Faculty Bibliography

Background A right aortic arch is present in 18% of patients with congenital heart disease, and is usually associated with Tetralogy of Fallot or truncus arteriosus. Its incidence in D-TGA ranges from <1% to 12% in the literature. An isolated left subclavian, defined as a left subclavian artery that is disconnected from the aorta and arises from a branch pulmonary artery, is a rare right aortic arch anomaly. It is typically associated with Tetralogy of Fallot and truncus arteriosus, and exceedingly rare in cases of D-TGA. Case A full-term neonate with prenatal diagnosis of D-TGA with VSD was born at our institution. At birth, the baby was vigorous but with low oxygen saturation in the right hand (60s). The baby was started on continuous positive airway pressure at 100% FiO2 as well as a prostaglandin infusion and transferred to the NICU. Our initial echocardiogram confirmed the diagnosis of D-TGA with moderate-size muscular VSD as well as a right-sided aortic arch. On echocardiogram there appeared to be an aberrant left subclavian artery that could not be imaged definitively. The baby was taken to the catheterization lab the next day for balloon atrial septostomy and angiograms to better delineate arch anatomy. Angiography showed a left subclavian artery arising from the left pulmonary artery Decision-making In the case of our patient, the typical practice of measuring pre- and post-ductal saturations in the right and left hands, respectively, did not yield the "classic" picture of reverse differential cyanosis. We opted to monitor pre-ductal saturations on the right earlobe to better monitor saturations. Conclusion It is important to consider variability in arch sidedness and aortic arch anatomy when evaluating a newborn with D-TGA. We recommend checking for a pre-ductal saturation on one of the ears, if feasible, as this data is more representative of the arterial saturation of the cerebral blood vessels. Another important clinical consideration with an isolated subclavian artery with or without TGA is the subclavian steal phenomenon. When pulmonary vascular resistance drops, flow will preferentially go from the systemic circulation into the pulmonary circulation, reducing flow in vertebral arteries.
Copyright

OBJECTIVES: Excessive bleeding can be a problem during or after cardiac surgery. While cardiopulmonary bypass-associated platelet dysfunction is an important inducer of coagulopathy, preoperative platelet dysfunction can also contribute to this bleeding. We investigated the relationship between preoperative platelet dysfunction and transfusion of blood products given to children undergoing cardiac surgery. METHODS: The platelet function analyser test measures platelet function in vitro by aspirating blood through a small standard hole (creating high shear) in a collagen membrane infused with a platelet agonist. The time taken to form a platelet plug is known as closure time and prolonged closure time (CT) indicates platelet dysfunction. Three hundred and thirty-eight children who had undergone surgery with cardiopulmonary bypass between 2008 and 2012 were included. The volume of red blood cells and fresh-frozen plasma transfused was recorded. The relationship between closure time and transfusion requirements was analysed using linear and logistic regression. RESULTS: Patients with prolonged closure time had greater odds of getting red blood cells and fresh-frozen plasma transfusions compared with patients with normal closure time (P <0.01). On univariate analysis, age, weight, haematocrit, cardiopulmonary bypass time, Risk Adjustment for Congenital Heart Surgery score and closure time were associated with increased odds of red blood cells and fresh-frozen plasma transfusion in the operation theatre (P <0.05). However, when logistic multivariable regression analysis was applied, only age, cardiopulmonary bypass time and closure time remained as significant predictive factors for transfusion. CONCLUSIONS: In children who have undergone cardiac surgery, when age and cardiopulmonary bypass time are accounted for, a prolonged preoperative closure time is significantly associated with increased odds of red blood cells and fresh-frozen plasma transfusion in the operation theatre. This may have implications for planning and utilization of blood products.

We describe a 7-week-old female infant who presented with refractory supraventricular tachycardia (SVT). During amiodarone infusion, she developed hypotension and cardiac arrest requiring extracorporeal membrane oxygenation (ECMO) support. After successful control of SVT using procainamide infusion, she was weaned from ECMO and discharged home on oral flecainide. We conclude that infants with acidosis, ventricular dysfunction, and prolonged refractory SVT may poorly tolerate intravenous amiodarone.

BACKGROUND: Readmission after operations for congenital heart conditions has significant implications for patient care. Readmission rates vary between 8.7% and 15%. The aim of this study was to determine the incidence, causes, and risk factors associated with readmission. METHODS: 811 consecutive patients undergoing operations for congenital heart conditions were analyzed. Readmission was defined as admission to any hospital within 30 days of discharge for any cause. Demographic, preoperative, operative, and postoperative variables were evaluated. Univariate comparisons were made between the nonreadmission and readmission groups, and multivariate logistic regression analysis was made to determine independent risk factors for readmission. RESULTS: There were a total of 92 readmissions in 79 patients (9.7%). The reasons included cardiac (36, 39%), pulmonary (20, 22%), gastrointestinal (13, 14%), infectious (20, 22%), and other adverse events (2, 2%). Patients with either single-ventricle palliation or nasogastric feeding accounted for 40 (50%) readmissions. On univariate analysis, there were significant differences between readmitted and nonreadmitted patients in relation to patient age, chromosomal abnormality, mortality risk score, duration of mechanical ventilation, postoperative length of stay, single-ventricle physiology, and nasogastric feeding at discharge (p < 0.05). On multivariate analysis, significant risk factors for readmission were single-ventricle physiology (odds ratio [OR] 2.39; 95% confidence interval [CI] 1.28 to 4.47; p=0.005), preoperative arrhythmia (OR 2.59; 95% CI 1.02 to 6.59; p=0.04), longer postoperative length of stay (OR 2.2; 95% CI 1.22 to 3.99; p=0.008), and nasogastric tube feeding at discharge (OR 2.2; 95% CI 1.15 to 4.19; p=0.01). CONCLUSIONS: The incidence of readmission after operations for congenital cardiac conditions remains high. Efforts focusing on patients with single-ventricle palliation and those with preoperative arrhythmia, prolonged postoperative length of stay and nasogastric tube feeding at discharge may be particularly beneficial.

The goal of pediatric intensive care is early identification, severity assessment and resuscitation of critical patients by utilizing standardized protocols. The primary or precipitating disorder should be the focus of attention and specific intervention. But in order to provide holistic care to a patient, due attention should also be rendered to supportive care. Monitoring of sick children in PICU is an essential part of management. Various monitoring technologies add to the clinical monitoring but cannot replace clinical monitoring. The treating team should follow a checklist to ensure all aspects of supportive care are taken care of in every patient. Due attention should be paid to glucose control, skin and eye care, oral hygiene, prevention of stress ulcer, care of various lines and catheters and prevention of nosocomial infections.

Asthma is a common chronic inflammatory disorder of the airways characterized by recurrent wheezing, breathlessness, and coughing. Acute exacerbations of asthma can be life-threatening; annual worldwide estimated mortality is 250,000 and most of these deaths are preventable. While most of the acute exacerbations can be managed successfully in the emergency room, few children have severe exacerbations requiring intensive care. Mainstay of treatment for status asthmaticus are inhaled beta2 agonist and anticholinergic agents, oxygen along with corticosteroids. Children who do not respond well to initial treatment require parenteral beta2 agonist and magnesium. Rarely, sick children need parenteral aminophylline infusion and mechanical ventilation. Guidelines for diagnosis, treatment, ventilator management and supportive care for status asthmaticus in children are discussed in the protocol.

Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are disorders of pulmonary inflammation characterized by hypoxemia and respiratory failure. Children have varying incidence of ALI/ARDS from 2.2 to 16 per 100,000 pediatric population associated with high morbidity, mortality, and financial burden. The diagnostic criteria include: acute onset, severe arterial hypoxemia resistant to oxygen therapy alone (PaO(2)/FIO(2) ratio