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OBJECTIVE The Glenn procedure may lead to the development of elevated cerebral venous pressures, which is believed to result in “Glenn headaches.” This manifests as excessive irritability, often requiring significant use of opioids and benzodiazepines. This study was designed to report our experience with the use of phenobarbital in the postoperative phase after the Glenn procedure. METHODS We performed a retrospective chart review to compare Glenn patients before and after implementation of a sedation protocol using phenobarbital. The 2 groups were compared for demographics, surgical characteristics, and cumulative sedation usage. Correlation coefficients between the preoperative catheterization variables and sedation usage were also calculated. RESULTS Groups A (pre-phenobarbital; n = 8) and B (post-phenobarbital; n = 11) were comparable in terms of demographics, cardiac anatomy, preoperative catheterization data, and hemodynamics. Patients in Group B received a median dose of 21.8 mg/kg of phenobarbital during their ICU stay. Although there was a decreased administration of morphine equivalents (2.60 mg/kg vs 2.25 mg/kg, p = 0.38), benzodiazepine (0.1 mg/kg vs 0.074 mg/kg, p = 0.43), and dexmedetomidine (47 mcg/kg vs 37.2 mcg/kg, p = 0.53) in Group B, the differences were not statistically significant. There was also no strong correlation between preoperative hemodynamic variables and the postoperative sedation requirement, and there was no statistically significant difference in overall outcomes between the 2 groups. CONCLUSIONS While phenobarbital may have mitigated the use of opioids, benzodiazepines, and alpha-agonist agents in some postoperative Glenn patients, the overall findings for all patients were not statistically significant. Further prospective studies are needed to ascertain the role of phenobarbital in these patients.

Dilated cardiomyopathy is the most common presentation of cardiomyopathy in children with 20–35% of patients having an identified genetic component. There are more than 30 genes implicated in the pathogenesis of dilated cardiomyopathy. We present the first report of a female infant with dilated cardiomyopathy with a genetic variant in the dispatched RND transporter family member 1 gene.

Purpose This article assesses the correlation of respiratory variation in inferior vena cava (IVC) with central venous pressure (CVP) in children. Secondary objective was to evaluate IVC variability with clinical hydration status. Methods IVC variability was assessed at the subcostal (SC) and right lateral (RL) region, and collapsibility index (CI) (spontaneously breathing) and distensibility index (DI) (positive pressure) and IVC/aortic ratio were calculated. Partial correlations were calculated between CI/DI with CVP adjusting for body mass index and age. Sensitivity of CI and DI to predict clinical dehydration was calculated using receiver operating characteristic curves. Results A total of 145 ultrasounds were performed on 72 patients (41% positive pressure). Only RL CI in spontaneously breathing patients strongly correlated with CVP (r = –0.65, p Conclusion SC CI is the most reliable measure to assess the hydration status of spontaneously breathing children, while the IVC/aortic ratio performs well for patients under positive pressure. RL CI has strong negative correlation with CVP in spontaneously breathing patients.

There are multiple management strategies and a wide array of therapeutic options in the management of pulmonary hypertension in children. This survey was designed to assess the current trends in diagnosis and management of pulmonary hypertension including use of inhaled nitric oxide (iNO) and utilization of other pulmonary vasodilators. A 21 question web-based survey was created on a survey design program (Qualtrics®) and distributed to participating pediatric ICUs in the virtual PICU systems network. The survey content and design was reviewed by the institutional review board and approved as exempt research. Survey was distributed to 135 institutions with a 23% response rate (31/135). Of all the respondents, 24 were pediatric intensivists; six described themselves as cardiac intensivists, and one cardiologist. Fifty percent of mixed PICU and CICU units had a protocol for iNO use compared 37% of primary pediatric cardiac ICU. 10/31 (32.2%) respondents used iNO more than 20 times a year. Sildenafil was the main second-line agent with 8/31 (25.8%) reported using sildenafil drip for refractory pulmonary hypertension. 23/31 (74.1%) use inhaled Flolane. Only 12/31 (38.7%) respondents weaned iNO within the first 24 to 48 h, 8/31 (25.8%) did not have a defined timeline of iNO use and based it off of clinical response. 27/31 (87%) primarily used iNO in children less than one year of age. There is need for standardization of iNO usage across institutions to be able to generate evidence of its efficacy and indications, dosage, and weaning strategies.

Survival of children with functionally univentricular hearts has improved due to staged correction, innovative venous-pulmonary artery connections, and structured perioperative care. A rapidly growing number of these patients are reaching adulthood and are being admitted to intensive care units in greater numbers. Although many adults with functionally univentricular hearts will have undergone a Fontan operation, some will present unoperated or with incomplete single ventricle surgical palliation. Acute on chronic heart failure and the end-organ manifestations of this physiologic state are the most common indications for intensive care unit admission. Given the unique cardiovascular anatomy, physiology, and non-cardiac comorbidities present in this patient population, an understanding of the nuances in cardiac critical care evaluation and management is necessary to achieve optimal outcomes. Access to medical and surgical subspecialists with expertise in adult congenital heart disease is often needed. Thus, when feasible, intensive care for this patient population should be provided in a regional adult congenital heart disease center. In this chapter, we summarize the most common indications for intensive care admission in adults with functionally univentricular hearts, as well as the general principles regarding evaluation in the context of critical illness. We also review critical care management strategies that seem most appropriate based upon the unique physiologic characteristics and cardiopulmonary interactions inherent to this patient population.

To describe an infant with Lemierre-like syndrome caused by community-associated methicillin-resistant Staphylococcus aureus whose course was complicated by hemorrhagic pericarditis.Case report.A 30-bed, pediatric intensive care unit at a tertiary care children's hospital.A 7-month-old infant presented with fever and torticollis attributable to a retropharyngeal abscess and left internal jugular venous thrombosis. He was treated with antibiotics and anticoagulation, and his course was complicated by hemorrhagic pericarditis and cardiac tamponade.Resuscitation of shock; video-assisted thoracoscopic drainage of bilateral empyema with pleural decortication; vancomycin and clindamycin treatment of methicillin-resistant Staphylococcus aureus; incision and drainage of retropharyngeal abscess; treatment of internal jugular venous thrombus with anticoagulation; and treatment of pericardial tamponade by insertion of pericardial drain.Methicillin-resistant Staphylococcus aureus from blood and pleural fluid peel cultures were multi-locus sequence type 8, Panton-Valentine leukocidin-positive, and contained SCCmec IV, findings consistent with USA300 pulsotype. There was complete recovery from this life-threatening infection.Septic jugular venous thrombophlebitis complicating upper airway infections is a rare but serious acute medical condition. We present an infant with methicillin-resistant Staphylococcus aureus infection and clinical features that overlapped those described by Lemierre, in whom hemorrhagic pericarditis developed while receiving anticoagulation therapy. Anticoagulation, if used, should be closely monitored in this population.

Objective We aimed to assess the willingness of Muslim Americans to be potential organ donors, to describe potential religious barriers to organ donation, and to evaluate the efficacy of a brief religious educational intervention. Methods Face-to-face survey with English-, Arabic-, and Urdu-speaking Muslim American adults in places of worship and gatherings. The two-part survey included questions about demographics and organ donation. A brief educational intervention followed, which included an explanation of organ donation, along with the evidence for Islam's support for organ donation. After this intervention, the questions about organ donation and brain death were repeated. Results The response rate was 81% (231 of 285). Fifty percent of the respondents would donate their organs. Twenty-five percent changed their opinion and accepted the idea of being donors after the educational intervention. Lack of awareness of the support of Islam to organ donation and fear of disfigurement were the most common barriers cited. Conclusion Muslim Americans are less likely than Caucasian Americans to be organ donors, and the perceived lack of support from Islam for organ donation is a factor. The effectiveness of our brief religious education intervention suggests that further education may improve organ donation rates among the Muslim community.