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Most children undergoing congenital heart surgery can be extubated in the operating room. Most neonates, including many undergoing complex procedures, can be extubated within the first 24 hours after surgery. Early extubation was associated with low morbidity rates and short lengths of intensive care unit and hospital stays.

Background Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an uncommon, potentially lethal, ion channelopathy. Standard therapies have high failure rates and little is known about treatment in children. Newer options such as flecainide and left cardiac sympathetic denervation (LCSD) are not well validated. We sought to define treatment outcomes in children with CPVT. Methods and Results This is a Pediatric and Congenital Electrophysiology Society (PACES) multicenter, retrospective cohort study of CPVT patients diagnosed before 19 years of age. The cohort included 226 patients, including 170 probands and 56 relatives. Symptomatic presentation was reported in 176 (78%). Symptom onset occurred at 10.8 (IQR 6.8–13.2) years with a delay to diagnosis of 0.5 (0–2.6) years. Syncope (p<0.001), cardiac arrest (p<0.001) and treatment failure (p=0.008) occurred more often in probands. Beta-blockers were prescribed in 205 of 211 patients (97%) on medication, and 25% experienced at least one treatment failure event. Implantable cardioverter defibrillators (ICDs) were placed in 121 (54%) and was associated with electrical storm in 22 (18%). Flecainide was used in 24% and LCSD in 8%. Six deaths (3%) occurred during a cumulative follow-up of 788 patient-years. Conclusions This study demonstrates a malignant phenotype and lengthy delay to diagnosis in CPVT. Probands were typically severely affected. Beta-blockers were almost universally initiated; however, treatment failure, non-compliance and sub-therapeutic dosing were often reported. ICDs were common despite numerous device-related complications. Treatment failure was rare in the quarter of patients on flecainide. LCSD was not uncommon although the indication was variable.

This large, multicentre study identifies contemporary challenges related to the diagnosis and prognostication of CPVT patients. Structural modelling of RyR2 can improve our understanding severe CPVT phenotypes. Wakeful rest, rather than exertion, often precipitated life-threatening cardiac events.

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Background— Focal atrial tachycardia (FAT) is an uncommon cause of supraventricular tachycardia in children. Incessant FAT can lead to tachycardia-induced cardiomyopathy. There is limited information regarding the clinical course and management of FAT. This study characterizes current management strategies for FAT in children including the prevalence of spontaneous resolution and the role of catheter ablation. Methods and Results— This is a retrospective chart review of pediatric patients with FAT managed between January 2000 and November 2010 at 10 pediatric centers. There were 249 patients with a median age at diagnosis of 7.2 (95% confidence interval, 5.8–10.4) years. Cardiomyopathy was observed in 28%. Resolution of FAT occurred in 89%, including spontaneous resolution without catheter ablation in 34%. Antiarrhythmic medications were used for initial therapy in 154 patients with control of FAT in 72%. Among first-line medications, β-blockers were the most common (53%) and effective (42%). Catheter ablation was successful in 80% of patients. FAT recurrence was less common with electroanatomic mapping compared with conventional mapping techniques (16% versus 35%; P =0.02). Patients were followed for a median of 2.1 (95% confidence interval, 1.8–2.6) years. Conclusions— FAT is managed successfully in most children. Current approaches are variable. Many patients have control of FAT with medications; however, catheter ablation is used for most patients. Spontaneous resolution is common for young children, emphasizing the role for delayed ablation in this group. Ablation is successful for all ages. Lower recurrence occurs when electroanatomic mapping techniques are used.

Objective. To measure aerobic fitness (maximum oxygen consumption [VO 2peak ]), fatigue, quality of life (QOL), and disease activity in young persons with systemic lupus erythematosus (SLE), and to determine an equation for predicting VO 2peak from the distance walked in 6 minutes (6MW). Methods. Fifteen young patients ages 12-19 years with SLE participated. VO 2 was measured by a graded treadmill exercise test. Submaximal exercise intensity was determined from the ventilatory anaerobic threshold. Submaximal aerobic capacity was measured using the 6MW. Patient questionnaires included measures of fatigue, QOL, and physical activity. Physician questionnaires included the Systemic Lupus Erythematosus Disease Activity Index and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. Results. Compared with age-matched norms, our patients had moderate impairment in aerobic fitness, with a mean ؎ SD VO 2peak of 31.1 ؎ 7.9 ml/minute/kg and a mean 6MW distance Z score of ؊2.4 ؎ 2.3. The regression equation to predict VO 2peak (ml/minute/kg) from 6MW was as follows: 57.1 ؉ [0.038 ؋ distance (meters)] ؉ (؊0.35 ؋ maximal heart rate) (R ‫؍‬ 0.67, P ‫؍‬ 0.027). Ten subjects (67%) reported significant fatigue. There was no significant correlation of fatigue with fitness measures. Neither fatigue nor fitness was significantly correlated with disease activity, disease damage, or QOL measures. Conclusion. Young SLE patients have lower aerobic fitness than reference norms. The 6MW may be used as a marker of fitness, but it is preferable to determine VO 2 with a graded exercise test. Fatigue is a significant symptom in young SLE patients. The application of fatigue measures in young persons is exploratory. The relationship between fatigue and aerobic fitness is not clear.

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