Critical Illness-Related Corticosteroid Insufficiency (CIRCI) After Pediatric Cardiac Surgery.

Although current studies do not support the routine use of corticosteroids after cardiopulmonary bypass in pediatric patients, there is incomplete understanding of the potential hemodynamic contribution of postoperative critical illness-related corticosteroid insufficiency in the intensive care unit. By reviewing the available studies and underlying pathophysiology of these phenomena in critically ill neonates, we can identify a subset of patients that may benefit from optimal diagnosis and treatment of receiving postoperative steroids. A suggested algorithm used at our institution is provided as a guideline for treatment of this high-risk population.

Impact of Perioperative Tachydysrhythmias on Mortality and Length of Stay in Complete Repair of Tetralogy of Fallot: A Multicenter Retrospective Cohort Study from the Pediatric Health Information System.

INTRODUCTION: Tachydysrhythmias (TDS) frequently occur after complete repair of tetralogy of Fallot (TOF). However, not much is known about the effect of TDS on morbidity and mortality after TOF repair. We sought to assess the associations between TDS and mortality and morbidity after repair of TOF using a multicentre database. MATERIALS AND METHODS: We identified all children aged 0-5 years in the Pediatric Health Information System who underwent TOF repair between 2004 and 2015. Codes for TDS were used to identify cases. Outcome variables were inpatient mortality and total length of stay (LOS). Univariate and multiple logistic and linear regression analyses were used to identify the effects of multiple risk factors, including TDS, on mortality and LOS. RESULTS: A total of 7,749 patients met inclusion criteria, of which 1,493 (19%) had codes for TDS. There was no association between TDS and inpatient mortality. However, TDS were associated with 1.1 days longer LOS and accounted for 2% of the variation observed in LOS. CONCLUSION: After complete repair of TOF, TDS were not associated with mortality and appeared to have only a modest effect on LOS.

Vaping Cannabis Butane Hash Oil Leads to Severe Acute Respiratory Distress Syndrome-A Case of EVALI in a Teenager With Hypertrophic Cardiomyopathy.

A 17-year-old with severe hypertrophic cardiomyopathy (HCM) presented to the emergency department with symptoms of cough, shortness of breath, chest pain, and tactile fevers. She was initially admitted to the cardiac floor, and later transferred to the cardiothoracic intensive care unit on day 5 of illness with deterioration over the next week from BiLevel positive airway pressure to endotracheal intubation. The patient met criteria for severe acute respiratory distress syndrome (ARDS). Standard ARDS lung-protective strategies were refined in consideration of complications caused by her HCM. Such complications included dynamic cardiac outflow obstruction, myocardial ischemia with tachycardia, elevated pulmonary vascular resistance from diastolic dysfunction, and narrow fluid balance window to reduce pulmonary edema while maintaining adequate left ventricular preload. The patient remained refractory despite broad-spectrum antibiotics requiring multiple vasoactive medications, aggressive ventilator management, and inhaled nitric oxide. Social history revealed "vaping" cannabis with butane hash oil prior to symptom onset. Corticosteroids were initiated 2 weeks after initial presentation (day 9 of mechanical ventilation) with rapid recovery and resolution of illness. Acute respiratory distress syndrome is an aggressive disease in the intensive care unit. E-cigarette or vaping product use-associated lung injury is increasingly recognized as a cause of ARDS in adolescents and adults. A complete social history is essential and must be obtained early in all such patients presenting with symptoms of acute respiratory distress and revisited throughout the hospital stay if no other reason for the ARDS is discovered. Disease progression may be subacute with a long interval between onset of symptoms and peak symptoms. The risk of barotrauma is high despite lung-protective ventilation strategies. Management is supportive with resolution over days to weeks. However, other clinical factors may considerably complicate management in cases of underlying comorbidities.

Late transcatheter recanalization of a closed ductus arteriosus in a 2-month-old infant with tetralogy of Fallot and isolated left pulmonary artery.

Ductal stenting is increasing as an alternative to surgical shunts in cyanotic newborns. However, most reports include newborns with patent ducts who are often on prostaglandin before the procedure. We report a successful late transcatheter recanalization of the closed ductus arteriosus in a 2-month-old infant with tetralogy of Fallot, right aortic arch, and an isolated left pulmonary artery (LPA). We achieved adequate LPA growth before complete repair. There were no procedural complications. This procedure is rare in Western countries due to universal pulse oximetry screening and increased fetal diagnoses. The procedure is technically challenging in the absence of any angiographic flow into the ductus. Evidence of a ductal ampulla increases the chances of success.

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa following endocarditis and aortic valve surgery in an infant-Case report and exhaustive systematic review of pediatric cases.

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (P-MAIVF) continues to be a rare diagnosis in children. Etiology, presentation, and management strategies are considerably different from adults. We report a fatal case of P-MAIVF with classical transthoracic and transesophageal echocardiographic findings complemented by CT imaging. The natural course of uncomplicated/asymptomatic P-MAIVF is largely unknown since most patients are offered surgery. We present an extensive literature review of pediatric P-MAIVF and highlight important differences from the adult form of this disease.

Age-Related Changes in Inferior Vena Cava Dimensions among Children and Adolescents with Syncope.

OBJECTIVE: To test the hypothesis that increased venous compliance manifested as inferior vena cava (IVC) dilation is an important substrate for syncope in children. STUDY DESIGN: IVC diameters were measured in 191 children and adolescents with syncope and in 95 controls. Subjects were divided based on age <12 years (younger group) and ≥12 years (older group). IVC measurements at the right atrial junction (IVC-RA), 10 mm below the IVC-RA junction (IVC-RA10), and at the point of maximal diameter (IVCmax) were made. The linear relation to body surface area (BSA) was confirmed, as were dimensions indexed to BSA (iIVC). Relationships between iIVC and the time of day were evaluated. RESULTS: In the syncope group, the mean age was 12.9 ± 3.6 years, mean weight was 54.7 ± 23 kg, and mean BSA was 1.5 ± 0.4 m2. Among controls, all IVC dimensions varied linearly with BSA (P < .001). In the older group (140 patients with syncope and 60 controls), all iIVC dimensions were larger in the syncope cohort: iIVC-RA, 9 vs 7.7 mm/m2 (P < .0001); iIVC-RA10, 9.4 vs 8.1 mm/m2 (P < .0001); iIVCmax, 11.7 vs 10.6 mm/m2 (P = .002). In the younger group (51 patients with syncope and 35 controls), there were no differences in iIVC measurements between the syncope cohort and controls: iIVC-RA, 10.2 vs 11.3 mm/m2; iIVC-RA10, 11.7 vs 12.0 mm/m2; iIVCmax, 14.2 vs 14.7 mm/m2 (P > .05 for all). CONCLUSIONS: The IVC is enlarged in teenagers with syncope compared with controls, suggesting that venous capacitance and resultant pooling play roles in the pathogenesis of syncope. In contrast, younger children with syncope do not demonstrate IVC dilation, suggesting that their syncope arises from a different mechanism.

Pre-excitation induced ventricular dysfunction and successful berlin heart explantation after accessory pathway ablation.

A 13 kg, 20 month-old, Caucasian girl, presented with cardiomyopathy, biventricular dysfunction and pre-excitation on electrocardiogram. She had normal intracardiac anatomy with severely dilated left ventricle and severely diminished biventricular function (Fig. 1). She was treated with milrinone and epinephrine infusions, mechanical ventilation and listed for heart transplant. She underwent Berlin Heart EXCOR biventricular assist device (BiVAD) placement (30 ml LVAD and 25 ml RVAD pumps). No supraventricular tachycardia (SVT) was inducible or noted during her hospitalization. First ablation attempt without BiVAD support was unsuccessful; however, 18 days post BiVAD implantation, another electrophysiology study and successful radiofrequency ablation of a right anterolateral accessory pathway was performed on BiVAD support. After successful ablation and loss of pre-excitation, the cardiac dysfunction rapidly improved with initial improvement noted as early as 48 h after the successful ablation. Due to recovery of cardiac function, a BiVAD wean protocol was initiated and BiVAD explantation was performed 48 days after the implant (30 days after the successful ablation). To the best of our knowledge, this is the first report of successful BiVAD explantation.

Teenage pregnancy with catecholaminergic polymorphic ventricular tachycardia and documented ICD discharges.

We report the first case of pregnancy in a pediatric patient with catecholiminergic polymorphic ventricular tachycardia (CPVT). Pregnant adolescents with CPVT are at high risk for NSVT and malignant VT during pregnancy, despite antiarrhythmic medication. They may receive multiple implantable cardioverter defibrillator (ICD) therapies. Such patients require close monitoring with special care during the first trimester.

Knowledge of asthma management by general practitioners in Karachi, Pakistan: comparison with international guidelines.

BACKGROUND: Suboptimal management of asthma by general practitioners (GPs) can lead to poor health outcomes AIMS: To assess the management of common asthma presentations by GPs using the Global Initiative for Asthma (GINA) guidelines as a comparative tool. METHODS: A cross-sectional survey was conducted in Karachi, Pakistan. Of 250 GPs approached, 192 completed a self-administered questionnaire regarding pathology, key history points, risk factors, diagnosis, and management of asthma. RESULTS: Overall, 28.6% of GPs had adequate knowledge of the core concepts of asthma, while only 10.4% had adequate practice in asthma management. About 78% of GPs had inadequate knowledge of pathology, about 90% had inadequate knowledge of medications to be used, and 63% had inadequate knowledge regarding diet restrictions. Knowledge regarding symptoms not usually associated with asthma was adequate, as was knowledge regarding non-pharmacological management (79% each). Practices regarding asthma diagnosis were good (99.0%). However, practices regarding acute exacerbations and patients who wish to exercise were inadequate in 85.9% and 82.8% of GPs, respectively. CONCLUSIONS: The majority of GPs had poor knowledge and practice of asthma. We recommend initiation of programmes to improve their knowledge and practices.