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Background: Innovation is broadly defined as the act of introducing a new product, idea, or process. The field of surgery is built upon innovation, revolutionizing technology, science, and tools to improve patient care. While most innovative solutions are aimed at problems with a significant patient population, the process can also be used on orphan pathologies without obvious solutions. We present a case of tracheal agenesis, a rare congenital anomaly with an overwhelming mortality and few good treatment options, that benefited from the innovation process and achieved survival with no ventilator dependence at three years of age. Methods: Utilizing the framework of the innovation process akin to the Stanford Biodesign Program, 1) the parameters of the clinical problem were identified, 2) previous solutions and existing technologies were analyzed, newly invented solutions were brainstormed, and value analysis of the possible solutions were carried out using crowd wisdom, and 3) the selected solution was prototyped and tested using 3D modeling, iterative testing on 3D prints of actual-sized patient parts, and eventual implementation in the patient after regulatory clearance. Results: A 3D-printed external bioresorbable splint was chosen as the solution. Our patient underwent airway reconstruction with "trachealization of the esophagus": esophageotracheal fistula resection, esophagotracheoplasty, and placement of a 3D-printed polycaprolactone (PCL) stent for external esophageal airway support at five months of age. Conclusions: The innovation process provided our team with the guidance and imperative steps necessary to develop an innovative device for the successful management of an infant survivor with Floyd Type I tracheal agenesis. Article summary: We present a case of tracheal agenesis, a rare congenital anomaly with an overwhelming mortality and few good treatment options, that benefited from the innovation process and achieved survival with no ventilator dependence at three years of age.The importance of this report is to reveal how the innovation process, which is typically used for problems with significant patient population, can also be used on orphan pathologies without obvious solutions.
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BACKGROUND /IMPORTANCE: The safety of direct cardiac shunts has been historically described in the pediatric population before the introduction of silastic catheters but are rarely utilized in modern practice. Herein, we describe several technical nuances regarding the placement of a direct ventriculoatrial catheter in a pediatric patient, including the creation of a sternal divot to accommodate for the movement of the catheter during growth. CLINICAL PRESENTATION: We report a complex case of a 2-year-old former premature infant with multiple systemic congenital abnormalities, including tracheal atresia (type 2), complete atrioventricular septal defect status post repair, and shunted hydrocephalus. She developed multiple shunt malfunctions secondary to abdominal malabsorption and shunt infections. CONCLUSION: Multiple options for distal shunt placement, including the atrium via open and endovascular techniques, the abdomen, gallbladder, and pleura, were considered, but the direct cardiac placement was felt to be the safest option given the patient's coexisting conditions. Placement requires a multidisciplinary team. Special consideration should be made for linear growth in children.
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Derivaciones del Líquido Cefalorraquídeo , Hidrocefalia , Lactante , Femenino , Humanos , Niño , Preescolar , Derivaciones del Líquido Cefalorraquídeo/métodos , Atrios Cardíacos , Procedimientos Neuroquirúrgicos/efectos adversos , Vesícula Biliar/cirugía , Catéteres/efectos adversos , Hidrocefalia/cirugía , Derivación Ventriculoperitoneal/efectos adversosRESUMEN
INTRODUCTION: Modified ultrafiltration (MUF) is employed at the termination of cardiopulmonary bypass (CPB) in pediatric and neonatal patients undergoing congenital heart surgery to reduce the accumulation of total body water thus increasing the concentration of red blood cells and the other formed elements in the circulation. Modified ultrafiltration has been reported to remove circulating pro-inflammatory mediators that result in systemic inflammatory response syndrome (SIRS) postoperatively. METHODS: Four hundred patients undergoing cardiac surgery requiring cardiopulmonary bypass and weighing less than or equal to 12 kg were retrospectively evaluated for the effectiveness of MUF. After the termination of CPB, blood was withdrawn through the aortic cannula and passed through a hemoconcentrator attached to the blood cardioplegia set and returned to the patient through the venous cannula. The entire CPB circuit volume in addition to the patient's circulating blood volume were concentrated until the hematocrit value displayed on the CDI cuvette within the MUF circuit reached 45% or there was no more volume to safely remove. At the same time a full unit of FFP can be infused as water is being removed, thus maintaining euvolemia. RESULTS: MUF was performed in all 400 patients with no MUF-related complications. Following the conclusion of MUF, anecdotal observations included improved surgical hemostasis, improved hemodynamic parameters, decreased transfusion requirements, and decreased ventilator times. CONCLUSIONS: Complete MUF enables the clinician to safely raise the post-CPB hematocrit to at least 40% while potentially removing mediators that could result in SIRS. In addition a full unit of FFP can be administered while maintaining euvolemia.
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Procedimientos Quirúrgicos Cardíacos , Hemofiltración , Recién Nacido , Niño , Humanos , Ultrafiltración , Estudios Retrospectivos , Puente Cardiopulmonar/efectos adversos , Síndrome de Respuesta Inflamatoria SistémicaRESUMEN
Objective: The objective of this retrospective review was to evaluate whether or not pulsatile flow improves cerebral hemodynamics and clinical outcomes in pediatric congenital cardiac surgery patients. Methods: This retrospective study included 284 pediatric patients undergoing congenital cardiac surgery with cardiopulmonary bypass support utilizing nonpulsatile (n = 152) or pulsatile (n = 132) flow. Intraoperative cerebral gaseous microemboli counts, pulsatility index, and mean blood flow velocity at the right middle cerebral artery were assessed using transcranial Doppler ultrasound. Clinical outcomes were compared between groups. Results: Patient demographics and cardiopulmonary bypass characteristics between groups were similar. Although the pulsatility index during aortic crossclamping was consistently higher in the pulsatile group (P < .05), a significant degree of pulsatility was also observed in the nonpulsatile group. No significant differences in mean cerebral blood flow velocity, regional cerebral oxygen saturation, or gaseous microemboli counts were observed between the perfusion modality groups. Clinical outcomes, including intubation duration, intensive care unit and hospital length of stay, and mortality within 180 days were similar between groups. Conclusions: Although the pulsatility index was greater in the pulsatile group, other measures of intraoperative cerebral perfusion and short-term outcomes were similar to the nonpulsatile group. These findings suggest that while pulsatile perfusion represents a safe modality for cardiopulmonary bypass support, its use may not translate into detectably superior clinical outcomes.
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BACKGROUND: The objective of this randomized clinical trial was to investigate the effects of perfusion modalities on cerebral hemodynamics, vital organ injury, quantified by the Pediatric Logistic Organ Dysfunction-2 (PELOD-2) Score, and clinical outcomes in risk-stratified congenital cardiac surgery patients. METHODS: This randomized clinical trial included 159 consecutive congenital cardiac surgery patients in whom pulsatile (n = 83) or nonpulsatile (n = 76) perfusion was used. Cerebral hemodynamics were assessed using transcranial Doppler ultrasound. Multiple organ injury was quantified using the PELOD-2 score at 24, 48, and 72 hours. Clinical outcomes, including intubation time, intensive care unit length of stay (LOS), hospital LOS, and mortality, were also evaluated. RESULTS: The Pulsatility Index at the middle cerebral artery and in the arterial line during aortic cross-clamping was consistently better maintained in the pulsatile group. Demographics and cardiopulmonary bypass characteristics were similar between the 2 groups. While risk stratification with The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Mortality Categories was similar between the groups, Mortality Categories 1 to 3 demonstrated more patients than Mortality Categories 4 and 5. There were no differences in clinical outcomes between the groups. The PELOD-2 scores showed a progressive improvement from 24 hours to 72 hours, but the results were not statistically different between the groups. CONCLUSIONS: The Pulsatillity Index for the pulsatile group demonstrated a more physiologic pattern compared with the nonpulsatile group. While pulsatile perfusion did not increase plasma-free hemoglobin levels or microemboli delivery, it also did not demonstrate any improvements in clinical outcomes or PELOD-2 scores, suggesting that while pulsatile perfusion is a safe method, it not a "magic bullet" for congenital cardiac operations.
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Puente Cardiopulmonar , Cardiopatías Congénitas , Puente Cardiopulmonar/métodos , Niño , Cardiopatías Congénitas/cirugía , Hemoglobinas , Humanos , Perfusión/métodos , Flujo PulsátilAsunto(s)
Insuficiencia de la Válvula Aórtica , Estenosis de la Válvula Aórtica , Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Válvula Aórtica/anomalías , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Insuficiencia de la Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/congénito , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/cirugía , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Cardiopatías Congénitas/cirugía , HumanosRESUMEN
BACKGROUND AND OBJECTIVES: The hand-off process between pediatric anesthesia and intensive care unit (ICU) teams involves the exchange of patient health information and plays a major role in reducing errors and increasing staff satisfaction. Our objectives were to (1) standardize the hand-off process in children's ICUs, and (2) evaluate the provider satisfaction, efficiency and sustainability of the improved hand-off process. METHODS: Following multidisciplinary discussions, the hand-off process was standardized for transfers of care between anesthesia-ICU teams. A pre-implementation and two post-implementation (6 months, >2 years) staff satisfaction surveys and audits were conducted to evaluate the success, quality and sustainability of the hand-off process. RESULTS: There was no difference in the time spent during the sign out process following standardization-median 5 min for pre-implementation versus 5 and 6 min for post-implementation at six months and >2 years, respectively. There was a significant decrease in the number of missed items (airway/ventilation, venous access, medications, and laboratory values pertinent events) post-implementation compared to pre-implementation (p ≤ 0.001). In the >2 years follow-up survey, 49.2% of providers felt that the hand-off could be improved versus 78.4% in pre-implementation and 54.2% in the six-month survey (p < 0.001). CONCLUSION: A standardized interactive hand-off improves the efficiency and staff satisfaction, with a decreased rate of missed information at the cost of no additional time.
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Anomalous origin of the right coronary artery from the pulmonary artery, a rare congenital cardiac defect, is typically not diagnosed during infancy. On the other hand, Turner syndrome is usually diagnosed early, and it is classically associated with bicuspid aortic valve and aortic coarctation. Individuals with Turner syndrome are also at increased risk for coronary artery anomalies. We present a case of anomalous right coronary artery from the pulmonary artery in a week-old neonate who also had Turner syndrome, patent ductus arteriosus, transverse aortic arch hypoplasia, and impaired ventricular function. Prostaglandin therapy through the ductus increased the patient's myocardial perfusion. Four months after corrective surgery, she was doing well. We discuss the reperfusion phenomenon in our patient's case, as well as other considerations in this combination of congenital defects.