Thoracoscopic Resection of Left Atrial Appendage Aneurysm in a 16-Year-Old Boy.

We report a case of a left atrial appendage aneurysm (LAAA) in a 16-year-old boy presenting with supraventricular tachycardia (SVT). The aneurysm was detected incidentally on a routine echocardiogram performed before an electrophysiology study for evaluation and management of the SVT. The aneurysm was successfully resected under cardiopulmonary bypass through video-assisted thoracoscopic surgery. This type of surgery is a useful approach for LAAA in the pediatric population.

Arrhythmogenic channelopathy syndromes presenting as refractory epilepsy.

BACKGROUND: Children and young adults with potentially lethal cardiac channelopathies often present to medical care with a history of syncope or seizures due to episodic ventricular arrhythmias and associated cerebral hypoperfusion. METHODS: Two important types of genetic arrhythmia syndromes-long QT syndrome and catecholaminergic polymorphic ventricular tachycardia-are discussed using relevant case examples. The pathophysiology and distinguishing clinical features of these conditions are reviewed. RESULTS: The patients in each case were ultimately diagnosed with a cardiac channelopathy as the cause for their syncope and refractory seizures. With appropriate medical management, no further events have occurred to date. CONCLUSIONS: Cardiac channelopathies can be misdiagnosed as refractory epilepsy when in fact these events represent convulsive syncopes. Knowledge of and suspicion for these arrhythmogenic conditions may expedite diagnosis and improve outcomes.

Urinary biomarkers and renal near-infrared spectroscopy predict intensive care unit outcomes after cardiac surgery in infants younger than 6 months of age.

OBJECTIVE: To assess the ability of urinary acute kidney injury biomarkers and renal near-infrared spectroscopy (NIRS) to predict outcomes in infants after surgery for congenital heart disease. METHODS: Urinary levels of neutrophil gelatinase-associated lipocalin (NGAL), interleukin-18 (IL-18), kidney injury molecule-1 (KIM-1), and cystatin C were measured preoperatively and postoperatively in 49 infants younger than 6 months of age. Renal NIRS was monitored for the first 24 hours after surgery. A composite poor outcome was defined as death, the need for renal replacement therapy, prolonged time to first extubation, or prolonged intensive care unit length of stay. RESULTS: Forty-two (86%) patients had acute kidney injury as indicated by at least Acute Kidney Injury Network/Kidney Disease: Improving Global Outcomes (AKIN/KDIGO) stage 1 criteria, and 17 (35%) patients had poor outcomes, including 3 deaths. With the exception of KIM-1, all biomarkers demonstrated significant increases within 24 hours postoperatively among patients with poor outcomes. Low levels of NGAL and IL-18 demonstrated high negative predictive values (91%) within 2 hours postoperatively. Poor outcome infants had greater cumulative time with NIRS saturations less than 50% (60 vs 1.5 minutes; P = .02) in the first 24 hours. CONCLUSIONS: Within the first 24 hours after cardiopulmonary bypass, infants at increased risk for poor outcomes demonstrated elevated urinary NGAL, IL-18, and cystatin C and increased time with low NIRS saturations. These findings suggest that urinary biomarkers and renal NIRS may differentiate patients with good versus poor outcomes in the early postoperative period, which could assist clinicians when counseling families and inform the development of future clinical trials.

Fluid overload in infants following congenital heart surgery.

OBJECTIVE: To describe postoperative fluid overload patterns and correlate degree of fluid overload with intensive care morbidity and mortality in infants undergoing congenital heart surgery. DESIGN: Prospective, observational study. Fluid overload (%) was calculated by two methods: 1) (Total fluid in - Total fluid out)/(Preoperative weight) × 100; and 2) (Current weight - Preoperative weight)/(Preoperative weight) × 100. Composite poor outcome included: need for renal replacement therapy, upper quartile time to extubation or intensive care length of stay (> 6.5 and 9.9 days, respectively), or death ≤ 30 days after surgery. SETTING: University hospital pediatric cardiac ICU. PATIENTS: Forty-nine infants < 6 months of age undergoing congenital heart surgery with cardiopulmonary bypass during the period of July 2009 to July 2010. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Patients had a median age of 53 days (21 neonates) and mean weight of 4.5 ± 1.3 kg. Forty-two patients (86%) developed acute kidney injury by meeting at least Acute Kidney Injury Network and Kidney Disease Improving Global Outcomes stage 1 criteria (serum creatinine rise of 50% or ≥ 0.3mg/dL). The patients with adverse outcomes (n = 17, 35%) were younger (7 [5 - 10] vs. 98 [33 - 150] days, p = 0.001), had lower preoperative weight (3.7 ± 0.7 vs. 4.9 ± 1.4 kg, p = 0.0002), higher postoperative mean peak serum creatinine (SCr) (0.9 ± 0.3 vs. 0.6 ± 0.3mg/dL, p = 0.005), and higher mean maximum fluid overload by both method 1 (12% ± 10% vs. 6% ± 4%, p = 0.03) and method 2 (24% ± 15% vs. 14% ± 8%, p = 0.02). Predictors of a poor outcome from multivariate analyses were cardiopulmonary bypass time, use of circulatory arrest, and increased vasoactive medication requirements postoperatively. CONCLUSIONS: Early postoperative fluid overload is associated with suboptimal outcomes in infants following cardiac surgery. Because the majority of patients developed kidney injury without needing renal replacement therapy, fluid overload may be an important risk factor for adverse outcomes with all degrees of acute kidney injury.