Ultrasound Cardiac Output Monitor (USCOM™) Measurements Prove Unreliable Compared to Cardiac Magnetic Resonance Imaging in Adolescents with Cardiac Disease.

The purpose of this stuy is to prospectively assess the reliability of the ultrasound cardiac output monitor (USCOM™) for measuring stroke volume index and predicting left ventricular outflow tract diameter in adolescents with heart disease. Sixty consecutive adolescents with heart disease attending a tertiary medical center underwent USCOM™ assessment immediately after cardiac magnetic resonance imaging. USCOM™ measured stroke volume index and predicted left ventricular outflow tract diameter were compared to cardiac magnetic resonance imaging-derived values using Bland-Altman analysis. Ten patients with an abnormal left ventricular outflow tract were excluded from the analysis. An adequate USCOM™ signal was obtained in 49/50 patients. Mean stroke volume index was 46.1 ml/m2 by the USCOM™ (range 22-66.9 ml/m2) and 42.9 ml/m2 by cardiac magnetic resonance imaging (range 24.7-59.9 ml/m2). The bias (mean difference) was 3.2 ml/m2; precision (± 2SD of differences), 17 ml/m2; and mean percentage error, 38%. The mean (± 2SD) left ventricular outflow tract diameter was 0.445 ± 0.536 cm smaller by the USCOM™ algorithm prediction than by cardiac magnetic resonance imaging. Attempted adjustment of USCOM™ stroke volume index using cardiac magnetic resonance imaging left ventricular outflow tract diameter failed to improve agreement between the two modalities (bias 28.4 ml/m2, precision 44.1 ml/m2, percentage error 77.3%). Our study raises concerns regarding the reliability of USCOM™ for stroke volume index measurement in adolescents with cardiac disease, which did not improve even after adjusting for its inaccurate left ventricular outflow tract diameter prediction.

Urgent Surgical Treatment of Aortic Endocarditis in Infants and Children.

Infective endocarditis (IE) in the pediatric population can present as a life-threatening condition. Optimal timing for surgical intervention should consider surgical risks versus the risk of neurologic complications. We herein report our experience with this group of critically ill children. Retrospective analysis of patient charts of all patients who underwent urgent surgical treatment of aortic IE from 1994 to 2014 was performed. Nine patients with acute storming IE of the aortic valve or the ascending aorta were urgently operated (eight normal heart, one congenital aortic stenosis), age ranged from 8 weeks to 4.2 years. Causative organisms were Staphylococcus aureus (2), Staphylococcus coagulase negative (1), Kingella kingae (2), Streptococcus pneumoniae (2), or culture negative (2). Presenting symptoms other than hemodynamic instability were neurologic decompensation (5) coronary embolization (1) and cardiogenic shock due to scalded skin syndrome (1). CT and MRI demonstrated significant brain infarcts in four patients. Operations performed were the Ross operation (7) and ascending aortic reconstruction (2). There were no operative deaths. At a median follow-up of 6 years (range 2-196 months), all patients are alive and well. Re-intervention included homograft replacement (2) and transcatheter Melody valve implantation (1). At their last follow-up, the neo-aortic valve was functional in all patients with minimal regurgitation and all had full resolution of the neurological deficits. Urgent surgical treatment for aortic valve IE in infants is challenging. Although surgery is complex and pre-disposing conditions such as sepsis, neurologic and cardiac decompensations are prevalent, operative results are excellent and gradual and significant neurologic improvement was noted over time.

Neonatal Cardiac Surgery in the New Era: Lessons learned from 1000 Consecutive Cases.

BACKGROUND: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success. OBJECTIVES: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years. METHODS: We conducted a retrospective analysis of surgical outcome in all neonatal patients who underwent cardiac surgery between January 2007 and July 2016 at Schneider Children's Medical Center of Israel. RESULTS: A total of 1003 neonates aged < 30 days underwent surgery for congenital heart defects at our center. Neonatal surgery accounted for 22.5% of all cardiac surgeries. Neonatal operative mortality was 7.3%, Operative mortality for individual lesions were: simple aortic coarctation (CoA) (198 patients, 2.5%), CoA with hypoplastic arch (24, 4%), CoA with ventricular septal defect (VSD) (84, 2.3%), transposition of the great arteries (TGA, simple and complex, 185, 6.3%), TGA with VSD (37, 0%), truncus arteriosus (26, 3.8%), interrupted aortic arch (25, 4%), Norwood Sano (71, 19.7%), neonatal tetralogy of Fallot (41, 0%), and shunt (131 patients, 12%). CONCLUSIONS: Neonatal surgical capabilities have improved substantially over the last decades. Excellent results can be expected for lesions that can be repaired to create biventricular circulation. Improved results can be attributed in part to the evolution of surgical strategies and assistive technologies, but essential is the collaborative effort of surgeons, cardiologists, anesthesiologists, and intensive care specialists acting as a cohesive team whose performance far exceeds the sum of its individual members' contributions.

Documented transient third-degree atrioventricular block and asystole in a child with familial dysautonomia.

An 11-year-old boy with familial dysautonomia presented with palpitations. Continuous 24-h Holter monitoring revealed intermittent high-grade atrioventricular block and asystole. The unopposed parasympathetic tone in patients with dysautonomia may make them susceptible to bradycardia and atrioventricular block. We recommend routine 24-h Holter monitoring screening and, when indicated, consideration of pacemaker implantation to reduce the high risk of sudden death phenomena in this patient population.