Case Report: Anesthetic Management and Electrical Cardiometry as Intensive Hemodynamic Monitoring During Cheiloplasty in an Infant With Enzyme-Replaced Pompe Disease and Preserved Preoperative Cardiac Function

Introduction: Pompe disease is caused by deficiency of the lysosomal enzyme acid α-glucosidase, which results in cardiac and muscular complications that can jeopardize perioperative outcomes. We report a 4-month-old infant with Pompe disease receiving cheiloplasty under general anesthesia with the aid of peripheral nerve blocks and intensive hemodynamic monitoring.Case Description: This case report describes a 4-month-old full-term Taiwanese female infant who presented with left unilateral cleft lip and palate in the prenatal examination. She was diagnosed with infantile-onset Pompe disease after acidic α-glucosidase (GAA) gene sequencing. She also received enzyme replacement therapy (ERT) 15 days after birth and regular ERT every other week. Cheiloplasty was performed under general anesthesia uneventfully, and peripheral nerve blocks were adopted for analgesia. Intensive hemodynamic monitoring using electrical cardiometry technology (ICON®) and pulse contour analysis (FloTrac system) were applied during the operation. No adverse effects were observed, and the wound healed well. Therefore, the patient was discharged 4 days after surgery.Conclusion: With the availability of ERT, severe organ dysfunction in infantile-onset Pompe disease patients is no longer common. However, moderate cardiac depression can still occur while increasing inspiratory pressure and deepening the anesthesia level despite a normal preoperative echocardiogram report. Therefore, careful, gradual titration is desirable. Furthermore, electrical cardiometry can detect hemodynamic changes more instantaneously and reliably than pulse contour analysis. In addition, we suggest taking advantage of the peripheral nerve block as a part of balanced anesthesia to alleviate the cardiac suppression caused by general anesthesia.

In-Silico Evaluation of Anthropomorphic Measurement Variations on Electrical Cardiometry in Neonates

Non-invasive cardiac output methods such as Electrical Cardiometry (EC) are relatively novel assessment tools for neonates and they enable continuous monitoring of stroke volume (SV). An in-silico comparison of differences in EC-derived SV in relation to preset length and weight was performed. EC (ICON, Osypka Medical) was simulated using the “demo” mode for various combinations of length and weight representative of term and preterm infants. One-centimetre length error resulted in a SV-change of 1.8–3.6% (preterm) or 1.6–2.0% (term) throughout the tested weight ranges. One-hundred gram error in weight measurement resulted in a SV-change of 5.0–7.1% (preterm) or 1.5–1.8% (term) throughout the tested length ranges. Algorithms to calculate EC-derived SV incorporate anthropomorphic measurements. Therefore, inaccuracy in physical measurement can impact absolute EC measurements. This should be considered in the interpretation of previous findings and the design of future clinical studies of EC-derived cardiac parameters in neonates, particularly in the preterm cohorts where a proportional change was noted to be greatest.

Signal Quality of Electrical Cardiometry and Perfusion Index in Very Preterm Infants

<b><i>Objective:</i></b> The use of noninvasive monitoring of neonatal hemodynamics is increasing in neonatal care. Methods include noninvasive cardiac output estimated by electrical cardiometry (EC) and peripheral perfusion as perfusion index (PI) using pulse oximetry. Our aim was to evaluate the feasibility to continuously monitor preterm infants with EC and PI during the first 2 postnatal days and the effects of averaging EC data in signal quality (SigQ) analysis. <b><i>Design:</i></b> Prospective observational study. <b><i>Setting:</i></b> Tertiary neonatal academic hospital. <b><i>Patients:</i></b> Preterm infants &#x3c;32 weeks gestation from birth until 48 h. <b><i>Main Outcome Measures:</i></b> Continuous EC and PI measurements. Feasibility was quantified as the time with high SigQ, classified using SigQ index in EC and exception codes in PI. Our predefined threshold for good feasibility was minimum of 24 h with high SigQ for both. <b><i>Results:</i></b> Twenty-two preterm infants (median [IQR] gestational age 28 + 6 (26 + 0, 30 + 4) weeks + days, birth weight 960 [773, 1,500] g) were included. We recorded a minimum of 24 h with high SigQ in 14 infants for EC (unaveraged data) and 22 infants for PI measurements. The median (range) % of recording time with high SigQ was 74% (50%, 88%) for EC and 94% (82%, 96%) for PI. Using 1 minute averaging for EC data resulted in an increase of infants with minimum 24 h of high SigQ to 21 infants. <b><i>Conclusions:</i></b> EC and PI monitoring are feasible in preterm infants within the first 48 h, but SigQ remains problematic for EC. Signal dropout is masked in averaged EC values.

Neonatal Septic Shock and Hemodynamic Monitoring in Preterm Neonates in a NICU: Added Value of Electrical Cardiometry in Real-Time Tailoring of Management and Therapeutic Strategies

Objective Electrical cardiometry is an impedance-based monitoring technique that provides data on several hemodynamic parameters in a noninvasive way. There is limited information on clinical utility of the application of this technique in neonates. Study Design In this study, we describe the case of a preterm neonate born at 302/7 weeks of gestational age who developed severe systemic infection with fluid refractory septic shock on day 2 of life. Discussion Electrical cardiometry was used and proved very helpful in real-time guiding the choice and the dosing of the most appropriate inotrope drugs in this patient. In addition, it promptly underlined an abrupt drop of systemic vascular resistances occurring after administration of the first dose of antibiotic, thus warning the attending neonatologist to institute appropriate treatment before the clinical conditions could further worsen. Conclusion This case report suggests that electrical cardiometry could be a useful tool in assessing, monitoring, and guiding care of neonates who develop severe septic shock. We suggest that electrical cardiometry is a promising approach in the management strategies of such patients that warrants informative clinical trials. Key Points

Electrical Cardiometry and Cardiac Biomarkers in 24-h and 48-h Ultramarathoners

Our study aimed to (i) utilize novel electrical cardiometry and observe acute changes in cardiac biomarkers among 24-h and 48-h ultra-marathoners, and (ii) examine whether alterations in cardiac responses were associated with the average running speed of these participants. Twenty-four 24-h and sixteen 48-h ultra-marathoners were recruited. Electrical cardiometry in the 2 groups showed significant post-race drops in systolic pressure (24-h: p=0.001; 48-h: p=0.016) and rapid increases in heart rate (24-h, p=0.004; 48-h, p=0.001). Cardiac output increased in 48-h runners (p=0.012) and stroke volume decreased in 24-h runners (p=0.009) at post-test. Six of 20 (30%) 24-h and 4 of 16 (25%) 48-h runners had high-sensitivity troponin T values above the reference interval after the races. N-terminal proB-type natriuretic peptide levels showed a 15-fold increase in 24-h runners and a 10-fold increase in 48-h runners at post-race. There was a positive correlation between delta N-terminal proB-type natriuretic peptide and running mileage (rs=0.629, p=0.003) in 24-h ultra-marathoners. In conclusion, stroke volume and cardiac output showed inconsistent changes between the 2 groups. Average running speed has a significant effect on post-exercise elevation in cardiac biomarkers.

Non-invasive electrical cardiometry cardiac output monitoring during prehospital helicopter emergency medical care: a feasibility study

Purpose Introducing advanced hemodynamic monitoring might be beneficial during Helicopter Emergency Medical Service (HEMS) care. However, it should not increase the on-scene-time, it should be easy to use and should be non-invasive. The goal of this study was to investigate the feasibility of non-invasive cardiac output measurements by electrical cardiometry (EC) and the quality of the EC signal during pre-hospital care provided by our HEMS. Methods A convenience sample of fifty patients who required HEMS assistance were included in this study. Problems with respect to connecting the patient, entering patient characteristics and measuring were inventoried. Quality of EC signal of the measurements was assessed during prehospital helicopter care. We recorded the number of measurements with a signal quality indicator (SQI) ≥ 80 and the number of patients having at least 1 measurement with a SQI ≥ 80. Furthermore, the SQI value distribution of the measurements within each patient was analysed. Results In the experience of the attending HEMS caregivers application of the device was easy and did not result in increased duration of on-scene time. Patch adhesion was reported as a concern due to clammy skin in 22% of all cases. 684 measurements were recorded during HEMS care. In 47 (94%) patients at least 1 measurement with an SQI ≥ 80 was registered. Of all recorded measurements 5.8% had an SQI < 40, 11.4% had an SQI 40–59, 14.9% had a SQI between 60 and 79 and 67.8% had SQI ≥ 80. Conclusion Cardiac output measurements are feasible during prehospital HEMS care with good quality of the EC signal. Monitoring was easy to use and quick to install. In our view it is an promising candidate for the prehospital setting. Further research is needed to determine its clinical value during clinical decision making.