“Never doubt that a small group of thoughtful, committed, citizens can change the world. Indeed, it is the only thing that ever has.”– Margaret Mead
In matters of history, politics, and social justice, it helps to take the long view to see how far we have come. Today's world seems hopelessly violent until one recognizes that we are collectively safer than ever before. Despite what we witness, a look over centuries demonstrates that modern civilization provides us with more safety than any other point in human history.
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Recognizing this fact puts the deluge of sadness and conflict depicted in the 24-hour news cycle in context.Pinker S. A History of violence: Edge Master Class 2011. Available at: https://www.edge.org/conversation/mc2011-history-violence-pinker. Accessed March 31, 2021.
Many anesthesiologists who work with infants and children with congenital heart disease may be similarly affected by our daily practice. Every day we care for children with complex disease, with prolonged recoveries and major morbidity and even mortality. It is easy to get bogged down in the sadness, particularly when we have a period in which a string of poor patient outcomes threatens our collective morale. Although not unexpected, such events take an emotional and mental toll on all of us working with these infants and children, and challenge our workplace resilience. But taking a step back and looking at medicine in a historic context is similarly inspiring, particularly in the case of children born with anatomy that results in a functional single ventricle. It is difficult to overstate the advances of the last several decades in improving both morbidity and mortality in this population. Forty years ago, the mortality for a child born with hypoplastic left heart syndrome was 100%. In 1980, Dr. Norwood described what would become his eponymic surgery—to create unobstructed flow to a reconstructed aorta and controlled flow to the pulmonary arteries.
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Advances followed, and by the late 1990s, the three-stage palliation for hypoplastic left heart syndrome and other single-ventricle physiologies permitted these children to have a chance at survival to a normal life. Advances in perioperative care, infectious disease, nutrition, perfusion, anesthesia, surgery, and generalized critical care have further increased survivability for these infants.Although overall survival continued to increase, case series and observational studies in the early 2000s showed a persistently high mortality rate in infants who had undergone stage I palliation but were awaiting stage II, including those who were well enough to be discharged home. The interstage mortality of this group was as high as 10%-to-20%.
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Addressing this gap in morbidity and mortality benefits became the first initiative of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a group of six congenital heart centers that joined together in 2006 with the intention of promoting collaboration and quality improvement across congenital heart centers.4
The goals of NPC-QIC were threefold—improve interstage growth failure, reduce interstage hospital readmission, and improve mortality. To this end, the NPC-QIC initiated programs focused on the interstage period to both reduce morbidity/morality and to improve infant and family quality of life. Given the lack of evidence to guide this group, collaboration and creativity were paramount. In an effort to reduce mortality in this high-risk patient population, an interstage home monitoring (IHM) program was developed that would enable early identification of decreased oxygen saturation, acute weight loss, or failure to gain weight after stage 1 palliation.
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This IHM strategy has since evolved to include home nutrition bundles and early healthcare team notification of common clinical changes that are considered “red flags.” These innovations have led to improved interstage survival and decreased mortality in several single-center and multicenter studies.6
The initial participants took a leap of faith in agreeing to practice changes with no guarantee of success. They successfully applied quality improvement methodologies to improve care of a specific population. Their collaborative efforts have paid off. In August 2020, the Journal of the American Heart Association published “Interstage Home Monitoring for Infants with Single Ventricle Disease: Education and Management,” a scientific statement published on the home monitoring program begun by the NPC-QIC.
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The statement provides a succinct yet easily digestible overview of single ventricle physiology, as well as the pathophysiologic forces associated with both increased and decreased oxygen saturations for infants palliated with stage I repair.This American Heart Association statement provides important information for any anesthesiologist caring for children with congenital heart disease, and even those caring for adults with single-ventricle physiology who may have benefitted from IHM programs in the past. Three key elements of this statement deserve particular attention. First, the authors described common causes of interstage mortality (eg, mortality that occurs after discharge home after stage I palliation, but before stage II palliation). Mortality related to cardiac causes is higher in those single-ventricle patients with a modified Blalock-Taussig shunt (as opposed to a right ventricular-to-pulmonary artery shunt), though the mechanism of this risk is unclear.
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Infants also succumb to long-term effects of failure to thrive, or gastrointestinal and respiratory illnesses that affect the balance of pulmonary-to-systemic blood flow, create metabolic disturbances, and result in cardiogenic or hypovolemic shock.Second, the authors described the most common “red flags” that should prompt immediate communication with the healthcare team. These red flags, include oxygen saturation ≤75% or an unanticipated increase in oxygen saturation from baseline ≥90% in an infant with Norwood physiology, failure to gain 20 g in three days, weight loss ≥30 g, enteral intake < 100 mL/kg per day, cyanosis or pallor, irritability or fussiness, diarrhea or vomiting, increased sweating, respiratory changes (tachypnea, distress), and a temperature >100.4°F.
Finally, and most impressively, the authors described the complex system necessary to ready these infants for hospital discharge, and then closely follow them to ensure safety at home. Home equipment, including portable pulse oximeters and a digital infant scale, are provided to each family. Caregiver education is of paramount importance and begins early during the inpatient stay to ensure understanding of complex tasks like calculating nutritional intake. Many checklists for both parents and staff are included in this process, including checklists to ensure adequate communication among physicians and other healthcare providers once the baby is discharged home. Nutritional support and neurodevelopment surveillance are included in the program. IHM has reduced mortality by more than 40% for some centers; the highest-performing programs have an interstage mortality of only 2%.
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Identified barriers to effective IHM include psychologic and socioeconomic factors, which can be mitigated with close follow-up and social support. Telehealth platforms improve communication, and clearly defined automatic thresholds for notification of the healthcare team provide well-delineated guidelines for evaluation.Coordination of care and communication were key drivers for success of the interstage mortality program, and for NPC-QIC projects overall. Of note, NPC-QIC now includes 55 congenital heart programs in the United States, a number that has been stable since 2012. Knowledge of this program is helpful to anesthesiologists for two reasons. First, it is important that we know about home monitoring programs and the red flags so that we are able to identify at-risk infants presenting for noncardiac surgery or cases when a cardiologist may not be involved. Interstage patients have high rates (17%) of intervention, most commonly cardiac catheterization with intervention; but these high-risk patients also present commonly for noncardiac procedures.
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Series examining noncardiac surgery in children with congenital heart disease show a high rate (11.7%) of major adverse cardiac events in the perioperative period.10
This emphasizes the tenuous nature of this single-ventricle physiology, and it is not surprising that patients with major and severe congenital heart disease have a higher incidence of mortality.11
Interestingly, a study that incorporated intrinsic surgical risk into its risk stratification methodology did not improve prediction of 30-day mortality, finding that the patient's comorbidities and severity of congenital heart disease (CHD) were the predominant predictors of 30-day mortality.
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Therefore, as the severity of CHD is a primary determinant in mortality, a thorough understanding of the patient's pathophysiology on the day of their procedure or surgery is vitally important to effectively manage these patients. It may be that assessment of nutritional status, growth, deviations from baseline oxygen saturations, and the presence of any other reported red flags during the interstage period might indicate that a patient has poor compensatory status and is more susceptible to anesthetic complications. This information then could lead to potential changes in strategy for anesthetic management.Given the complex and often nuanced cardiopulmonary physiology of infants with complex congenital heart lesions, who is best suited to anesthetize these infants for noncardiac surgery in the interstage period must be considered. This does not have a simple answer and depends on many factors including the nature of the planned procedure and local resources. No studies to date have demonstrated any benefit in adult or pediatric patients who were managed by anesthesiologists with specialized training in cardiac anesthesiology. However, one study has shown an association between anesthesia-related pediatric cardiac arrest and anesthesiologists with lower annual caseload and fewer annual days delivering anesthetics.
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Another study at a European tertiary care university hospital demonstrated lower incidence of perioperative pediatric cardiac arrest after implementation of a specialized pediatric anesthesia team.14
Thus, it seems reasonable that anesthesiologists caring for patients with CHD, and specifically patients during the interstage period, should have a thorough knowledge of complex single-ventricle lesions, repairs, and physiology. Most pediatric anesthesiologists possess a basic fundamental knowledge of congenital heart disease, but patients with single-ventricle physiology specifically during the interstage period represent a unique patient population with many physiologic considerations, and it stands to reason that someone with specialized training in congenital cardiac anesthesia may be best suited to provide this care.To address this conundrum, preoperative risk stratification tools have been used successfully to determine how to assign pediatric anesthesia staff in noncardiac surgery.
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While this may work in regional and tertiary centers with practitioners trained in congenital cardiac anesthesia, many hospitals may not have specially trained staff to manage these patients, particularly in emergent or urgent settings when there is insufficient time to transfer to a larger center.16
Furthermore, even regional centers may lack anesthesia practitioners with formalized training in congenital cardiac anesthesia. As patient outcomes in CHD continue to improve because of better surgical, medical, anesthetic and intensive care, and programs such as IHM, the population of patients living into childhood, adolescence, and adulthood with congenital heart disease also will continue to increase.The success of interstage monitoring programs and this report from the American Heart Association should prompt our own evaluation in anesthesiology regarding care of infants with single-ventricle palliation, as well as all pediatric and adult patients living with complex cardiac disease who present for noncardiac surgery. Nutritional support, neurobehavioral surveillance, family support, and close ongoing monitoring throughout childhood and adolescence may mitigate long-term adverse events or allow early intervention into sequelae that are part of the natural history of the disease. As leaders of the perioperative surgical home, anesthesiologists are well-poised to participate in and improve care coordination in this complex population.
Conflict of Interest
None.
References
Pinker S. A History of violence: Edge Master Class 2011. Available at: https://www.edge.org/conversation/mc2011-history-violence-pinker. Accessed March 31, 2021.
- Current therapy for hypoplastic left heart syndrome and related single ventricle lesions.Circulation. 2016; 134: 1265-1279
- Factors associated with interstage mortality following neonatal single ventricle palliation.World J Pediatric Congenit Hear Surg. 2018; 9: 616-623
- Improvement in interstage survival in a national pediatric cardiology learning network.Circulation Cardiovasc Qual Outcomes. 2018; 8: 428-436
- Home surveillance program prevents interstage mortality after the Norwood procedure.J Thorac Cardiovasc Surg. 2003; 126: 1367-1375
- Power of a learning network in congenital heart disease.World J Pediatric Congenit Hear Surg. 2018; 10: 66-71
- Interstage home monitoring for infants with single ventricle heart disease: Education and management.J Am Heart Assoc. 2020; 9e014548
- Comparison of shunt types in the Norwood procedure for single-ventricle lesions.New Engl J Medicine. 2010; 362: 1980-1992
- Outcomes associated with unplanned interstage cardiac interventions after Norwood palliation.Ann Thorac Surg. 2019; 108: 1423-1429
- Patients with single ventricle physiology undergoing noncardiac surgery are at high risk for adverse events.Pediatr Anesth. 2015; 25: 846-851
- Post-operative outcomes in children with and without congenital heart disease undergoing noncardiac surgery.J Am Coll Cardiol. 2016; 67: 793-801
- Integration of the intrinsic surgical risk with patient comorbidities and severity of congenital cardiac disease does not improve risk stratification in children undergoing noncardiac surgery.Anesth Analg. 2020; 131: 1083-1089
- Anesthesiologist- and system-related risk factors for risk-adjusted pediatric anesthesia-related cardiac arrest.Anesth Analg. 2016; 122: 482-489
- Incidence of peri-operative paediatric cardiac arrest and the influence of a specialised paediatric anaesthesia team.Eur J Anaesth. 2019; 36: 55-63
- Children with heart disease: Risk stratification for non-cardiac surgery.J Clin Anesth. 2016; 35: 479-484
- Hospital distribution and patient travel patterns for congenital cardiac surgery in the United States.Ann Thorac Surg. 2019; 107: 574-581
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Published online: May 21, 2021
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