Validation of administrative claims to identify ultrasound enhancing agent use.

BACKGROUND: Ultrasound enhancing agents (UEAs) are an invaluable adjunct to stress and transthoracic echocardiography (STE) to improve left ventricular visualization. Despite multiple single center studies evaluating UEA use, investigation into the rates, sources of variation, and outcomes of UEA use on a national level in the United States (US) has been limited by lack of validation of UEA codes for claims analyses. METHODS: We conducted a retrospective cross-sectional study, 2019-2022, using linked multicenter electronic medical record (EMR) data from > 30 health systems linked to all-payor claims data representing > 90% of the US population. Individuals receiving STE in both EMR and claims data on the same day during the study window were included. UEA receipt as identified by presence of a Current Procedural Terminology (CPT) or National Drug Code (NDC) for UEA use within 1-day of the index STE event. We evaluated the performance of claims to identify UEA use, using EMR data as the gold standard, stratified by inpatient and outpatient status. RESULTS: Amongst 54,525 individuals receiving STE in both EMR and claims data, 12,853 (23.6%) had a UEA claim in EMR, 10,461 (19.2%) had a UEA claim in claims, and 9140 (16.8%) had a UEA claim in both within the 1-day window. The sensitivity, specificity, accuracy, positive, and negative predictive values for UEA claims were 71.1%, 96.8%, 90.8%, 87.4%. and 91.6% respectively. However, amongst inpatients, the sensitivity of UEA claims was substantially lower (6.8%) compared to outpatients (79.7%). CONCLUSIONS: While the overall accuracy of claims to identify UEA use was high, there was substantial under-capture of UEA use by claims amongst inpatients. These results call into question published rates of UEA use amongst inpatients in studies using administrative claims, and highlight ongoing need to improve inpatient coding for UEA use.

Epidemiology of rabies immune globulin use in paediatric and adult patients in the USA: a cross-sectional prevalence study.

OBJECTIVES: To compare the epidemiology of paediatric and adult patients receiving rabies immune globulin (RIG). DESIGN: Cross-sectional prevalence study. SETTING: Eligible participants from the Symphony Integrated Dataverse presenting between 2013 and 2019. PARTICIPANTS: All adult and paediatric patients with integrated claims and demographic data associated with RIG use from the Symphony Integrated Dataverse from 2013 to 2019. PRIMARY AND SECONDARY OUTCOME MEASURES: Prevalence of diagnoses and procedures associated with paediatric and adult patient population based on frequency of International Classification of Diseases (ICD-9/ICD-10) and Current Procedural Terminology codes, respectively. METHODS: We used mutual information to identify features that differentiate the paediatric from adult patient population. Prevalence ratios were calculated to compare adult and paediatric patients. RESULTS: There were 79 766 adult and 20 381 paediatric patients who met the inclusion criteria. Paediatric patients had a 5.92-fold higher prevalence of 'open wounds to the head; neck; and trunk', 3.10-fold higher prevalence of 'abrasion or friction burn of face; neck; and scalp except eye; without mention of infection', 4.44-fold higher prevalence of 'open wound of scalp; without mention of complication' and 6.75-fold higher prevalence of 'laceration of skin of eyelid and periocular area | laceration of eyelid involving lacrimal passages'. Paediatric patients had a 3.83-fold higher prevalence of complex repairs compared with adult patients (n=157, 0.7% vs n=157, 0.2%, respectively). CONCLUSIONS: Paediatric patients represent a significant proportion of the patient population receiving RIG, and are associated with higher prevalence of codes reporting repair of larger, more complex wounds in highly innervated anatomical regions. Dosing and administration of RIG must be informed by animal bite wound characteristics; clinicians should understand the differences between presentations in adults and children and treat accordingly.

The IDeaS initiative: pilot study to assess the impact of rare diseases on patients and healthcare systems.

BACKGROUND: Rare diseases (RD) are a diverse collection of more than 7-10,000 different disorders, most of which affect a small number of people per disease. Because of their rarity and fragmentation of patients across thousands of different disorders, the medical needs of RD patients are not well recognized or quantified in healthcare systems (HCS). METHODOLOGY: We performed a pilot IDeaS study, where we attempted to quantify the number of RD patients and the direct medical costs of 14 representative RD within 4 different HCS databases and performed a preliminary analysis of the diagnostic journey for selected RD patients. RESULTS: The overall findings were notable for: (1) RD patients are difficult to quantify in HCS using ICD coding search criteria, which likely results in under-counting and under-estimation of their true impact to HCS; (2) per patient direct medical costs of RD are high, estimated to be around three-fivefold higher than age-matched controls; and (3) preliminary evidence shows that diagnostic journeys are likely prolonged in many patients, and may result in progressive, irreversible, and costly complications of their disease CONCLUSIONS: The results of this small pilot suggest that RD have high medical burdens to patients and HCS, and collectively represent a major impact to the public health. Machine-learning strategies applied to HCS databases and medical records using sentinel disease and patient characteristics may hold promise for faster and more accurate diagnosis for many RD patients and should be explored to help address the high unmet medical needs of RD patients.

Use of Outpatient Intravenous Calcitropes for Heart Failure in the United States.

BACKGROUND: Outpatient calcitrope infusions-that is, the cardiac inotropes milrinone and dobutamine-are often used for bridge to transplantation and palliation in advanced heart failure, but few data exist about the real-world use of these agents. METHODS AND RESULTS: We used the Symphony Integrated DataVerse of commercial, managed Medicare, and Medicaid insurance claims of approximately 280 million people (2012-2020) to determine the incidence and characteristics of ambulatory calcitrope use. Demographics were calculated, including geographic densities at the metropolitan statistical area level. A population projection normalized for age, sex, and location was extrapolated to the total US population. Ambulatory dispensing of milrinone was found in 10,533 outpatients, 1867 in 2019. Ambulatory dobutamine use was found in 4967 outpatients, 836 in 2019. The 2019 total US projection was 3411 for milrinone and 1281 for dobutamine. The mean age was 62 years for milrinone and 68 for dobutamine. Males represented 70% of use. There were differences between drugs in geographic distribution, with more milrinone use in the Northeast and South and more dobutamine use in the Midwest. Duration of use was 4.6 ± 7.2 months for milrinone and 1.8 ± 4.0 months for dobutamine. Of the patients receiving milrinone, 30.6% subsequently underwent cardiac transplantation or left ventricular assist device placement, whereas 10% receiving dobutamine went on to advanced therapies. Less than 0.5% of patients received calcitropes while enrolled in hospice care. CONCLUSIONS: More than 4000 patients receive outpatient infusion of calcitropes annually in the outpatient setting. Men are much more likely to receive these medications. A minority of the use is as a bridge to advanced therapies. Geographic variability in use suggests better evidence and consistent guidelines may be helpful.

Changes in near infrared spectroscopy during deep hypothermic circulatory arrest.

Monitoring cerebral oxygenation with near infrared spectroscopy may identify periods of cerebral desaturation and thereby the patients at risk for perioperative neurocognitive issues. Data regarding the performance of near infrared spectroscopy monitoring during deep hypothermic circulatory arrest are limited. The current study presents data regarding use of a commercially available near infrared spectroscopy monitor during deep hypothermic circulatory arrest in paediatric patients undergoing surgery for congenital heart disease. The cohort included 8 patients, 2 weeks to 6 months of age, who required deep hypothermic circulatory arrest for repair of congenital heart disease. The baseline cerebral oxygenation was 63 +/- 11% and increased to 88 +/- 7% after 15 min of cooling to a nasopharyngeal temperature of 17-18 degrees C on cardiopulmonary bypass. In 5 of 8 patients, the cerebral oxygenation value had achieved its peak value (either >or=90% or no change during the last 2-3 min of cooling on cardiopulmonary bypass). In the remaining 3 patients, additional time on cardiopulmonary bypass was required to achieve a maximum cerebral oxygenation value. The duration of deep hypothermic circulatory arrest varied from 36 to 61 min (43.4 +/- 8 min). After the onset of deep hypothermic circulatory arrest, there was an incremental decrease in cerebral oxygenation to a low value of 53 +/- 11%. The greatest decrease occurred during the initial 5 min of deep hypothermic circulatory arrest (9 +/- 3%). Over the entire period of deep hypothermic circulatory arrest, there was an average decrease in the cerebral oxygenation value of 0.9% per min (range of 0.5 to 1.6% decline per minute). During cardiopulmonary bypass, cooling and deep hypothermic circulatory arrest, near infrared spectroscopy monitoring followed the clinically expected parameters. Such monitoring may be useful to identify patients who have not achieved the highest possible cerebral oxygenation value despite 15 min of cooling on cardiopulmonary bypass. Future studies are needed to define the cerebral oxygenation value at which neurological damage occurs and if interventions to correct the decreased cerebral oxygenation will improve perioperative outcomes.

Measurement of central venous pressure from a peripheral intravenous catheter following cardiopulmonary bypass in infants and children with congenital heart disease.

The current study evaluates the feasibility and accuracy of measuring central venous pressure from a peripheral intravenous catheter following cardiopulmonary bypass in infants and children. Central venous pressure was simultaneously measured from a right atrial catheter and from a peripheral intravenous cannula. The continuity of the peripheral intravenous cannula with the central venous system was evaluated by noting the change in the pressure during a sustained inspiratory effort and during occlusion of the vessel above (proximal to) the catheter. The cohort for the study included 29 infants and children. In 5 of the 29 patients (17%), there was no increase in the peripheral venous pressure in response to a Valsalva maneuver or occlusion of the extremity proximal to the intravenous site. The difference between peripheral venous pressure and central venous pressure in these patients was 11 +/- 3 mm Hg versus 2 +/- 1 mm Hg in the patients in whom the peripheral venous pressure increased with these maneuvers (P < .0001). No clinically significant variation in the accuracy of the technique was noted based on the actual CVP value, size of the PIV, its location, or the patient's weight. Provided that the peripheral venous pressure increases to a sustained inspiratory breath and occlusion above the intravenous site, there is a clinically useful correlation between the peripheral venous pressure and the central venous pressure following cardiopulmonary bypass in infants and children with congenital heart disease.

Use of a ventricular assist device as a bridge to transplantation in a patient with single ventricle physiology and total cavopulmonary anastomosis.

Mechanical circulatory support can be used to manage acute and chronic cardiac failure in both adult and pediatric patients. Traditionally, extracorporeal membrane oxygenation (ECMO) has been the most common form of mechanical circulatory support in children. However, more recently, in cases of pure ventricular dysfunction, ventricular assist devices (VADs) have offered specific advantages over ECMO, including better ventricular recovery, reduced anticoagulation requirements, decreased use of blood products and decreased cost. We present the use of a VAD in an adolescent with single-ventricle physiology, who could not be weaned from cardiopulmonary bypass (CPB) after undergoing a revision of a modified Fontan operation. Gas exchange was provided by the patient's lungs while the centrifugal VAD was used successfully to support the circulation as a bridge, first to a totally implantable pulsatile VAD and subsequently to heart transplantation.

Tetralogy of Fallot, hypertrophic cardiomyopathy, and Down's syndrome: a rare and challenging combination.

A case of the combination of tetralogy of Fallot, hypertrophic cardiomyopathy, and Down's syndrome is reported here and is the first report on the combination of the 3 entities. Tetralogy of Fallot is often associated with chromosomal aberration, while hypertrophic cardiomyopathy associates with certain gene loci. Our experience with treating this patient, although ultimately unsuccessful, may provide useful information in any future cases.

Anesthetic care during modified fontan procedure without cardiopulmonary bypass.

OBJECTIVE: The purpose of this study was to report the anesthetic care of patients during performance of a Fontan procedure without cardiopulmonary bypass (CPB). DESIGN: Retrospective chart review. SETTING: Operating room of a university hospital. PARTICIPANTS: Seven pediatric-patients undergoing inferior vena cava (IVC)-to-pulmonary artery (PA) anastomosis for completion of the Fontan procedure. INTERVENTIONS: Charts were reviewed for anesthetic technique, hemodynamic and ventilatory changes occurring during the procedure, and anesthetic interventions that were provided. MEASUREMENTS AND MAIN RESULTS: The off-bypass Fontan procedure was attempted in 7 patients (age: 26 months-7 years, weight: 13 to 28 kg). Exposure of the PA was not feasible in 1 patient because of a markedly enlarged right atrium. In the remaining 6 patients, before cross-clamping of the PA to allow for the proximal anastomosis between the PA and the conduit, alkalosis (pH > or =7.5) was maintained by the administration of sodium bicarbonate. After PA cross-clamping, fluid administration was necessary in 5 patients and dopamine (3-7 microg/kg/min) was necessary in 4 patients. The minute ventilation was increased by 18 +/- 7% to maintain baseline PaCO2 values. Before the placement of the PA cross-clamp, the end-tidal PaCO2 difference was 7 +/- 4 mmHg and the transcutaneous (TC)-PaCO2 difference was 3 +/- 2 mmHg. The end-tidal PaCO2 difference increased to 14 +/- 6 mmHg during cross-clamping of the PA, whereas no change was noted in the TC-PaCO2 difference. Once the proximal anastomosis was completed, a bridge was placed to redirect blood from the IVC to the right atrium while the IVC was clamped and attached to the distal end of the conduit. After placement of the distal end of the bridge into the IVC, fluid administration to maintain the blood pressure was necessary in 3 patients. In 1 patient, 20 minutes after placement of the bridge, the authors noted a progressive increase in the central venous pressure reading measured from the left femoral vein and the need for the administration of volume to maintain the mean arterial pressure. Examination of the bridge revealed occlusion with thrombus despite an activated coagulation time value of 250 to 300 seconds. The tracheas of 3 of the 6 patients were extubated in the operating room, whereas the other 3 were extubated in the pediatric intensive care unit within 4 hours of completion of the procedure. The 1 patient who required the use of CPB required reintubation and had a protracted intensive care unit course. The other 6 patients were discharged home on postoperative days 7 to 12. CONCLUSIONS: With alteration of the anesthetic technique, the Fontan procedure can be performed in selected patients without the need for CPB.

Noninvasive monitoring of carbon dioxide in infants and children with congenital heart disease: end-tidal versus transcutaneous techniques.

End-tidal CO2 (ET(CO2)) monitoring and transcutaneous (TC) CO2 monitoring were prospectively compared in 53 patients, 1 month to 16 years of age, with congenital heart disease (CHD). There were 32 patients with cyanotic CHD and 21 with acyanotic CHD. The TC-Pa(CO2) difference was 2 +/- 1 mm Hg and the ET-Pa(CO2) difference was 5 +/- 3 mm Hg (P < .0001). The TC-Pa(CO2) difference was < or = 2 mm Hg in 30 of 53 patients and < or = 5 mm Hg in 53 of 53 patients. The ET-Pa(CO2) difference was < or = 2 mm Hg in 9 of 53 patients and < or = 5 mm Hg in 30 of 53 patients (P < .001). No variation in the TC-Pa(CO2) difference was noted based on the type of CHD (acyanotic vs cyanotic) or age. The ET-Pa(CO2) difference was greater in patients with cyanotic versus acyanotic CHD (7 +/- 3 mm Hg vs 4 +/- 2 mm Hg, P < .0001) and in patients < or = 1 year of age versus patients > or = 1 year of age (6 +/- 3 mm Hg vs 4 +/- 2, P = .008). In infants and children with CHD, TC monitoring provides a more accurate estimation of Pa(CO2) than ET monitoring.

Dexmedetomidine in the treatment of withdrawal syndromes in cardiothoracic surgery patients.

Dexmedetomidine (Precedex, Abbott Laboratories, Abbott Park, IL) is an alpha 2 adrenergic agonist that possesses a high ratio of specificity for the alpha 2 versus the alpha 1 receptor. It is currently approved for the provision of sedation during mechanical ventilation in adults. Given previous experience with clonidine for the treatment of substance withdrawal and the preliminary anecdotal experience with dexmedetomidine, it appears that dexmedetomidine may be a useful agent for treatment of substance withdrawal in the intensive care setting. The authors present their experience with the use of dexmedetomidine to control withdrawal behavior in 3 patients following cardiothoracic surgery. Previous reports regarding the use of dexmedetomidine to treat withdrawal and its potential application in this clinical arena are reviewed.

Argatroban for anticoagulation during cardiopulmonary bypass in an infant.

Heparin induced thrombocytopenia (HIT) is a rare, but potentially life-threatening complication of heparin therapy. In patients with HIT, alternative means of anticoagulation are necessary. The authors present an infant with HIT who required anticoagulation during cardiopulmonary bypass for tricuspid valve excision in the treatment of bacterial endocarditis. The direct thrombin inhibitor, argatroban, was successfully used. Previous reports regarding the use of argatroban and other nonheparin anticoagulants for anticoagulation are reviewed and suggestions regarding argatroban dosing in infants are presented.

Heparin-induced thrombocytopenia in the pediatric intensive care unit population.

OBJECTIVES: To report the occurrence of heparin-induced thrombocytopenia (HIT), discuss its pathophysiology, and outline an approach to management in the pediatric intensive care unit (ICU) patient. DESIGN: Retrospective case reports. SETTING: Pediatric ICU in a tertiary-care center. PATIENTS AND RESULTS: Two pediatric ICU patients (2 and 6 mos of age) who developed HIT in the pediatric ICU. One was receiving heparin as a flush solution through a central line and the other had full heparinization during cardiopulmonary bypass. Both had received heparin during their neonatal course and developed thrombocytopenia; however, HIT was not considered as a possible diagnosis. HIT was diagnosed using a heparin-induced platelet aggregation study. The thrombocytopenia resolved with the cessation of heparin administration. One of the patients developed a deep vein thrombosis around a femoral venous catheter. CONCLUSION: Although well described in the adult literature, there have been a limited number of reports of HIT in pediatric-aged patients. Given its potential for morbidity, HIT should be considered in the differential diagnosis of thrombocytopenia in the pediatric ICU patient.

Perioperative care of the patient with Williams syndrome.

Williams syndrome, initially described by Williams, Barratt-Boyes, and Lowe in 1961, consists of characteristic dysmorphic features, congenital heart disease, and distinctive behavioral and emotional traits. In addition to acquired and congenital heart disease, manifestations in the renal, endocrine, musculoskeletal, and central nervous system may have implications during the perioperative period. Congenital and acquired heart disease can be a significant issue as sudden death, related to abnormalities of the coronary arteries, has been reported perioperatively in these patients. The authors present a 7-month-old infant, previously diagnosed with Williams syndrome, who required anesthetic care for repair of subaortic and supravalvular aortic stenosis. The potential perioperative implications of Williams syndrome are discussed.

Nesiritide during extracorporeal membrane oxygenation.

Nesiritide is a recombinant formulation of B-type natriuretic peptide (BNP). Preliminary experience in the adult population has shown nesiritide to be an effective agent in the treatment of decompensated congestive heart failure (CHF) in adults. Given its physiological effects, it may be an effective agent in other clinical scenarios. We report the use of nesiritide in two infants during extracorporeal membrane oxygenation (ECMO). In one patient, nesiritide in doses up to 0.09 microg.kg(-1).min(-1) were used to control mean arterial pressure while in the other patient, doses of 0.01-0.03 microg.kg(-1).min(-1) were used to augment urine output. The potential applications of nesiritide and dosing regimens for this agent in the ECMO population are discussed.

Preliminary experience with nesiritide in the pediatric population.

Nesiritide is a recombinant formulation of brain-type natriuretic factor. Preliminary experience in the adult population suggests that nesiritide may be an effective agent in the treatment of decompensated congestive heart failure. Given its physiologic effects, it may be an effective agent in the pediatric population; however, to date, there are no reports regarding its use in infants and children. The authors retrospectively review their experience with nesiritide in 5 pediatric patients. The cohort of 5 patients included a diverse population with 2 patients who were status postcardiothoracic surgery, 2 with adult respiratory distress syndrome, and 1 in the recovery phase from septic shock. Although no direct measurement of cardiac output was feasible as none of the patients had a pulmonary artery catheter, other indicators of increased cardiac output were noted. These included improved peripheral perfusion with warming of the extremities and improvement of peripheral pulses in all of the patients, increased venous saturation in 2 of the patients, and maintenance of or increased urine output despite weaning or discontinuation of diuretics. In 3 of the patients, nesiritide was started as the primary agent to provide a decrease in systemic vascular resistance and augment cardiac output, while in the other 2 patients, nesiritide was used when other vasoactive agents failed to provide the desired effect or resulted in adverse effects.

Anaesthetic implications of LEOPARD syndrome.

LEOPARD syndrome is a neuroectodermal disorder presumed to result from an abnormality in neural crest cells. The acronym 'LEOPARD' is derived from the clinical features which include multiple lentigines, electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, retarded growth, and deafness. Given the multisystem nature of the disease process, several issues may affect the perioperative care of these patients. Of primary importance are associated conditions of the cardiovascular system including congenital heart disease, conduction disturbances, and progressive hypertrophic obstructive cardiomyopathy. The authors present a 4-year old boy who presented for anaesthetic care for repair of a ventricular septal defect and pulmonary valvotomy for congenital pulmonary stenosis. The potential perioperative implications of LEOPARD syndrome are discussed.