-risk surgical patient as a bridge to surgical intervention.

Objective The study aimed to alert the neonatal community to the possibility of multisystem inflammatory syndrome in children (MIS-C) like disease in critically ill neonates born to mothers with coronavirus disease 2019 (COVID-19). Study Design Diagnosis of MIS-C like disease was pursued after echocardiography showed severely depressed ventricular function and pathological coronary artery dilation in the setting of medically refractory multisystem organ failure and maternal COVID-19 infection. The neonate did not respond to standard medical therapy, and there was no alternative disease that could explain the clinical course. High index of clinical suspicion coupled with low risk of intravenous immunoglobulin (IVIG) prompted us to pursue IVIG administration even though the neonate did not meet classic criteria for MIS-C. Result Following treatment with IVIG, there was rapid clinical improvement. Ventricular function improved within 15 hours and coronary artery dilation resolved in 8 days. There was no recurrence of disease during follow-up. Conclusion COVID-19 associated MIS-C like disease has not been well described in neonates. As typical features may be conspicuously absent, a high index of suspicion is warranted in critically ill neonates born to mothers with COVID-19. Echocardiography may provide critical diagnostic information and narrow the differential diagnosis. Key Points

Idiopathic dilatation of the right atrium (IDRA) is a rare anomaly defined as isolated enlargement of the right atrium in the absence of other cardiac lesions known to cause right atrial dilatation. IDRA is a congenital anomaly with unknown pathogenesis and highly variable clinical presentation. Optimal management of severe IDRA is controversial and individualized. Literature reports of long-term follow-up have been limited. We describe a child with IDRA with rapid atrial tachycardia (AT) refractory to both medical and surgical management, and we provide long-term follow-up on our two previously reported cases, both of whom had documented AT. For infants with AT, the clinical course is unpredictable, and medical therapy is the first line of treatment. The decision to proceed with surgical resection of a giant right atrium should be made on an individual basis. Atrial resection along with a modified right atrial MAZE procedure could be considered in infants with life-threatening atrial tachyarrhythmia refractory to medical treatment. Surgical scarring of the right atrium may produce substrate for atrial arrhythmia, which may also be refractory to medical therapy. Histological examination of excised atrial tissue remains inconsistent and not contributory to the determination of the etiology of IDRA. Our three infants with IDRA illustrate unique features of their variable clinical courses, as well as continued difficulties with establishing clear guidelines with regard to surgical management of this unusual disorder.

Mean developmental scores were in the low average range of normative data. Socioeconomic factors predicted mental outcome. High postoperative lactate level on day 1 predicted low motor scores. Other potentially modifiable variables will need to be explored. Further epidemiologic study relating race to occurrence of total anomalous pulmonary venous connection is suggested. Early neurodevelopmental assessment is recommended.