A retrospective analysis of a pediatric tele-echocardiography service to treat, triage, and reduce trans-Pacific transport.

Introduction Tele-echocardiography can ensure prompt diagnosis and prevent the unnecessary transport of infants without critical congenital heart disease, particularly at isolated locations lacking access to tertiary care medical centers. Methods We retrospectively reviewed all infants who underwent tele-echocardiography at a remote 16-bed level IIIB NICU from June 2005 to March 2014. Tele-echocardiograms were completed by cardiac sonographers in Okinawa, Japan, and transmitted asynchronously for review by pediatric cardiologists in Hawaii. Results During the study period 100 infants received 192 tele-echocardiograms: 46% of infants had tele-echocardiograms completed for suspected patent ductus arteriosus, 28% for suspected congenital heart disease, 12% for possible congenital heart disease in the setting of likely pulmonary hypertension, and 10% for possible congenital heart disease in the setting of other congenital anomalies. Of these, 17 patients were aeromedically evacuated for cardiac reasons; 12 patients were transported to Hawaii, while five patients with complex heart disease were transported directly to the United States mainland for interventional cardiac capabilities not available in Hawaii. Discussion This study demonstrates the use of tele-echocardiography to guide treatment, reduce long and potentially risky trans-Pacific transports, and triage transports to destination centers with the most appropriate cardiac capabilities.

Return on Investment Analysis of Health Experts onLine at Portsmouth: A 2-Year Review of the Navy's Newest Teleconsultation System.

BACKGROUND: Health Experts onLine at Portsmouth (HELP) is a web-based teleconsultation system launched in June 2014 to facilitate communication between specialists at Naval Medical Center Portsmouth and providers assigned to both the fleet forces and primary care clinics across the eastern United States, Europe, and the Middle East. Specialist consultations through the HELP system purport to improve access to care for patients who otherwise might be referred to the civilian network or medically evacuated (MEDEVACed) to Naval Medical Center Portsmouth for specialized care. If HELP-facilitated communications help avoid civilian referrals or MEDEVACs, the associated costs of that care should be reduced. METHODS: We evaluated cost savings associated with prevented MEDEVACs by analyzing both tangible savings (prevented costs of flights, per diems, and consults) and intangible savings (reduced lost productivity time). We compared these savings to the costs of maintaining and utilizing the HELP system: startup costs, administrative costs, and provider time costs. We used patient and provider data from the HELP database to evaluate clinical consult cases. Before this analysis, a panel of 3 physicians associated with HELP reviewed each consult to determine whether a case qualified as a prevented MEDEVAC. Data from the Military Health System (MHS) Management and Analysis Reporting Tool and the MHS Data Repository were used to estimate costs associated with provider time, patient time, and direct care medical encounters. FINDINGS: The HELP program delivered measurable, positive returns on investment (ROIs) between June 2014 and December 2015. In that time frame, 559 consult cases occurred in the HELP system. Of the 559 total consult cases, 50 consults prevented MEDEVACs. Incorporating only tangible savings, HELP produced an 80% ROI on the basis of prevented medical evacuations; the addition of intangible savings such as reduced lost productivity increased the ROI to 250%. The dollar values of these savings were $693,461 and $1,337,628, respectively. IMPACT: The HELP program produces considerable savings (both tangible and intangible) to the Military Healthcare System for small costs. It does this both by increasing access to care at previously inaccessibly remote medical treatment facilities and by consequently decreasing the forward provider's reliance on medical evacuation in questionable cases. This positive ROI was potentially underestimated as this analysis did not account for recapture of care that would otherwise have been sent to the civilian market. On the basis of this analysis, a low bandwidth, asynchronous, and internet accessible teleconsultation system is both a feasible and effective means of projecting quality care forward into the deployed setting. Future implementation of similar initiatives throughout the MHS can be expected, and will likely draw from the lessons learned during the successful implementation and execution of the HELP system.

Automated Bone Age Analysis with Lossy Image Files.

BACKGROUND: In our U.S. Department of Defense hospital system, pediatric endocrinology and radiology resources to evaluate bone age radiographs are limited. Our tertiary care center provides expert specialty support to remotely stationed beneficiaries at more than 30 Department of Defense medical facilities using a well-established, asynchronous, Health Insurance Portability and Accountability Act compliant system that allows for physician-to-physician teleconsultation. Up to 14% of these teleconsultations are for endocrinology assessment, many of which include bone age analysis. We sought to evaluate the feasibility of using an automated bone age analysis program using the file format most commonly provided to us, lossy JPEG image files saved at lower quality, to improve access to our consultation services. METHODS: All patients seen in the Tripler Army Medical Center pediatric endocrinology clinic, who were being evaluated for poor growth during the 2-month study period and who had a bone age film performed at Tripler Army Medical Center during that time, were eligible to have their deidentified bone age films analyzed. We imported lossy JPEG bone age image files from our hospital web viewer to BoneXpert, version 2.1, using a fully automated, custom built system that reconstructed each file's true resolution and then packaged the original image into a Digital Imaging and Communications in Medicine header. The original JPEG files were saved at 70% quality. Bone age readings were compared between our pediatric endocrinologists (ENDO), pediatric radiologists (RADS), and BoneXpert (BONE). Additionally, adult height prediction from ENDO and BONE were compared. FINDINGS: 35 bone age images were evaluated over a 2-month period. Most patients were being evaluated for idiopathic short stature or growth hormone deficiency. Analysis of variance showed no significant differences in mean bone age readings between the 3 groups (mean bone age reading = 9.0, 9.1, and 9.1 years for ENDO, RADS, and BONE, respectively, p = 0.827). Mean (SD) differences between physician and software bone age readings were -0.09 (0.89) years (ENDO) and -0.03 (1.01) years (RADS). Mean difference for adult height predictions was only -0.2 cm (p = 0.806). DISCUSSION: Automated analysis of lossy JPEG files of bone age images using the BoneXpert software appears to be feasible and accurate. Larger studies are needed to validate these results.

Extensive clinical experience: a simple guide to basal insulin adjustments for long-distance travel.

Long-distance travel across multiple time zones presents unique challenges for patients taking insulin, requiring adjustments in both timing and dosage of basal insulin when several times zones (≥5) are traversed. Travel across the International Date Line adds to the confusion, as existing resources and dosing calculators often do not account for the date change. We review recommendations from available guidelines and dosage calculators used for long-distance travel basal insulin adjustments and then present our patient handouts which allow for a safe, specific, single dose adjustment for eastward and westward travel. The included handouts are easy to use and can be freely reproduced for use in diabetes clinics.

Transthoracic echocardiography visualization of bicaval dual lumen catheters for veno-venous extracorporeal membrane oxygenation.

Bicaval dual lumen catheters improve the efficiency of veno-venous extracorporeal membrane oxygenation by minimizing recirculation with an innovative design, which requires precise placement of three catheter ports in the superior vena cava, right atrium, and inferior vena cava, respectively. However, the exact position of these catheter ports is usually not known during placement because they cannot be visualized with conventional radiography. We performed a retrospective review of our experience over the past year using transthoracic echocardiography to evaluate the position of the catheter ports. From a subcostal, sagittal imaging approach, we were able to identify all three catheter ports in 11 of 11 studies. At least one of the catheter ports was incorrectly positioned in 5 of 11 studies. Further prospective evaluation is necessary to determine if catheter repositioning based on transthoracic echocardiography findings can further improve the clinical efficiency of veno-venous extracorporeal membrane oxygenation.

Pacemaker upgrade causing new-onset heart failure in a patient with complete congenital atrioventricular block.

For infants with congenital complete atrioventricular block (CCAVB), the choice of pacing modalities is limited. Due to their size and surgical limitations, neonates typically start with an epicardial right ventricular pacing system, then upgrade to right-sided dual-chamber pacing once appropriate size is achieved. These modes are generally well tolerated. However, the reported case involved a patient with CCAVB who paradoxically experienced congestive heart failure after upgrading to a dual-chamber system, a theoretically superior pacing modality. With conversion to biventricular pacing, a relatively new modality for adults with very little pediatric experience to date, the patient's symptoms dramatically improved.

A portable graphical representation tool for phonocardiograms.

This paper describes a prototype software application to display graphical and editable representations of patient data for use in electronic medical records (EMRs). The application dynamically generates graphics of cardiac and other patient data, and displays or saves them both in graphic and in text formats. The presentation of heart and other data in a consistent, clinically familiar, graphical format is designed to reduce the time necessary for anyone to review and understand this important information. Results of preliminary testing on actual case data are encouraging.

Generalized arterial calcification of infancy associated with meconium peritonitis: a case report and review of the literature.

Generalized arterial calcification of infancy (GACI) is a rare genetic disorder consisting of diffuse arterial calcification and intimal proliferation. The disease typically results in progressive arterial stenosis and frequently leads to death from myocardial ischemia by 6 months of life. Affected infants are usually diagnosed before birth or in the neonatal period with symptoms of congestive heart failure. Therapy with bisphosphonate has been used to treat the condition, but with inconsistent results. The disease is associated with mutations in ENPP1 in the majority of the cases. Here we report a unique case of GACI associated with in utero meconium peritonitis and without coding region mutations of the ENPP1 gene. GACI should be considered in the differential diagnosis in infants presenting with arterial calcifications and congenital anomalies of the gastrointestinal tract.

Neonatal pulmonary artery thrombosis presenting as persistent pulmonary hypertension of the newborn.

Pulmonary artery thrombosis in neonates occurs rarely. This report describes the case of a term infant with a pulmonary artery thrombosis presenting as persistent pulmonary hypertension of the newborn. The risk factors identified in the case included maternal diabetes and heterozygous factor V Leiden deficiency. The pulmonary thrombus was successfully treated with percutaneous catheter-based embolectomy.

Utility of store-and-forward pediatric telecardiology evaluation in distinguishing normal from pathologic pediatric heart sounds.

Because pediatric cardiologists can accurately diagnose innocent murmurs by physical exam alone, the authors developed a system for remote cardiac auscultation. They hypothesized that their system could accurately classify auscultatory findings as normal/innocent or pathologic. Patients undergoing evaluation underwent examination, echocardiography, and heart sound recording. Pediatric cardiologists evaluated the heart sounds and classified the case as either normal/innocent or pathologic. They reviewed103 heart sound data sets; 85% of the cases were accurately classified as either normal/innocent or pathologic, with a sensitivity of 82% and specificity of 86%. However, when accounting for clinical diagnosis, reviewer uncertainty, and ECG abnormalities, the sensitivity and specificity improved to 91% and 88% (accuracy 89%), respectively. Degree of certainty with the telecardiology diagnosis correlated with correct interpretation (P < .005). Digital heart sound recordings evaluated via telemedicine can distinguish normal/innocent murmurs from pathologic ones. Such a system could improve the use of pediatric cardiology services.

Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome.

OBJECTIVE: To test the hypothesis that chronic beta-blocker therapy in pediatric patients with Marfan syndrome alters the rate of aortic root dilation. Beta-blockade has been advocated as preventive therapy for Marfan syndrome based on reports indicating a decreased rate of aortic root dilation in treated patients. STUDY DESIGN: Patients with Marfan syndrome (n = 63) followed at Children's Hospital of Pittsburgh or Children's Hospital of New York-Presbyterian who had > or =18 months of echocardiographic follow-up were studied. All clinical data and 213 serial echocardiograms were reviewed, and aortic root dimensions were measured. Patients were divided into 2 groups for comparison: untreated (n = 34) and treated (n = 29). RESULTS: At study entry, the 2 study groups were comparable in terms of age, sex, body surface area (BSA), aortic root measurements, heart rate, and corresponding z scores. Follow-up duration in each group was similar. At last follow-up, heart rates and heart rate z scores were lower in the treated group. Rates of change of aortic root measurements (P = .52) and the corresponding z scores were not statistically different between the 2 group at the study's end. CONCLUSIONS: This study suggests that that beta-blocker therapy does not significantly alter the rate of aortic root dilation in children with Marfan syndrome. Based on these data, the recommendation of lifetime beta-blocker therapy instituted during childhood should be reassessed.

Comparison of two educational interventions on pediatric resident auscultation skills.

OBJECTIVE: Multiple cross-sectional physician surveys have documented poor cardiac auscultation skills. We evaluated the impact of 2 different educational interventions on pediatric resident auscultation skills. METHODS: The auscultation skills of all first-year (PGY1; n = 20) and second-year pediatric residents (PGY2; n = 20) were evaluated at the beginning and end of the academic year. Five patient recordings were presented: atrial septal defect, ventricular septal defect, pulmonary valve stenosis, bicuspid aortic valve with insufficiency, and innocent murmur. Residents were asked to classify the second heart sound, identify a systolic ejection click, describe the murmur, and provide a diagnosis. All PGY1 and most PGY2 (14 of 20) participated on the inpatient cardiology service for 1 month. PGY2 on the cardiology service also attended outpatient clinic. PGY1 did not attend outpatient clinic but were allotted 2 hours/week to use a self-directed cardiac auscultation computer teaching program. RESULTS: Resident auscultation skills on initial evaluation were dependent on training level (PGY1: 42 +/- 15% correct; PGY2: 53 +/- 13% correct), primarily as a result of better classification of second heart sound (PGY1: 45%; PGY2: 63%) and diagnosis of an innocent murmur (PGY1: 35%; PGY2: 65%). There was no difference in the ability to identify correctly a systolic ejection click (20% vs 23%) or to arrive at the correct diagnosis (35% vs 40%). At the end of the academic year, the PGY1 scores improved by 21%, primarily as a result of improved diagnostic accuracy of the innocent murmur (35% to 65%). PGY2 scores remained unchanged (53% vs 51%), regardless of participation in a cardiology rotation (cardiology rotation: 50%; no cardiology rotation: 51%). Combined, diagnostic accuracy was best for ventricular septal defect (55%) and innocent murmur (60%) and worst for atrial septal defect (18%) and pulmonary valve stenosis (15%). However, 40% identified the innocent murmur as pathologic and 21% of pathologic murmurs were diagnosed as innocent. CONCLUSIONS: Pediatric resident auscultation skills were poor and did not improve after an outpatient cardiology rotation. Auscultation skills did improve after the use of a self-directed cardiac auscultation teaching program. These data have relevance given the American College of Graduate Medical Education's emphasis on measuring educational outcomes and documenting clinical competencies during residency training.

Tacrolimus dosage requirements after initiation of azole antifungal therapy in pediatric thoracic organ transplantation.

Azole antifungals inhibit the metabolism of tacrolimus mediated by CYP3A4. Upon initiation of azole therapy, the required dose reduction of tacrolimus is unknown. We reviewed our experience with azole antifungals in our pediatric thoracic transplant population receiving tacrolimus. Tacrolimus levels and dosage requirements were compared before and during azole therapy. Thirty-one patients received both tacrolimus and an azole antifungal (fluconazole = 9, itraconazole = 22). The tacrolimus dose was empirically reduced by approximately one-third when azole therapy was initiated. Mean tacrolimus dose requirements decreased by 68% within the first month of therapy (pre-azole: 0.27 +/- 0.14 mg/kg/day; 30 day post-azole: 0.087 +/- 0.069 mg/kg/day; p < 0.001). Despite a mean decrease in tacrolimus dose from baseline of 33, 42, and 55% on day 1, 2, and 4 of azole therapy, respectively, there was still an unintended 38% increase in tacrolimus levels during the first month of azole therapy. A calculated dose-reduction protocol of 50% on day of azole initiation, 70% on day 3, and 75% on day 14 should result in minimal mean changes in the tacrolimus levels. There was no difference in tacrolimus dose reduction between fluconazole and itraconazole groups. Azole antifungals markedly decrease tacrolimus requirements within the first few days of therapy. An initial reduction in tacrolimus dose by one-third is insufficient, and dose reduction of at least 50% upon azole initiation seems warranted. Once azole antifungal therapy is initiated, frequent therapeutic drug monitoring is required.