Aditya Badheka

Multicenter Analysis of Early Childhood Outcomes Following Repair of Truncus Arteriosus

BACKGROUNDnLiterature describing morbidity and mortality after truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population.nnnMETHODSnWe retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after operation. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modeling.nnnRESULTSnWe reviewed 216 patients with median follow-up of 2.9 years (range, 0.1 to 8.8). Operative mortality occurred in 15 patients (7%). Of the 201 survivors there were 14 (7%) late deaths. DiGeorge syndrome (hazard ratio [HR], 5.4; 95% confidence interval [CI], 1.6 to 17.8) and need for postoperative tracheostomy (HR, 5.9; 95% CI, 1.8 to 19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention, 23 months; range, 0.3 to 93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra (Medtronic, Inc, Minneapolis, MN) bovine jugular vein conduits (HR, 1.9; 95% CI, 1.2 to 3.1) and smaller conduit sizexa0(HR per mm/m2, 1.05; 95% CI, 1.03 to 1.08).nnnCONCLUSIONSnIn a multicenter dataset DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, whereas risk of conduit reintervention was independently influenced by both initial conduit type and size.

1325: OUTCOMES ASSOCIATED WITH PERIPHERALLY INSERTED CENTRAL CATHETERS (PICCS) IN HOSPITALIZED CHILDREN

www.ccmjournal.org Critical Care Medicine • Volume 46 • Number 1 (Supplement) Learning Objectives: The impact of widespread sepsis education and quality improvement projects to improve the outcomes of children with severe sepsis and septic shock are not well reported. This study investigated both the positive and negative outcomes in staff knowledge, practice patterns and mortality that occurred after a multidisciplinary quality initiative to improve recognition and treatment for children with severe sepsis and septic shock. Methods: Hospital-wide quality initiative involving targeted educational efforts, improvements in severe sepsis protocol design and availability, and continued feedback to all staff involved in the care of affected children including nurses, physicians, and respiratory therapists. All efforts were tailored to preferences staff provided on a survey given 6 months before the project began. Results: Overall staff performance on sepsis scenario questions improved from 65% to 74% correct. After the first 9 months of the initiative, the hospital-wide mortality rate in children with severe sepsis and septic shock improved from an unadjusted rate of 17% with a PRISM-adjusted standardized mortality ratio of 4.0 to an unadjusted rate of 1.4% with a SMR of 0.43. In all cases of severe sepsis and septic shock, severe sepsis protocol use improved to almost 90% and was statistically significantly higher than before the initiative, p = 0.002. In addition, 1-hour bundle compliance improved from 3.8% to 40%, higher than the state average. To ensure that raising sepsis awareness did not raise anxiety and provider overreaction, the number of doses of the antibiotics recommended in the severe sepsis protocol given to inpatients hospital-wide were measured. The absolute number of doses ordered did not statistically significantly increase in the first 12 months after the initiative began (p> 0.05). Conclusions: Staff knowledge and clinical performance in treating children with severe sepsis or septic shock can improve using widespread engagement, education, and feedback. Patient outcomes do improve as a result.

223: LIFE-THREATENING FLECAINIDE OVERDOSE TREATED SUCCESSFULLY WITH LIPID EMULSION AND ECMO IN A CHILD

www.ccmjournal.org Critical Care Medicine • Volume 46 • Number 1 (Supplement) Learning Objectives: Flecainide is a class 1C slow Na channel antagonist used to treat tachyarrhythmias. The cardiac toxic effects include QRS prolongation, AV block, hypotension and ventricular dysrhythmias--especially in those with structural heart diseases. Severe Flecainide overdose is associated with high risk of mortality. Flecainide toxicity is difficult to treat as it is highly bioavailable with high volume of distribution with long & variable half-life (7-23hrs). There are few reports of optimal treatment [NaHCO3, intravenous fat emulsion (IFE), pacing, vasopressors and ECMO] and no reports of using ECMO in the pediatric age group for this life threatening toxicity. Methods: A 5 y/o, with hx of supraventricular tachycardia(SVT) who failed propranolol, sotalol, amiodarone and digoxin was started on flecainide with better control for the past 2 yrs. He presented at the outside ER with a syncopal episode. ECG showed SVT with aberrancy. He received amiodarone and esmolol infusion; but suffered a cardiac arrest with pulseless electrical activity (PEA). He received epinephrine, atropine, NaHCO3, glucagon and IFE with ROSC. He was placed on Veno-Arterial (VA) ECMO due to persistent SVT. The initial flecainide level was 3.48 decreased to 1.68 μg/dL in 6 hrs (normal 0.2–1 μg/dL). IFE was continued and his cardiac function improved over 2 days leading to d/c of ECMO. He was discharged home with good neurological exam. Results: Flecainide toxicity is associated with high risk of mortality with no clear consensus guidelines for its management. IFE has been used successfully for local anesthetic & lidocaine toxicity. It is based on 1. the “lipid-sink” theory which suggests that the lipophilic drug is sequestered with reduced activity in the myocytes 2. positive inotropic effects due to efficient fatty acid metabolism. Toxic effects of flecainide are reversible with decreasing level due to metabolism and elimination. The V-A ECMO provides CRsupport and maintains organ perfusion. To our knowledge, we present the first case of flecainide toxicity successfully treated with both IFE and ECMO in a pediatric age group.

9: PREVALENCE AND PREDICTORS OF CLABSI ASSOCIATED WITH PICCS IN HOSPITALIZED EARLY CHILDHOOD POPULATION

Learning Objectives: Peripherally inserted central catheters (PICCs) are commonly placed in hospitalized children for a variety of reasons. Any central line is a potential risk for Central Line Associated Blood Stream Infection (CLABSI). It is unclear if certain PICC associated variables predict occurrence of CLABSI in early childhood. We hypothesize that a mix of patient, provider and PICC-related variables are associated with development of CLABSI in the hospitalized early childhood population. Methods: We performed a retrospective analysis of all hospitalized children between the age of 1 to 5 years who had PICC placed at our institution from Jan 2010 to Dec 2016. The primary outcome was development of CLABSI. The independent variables were age, gender, race, location of PICC placement (PICU, Floor, Operating Room, Sedation unit, Burn unit, Interventional radiology or Catheterization laboratory), presence of central venous catheter at PICC placement, use of ultrasound guidance, number of attempts, lumen size, length inside and outside of the skin, need for a surgical procedure, presence of cardiac condition and the number of days the PICC was present during the hospitalization. The simultaneous effect of the independent variables on development of CLABSI was examined by a multivariable logistic regression model. Results: During the 7-year period, a total of 392 PICCs were placed in children aged 1 to 5 years. The rate of CLABSI was 3.1% of the study group. Following adjustment for all available patient, provider, location and PICC confounders, each increase in length of PICC outside the skin was associated with significantly higher odds for developing CLABSI (OR = 1.30;95% CI = 1.071.57; p = 0.008). PICCs placed in the operating room were associated with significantly higher odds for developing CLABSI when compared to placement in the PICU (OR = 5.38; 95% CI = 1.03–29.19; p = 0.04). Conclusions: The presence of residual catheter “outside” the skin and each increase in the length “outside” the skin was an independent predictor for development of CLABSI in hospitalized early childhood cohort. In addition, PICC placement in the OR was at a higher risk of developing subsequent CLABSI. Our findings may have practical implications.

598: MANAGEMENT OF CYTOKINE STORM SYNDROME

Critical Care Medicine • Volume 46 • Number 1 (Supplement) www.ccmjournal.org Learning Objectives: Cytokine storm syndromes (CSS) are a group of disorders with uncontrolled, life threatening systemic inflammation due to a variety of causes. Hemophagocytic lymphohistiocytosis (HLH), macrophage activation syndrome and Hyperferritinemic sepsis fall under the spectrum of CSS. Patients usually present with prolonged fever and may mimic septic shock. This uncontrolled inflammation can lead to multiorgan failure and death. Patients usually present with prolonged fever, may have multi organ involvement, ferritin is usually > 10,000 ng/dl. Serological studies usually show elevated soluble interleukin-2 receptor. NK cell activity may be low or absent. Bone marrow exam may show hemophagocytes, but is not necessary for diagnosis. Therapy in CSS is directed at restoration of immune homeostasis. The management of secondary HLH/MAS is not standardized. Many treat MAS with an HLH-04-like approach, but less toxic strategies targeted at specific inflammatory defects seem to have better outcomes. We are reporting our experience in management of CSS with less toxic and less immunosuppressive medications Methods: Data for this descriptive study was collected retrospectively. All patients diagnosed with CSS or disorders under the spectrum of CSS were included. Basic statistics was used to calculate median, averages and percentage reduction in serum ferritin levels. Primary outcome was survival to discharge. Secondary outcome was percent reduction in serum ferritin and survival. Results: We had a total of 9 patients diagnosed with CSS over a period of 3 years at our institution. 67% (6/9) patients survived and were discharged from the PICU. Overall in hospital mortality was 33% (3/9). 44% (4/9) patients were diagnosed with MAS. Except one all patients diagnosed with MAS received anakinra, intravenous immunoglobulins (IVIG), methylprednisolone and all patients survived to discharge. 33% (3/9) patients were diagnosed with HLH. Two patients with HLH survived to discharge. The patients who survived had a median reduction in ferritin by 84% v/s 62% in non-survivors at the time of discharge. Conclusions: CSS is a spectrum of disorders with life threatening systemic inflammation. There has been sparse evidence on the approach to manage these patients. In our experience so far we found significant survival with limited immunosuppression avoiding chemotherapy and its side effects. Serum ferritin seems to be a reliable marker for diagnosis and to monitor response to therapy in patients diagnosed with CSS

A Rare Case of Isolated Congenital Asplenia Presenting in Septic Shock: Howell-Jolly Bodies a Clue to Early Diagnosis:

An 8-month-old female infant, previously healthy with up to date immunizations, presented to an outside emergency department with fever of 103°F. She had signs/symptoms consistent with upper respiratory tract infection since past 1 week. She was managed symptomatically in the emergency department and discharged home. However, she returned with vomiting, lethargy, and signs of dehydration. She was fluid resuscitated and initially admitted to the pediatric floor. She received broad spectrum antibiotics after the cultures were obtained. Over the next several hours, she progressively developed hemodynamic instability and respiratory compromise requiring intubation, mechanical ventilation and initiation of vasoactive/inotropic support. She was transferred to our pediatric intensive care unit for further evaluation and advanced care. Within an hour of presentation, she needed higher doses of vasopressors, including epinephrine (0.2 μg/ kg/min), norepinephrine (0.15 μg/kg/min), and vasopressin (0.4 mU/kg/min) infusions. Echocardiography showed normal anatomy and biventricular function. Initial laboratory workup demonstrated leukopenia 4400/mm3 (normal range, 600017 500/mm3), thrombocytopenia 48 000/mm3 (normal range, 150 000-400 000/mm3), and hemoglobin 13.1 g/dL with HowellJolly bodies on the peripheral blood smear (Figure 1). She also had elevated blood urea nitrogen (BUN) 28 mg/dL (normal range, 10-20 mg/dL), creatinine 1.2 mg/dL (normal range, 0.2-0.4 mg/dL) consistent with renal dysfunction, elevated aspartate transferase (AST) 2446 U/L (normal range, 10-50 U/L) and alanine transferase (ALT) 661 U/L (normal range, 3-30 U/L), and plasma ammonia 85 μmol/L (normal range, 21-50 μmol/L) consistent with hepatic dysfunction. Elevated prothrombin time (PT) 30 seconds (normal range, 9-12 seconds), partial thromboplastin time (PTT) 87 seconds (normal range, 22-31 seconds), and decreased fibrinogen 142 mg/dL (normal range, 180-400 mg/dL) were consistent with disseminated intravascular coagulation (DIC). She had severe lactic acidosis 12.1 mEq/L (normal range, 0.5-2.2 mEq/L).

Peripherally inserted central catheter (PICC) placement: beware of the bends

The peripherally inserted central catheter (PICC) is used as a long-term vascular access to deliver medications and venous nutrition. The PICC tip terminates close to the heart or in one ofxa0the great vessels—the superior vena cava or thexa0inferior vena cava. Catheter tip confirmation is usually achieved by plain radiography. We describe a case of axa09-month-old boy with complex congenital heart disease (heterotaxy syndrome, polysplenia type) who underwent pulmonary artery banding to reduce pulmonary blood flow to control heart failure symptoms. PICC was placed in the left femoral vein during …

Prostaglandin E1-Induced Periostitis and Reversibility with Discontinuation

A 4.1 kg full term infant with a known diagnosis of hypoplastic left heart syndrome was admitted to our pediatric intensive care unit after birth. Prostaglandin E1 (PGE1) infusion at 0.03 mg/kg/min was initiated immediately and continued thereafter for a prolonged period. He was listed for heart transplantation because of persistent poor right ventricle function and significant atrio-ventricular valve regurgitation. He underwent pulmonary arteries band placement to prevent excessive pulmonary blood flow and worsening heart failure. After being on PGE1 for 33 days, the cortical bone thickening was noted on all his long bones (Figure). The cortical bone thickening and intracortical bone remodeling progressively worsened to give the “bone-in the bone” appearance. He was successfully transplanted, and PGE1 was discontinued after 103 days. The bone changes persisted for 60 days after discontinuation of PGE1, followed by complete resolution on imaging obtained at 120 days. PGE1 intravenous infusion is being used in infants with ductal (ductus arteriosus) dependent congenital heart disease to maintain ductal patency and sustain life until palliative or corrective surgery is performed; or until heart transplant is possible. Cortical bone thickening is a known adverse effect of prolonged PGE1 infusion. In most instances, these infusions are for short duration, usually <7 days, before surgical options are considered. However, with the advent of technology, newer procedures (eg, hybrid procedure for hypoplastic left heart syndrome), and the intent to optimize care of the pre-term/low birth weight neonates with complex ductal dependent congenital heart diseases, PGE1 is being used for a longer duration of time before surgical options are considered. It is, thus, pertinent to be aware of the “common” side effects related to the “prolonged” use of PGE1. A wide array of side effects related to PGE1 infusion has been reported in literature. These include the common cutaneous vasodilatation, apnea or hypoventilation, fevers, diarrhea, neuroirritability, seizures, and flushing; the rare gastric outlet obstruction related to PGE1 induced gastric foveolar hyperplasia; and the life-threatening increased risk of spontaneous or surgically related rupture of the ductus arteriosus. Cortical proliferation of the skeletal system with PGE1 infusion has been reported in both animal and clinical studies. Clinical periostitis is seen with both shortand long-term use of PGE1 infusion. These changes have been reported as early as within 9 days of start of infusion. Complete resolution of periostitis related to PGE1 use is well documented in prior experiences. The time to resolution of periostitis can vary from few weeks to months after stopping the medication. This is clinically relevant because periostitis usually presents with limb pain, swelling, fevers, and overall irritability. The differential diagnosis of diffuse periosteal reaction in an infant is wide. It mainly includes the infections, metabolic disorders (calcium-hormonal dysregulation), chronic inflammatory disorders Juvenile Idiopathic Arthritis, cystic fibrosis, and the rare congenital anomalies. Congenital syphilis can produce similar bony changes but can be excluded with good history, examination, and limited clinical or laboratory evidence. Caffey disease or infantile cortical hyperostosis is a very rare inherited disease affecting mandible, clavicle, scapula, and long bones with contagious connective tissue. It is characterized by fever, soft tissue swelling, and hyperirritability. In our patient, extensive infectious, inflammatory, and metabolic work-up was negative. Knowledge of side effects related to prolonged PGE1 infusion, including the awareness of reversibility of periostitis upon discontinuation of the drug, may prevent the frequent and unnecessary testing/management in this cohort. ■

Cheyne-Stokes respiration: poor prognostic sign in a patient with heart failure

Patients with congestive heart failure (CHF) have high incidence of sleep-disordered breathing. Two distinct types are known: obstructive sleep apnoea (OSA) and Cheyne-Stokes respiration (CSR).1 Effective heart failure treatment improves CSR but not OSA, indicating that the development of CSR is secondary to heart failure. CSR is characterised by recurrent episodes of central apnoea/hypopnoea interposed with periods of hyperpnoea with waning and waxing pattern of tidal volume. Axa05-month-old girl who presented with acute onset of …

Pulsus alternans: a visual clue to a grave disorder!

A 15-year-old, previously healthy teenager who had fatigue, shortness of breath, vomiting, chest discomfort, lower extremity oedema and inability to lay flat for 3 weeks prior to her initial presentation. She was warm, well perfused with alternating strong and weak central as well as peripheral pulses and laterally displaced cardiac impulse. Her monitoring showed visual evidence of ‘pulsus alternans’—alternating low and high amplitude waveforms on the arterial waveform and plethysmography (figure 1). Her echocardiography showed severely …