Perioperative Care of Pediatric Patients with Congenital Heart Disease Undergoing Non-Cardiac Procedures: A Survival Guide
Susan Nicolson MD, Viviane Nasr MD, James DiNardo MD, Lindsey Loveland Baptist MD
Today’s PAAD, appropriately published on Valentine’s Day, is a MUST read for everyone who practices pediatric anesthesia, regardless of your place and type of practice or how long you’ve been in practice. Indeed, it is essential reading and quite frankly I don’t think our PAAD review, as good as it is, does this article justice. It is packed with information on physiology, pathophysiology, and specific anesthetic management issues that are essential in taking care of patients with congenital heart disease presenting for non-cardiac surgery. I would urge all of you to read the article in full. Further, I would strongly suggest that it be seriously considered for a journal club or a grand rounds at your home institution, whether you practice in a children’s hospital, an academic or private practice, free standing outpatient facility or even an office based practice.
As more and more children with congenital heart disease survive, many will show up at your door step for non-cardiac surgery. Can you do it, should you do if? Are you even allowed/credentialled to do it? Do you feel comfortable doing it? Is your facility capable of caring for the patient post operatively? Will you get support if you refuse to it? Is our pediatric fellowship training producing pediatric anesthesiologists who can provide care to these children? Please send me your thoughts and how you individually and institutionally handle these questions and I will post in a reader response.
Finally, today’s PAAD was written by our heart group, Drs. Susan Nicolson, Viviane Nasr, James DiNardo, Lindsey Loveland Baptist who submit and review one or two cardiac PAAD/month. Today’s article was co-authored by 2 of or regular reviewers, Drs. Viviane Nasr and Jim DiNardo of the Boston Children’s Hospital. Viviane chaired the committee and Jim was a member of the group who drafted the Scientific Statement for the American Heart Association. Susan Nicolson wrote an invited commentary on the publication which we have modified for the PAAD audience.
Some of the discussion points in the article will be part of the program at the upcoming SPA meeting in Austin, specifically “pulmonary hypertension management pearls” (Dr. Natali a Diaz-Rodriguez Friday 4:10-4:30 PM). In on-going discussions with the PAAD executive council we are considering on adding podcasts for our readers. This article will almost certainly be our maiden voyage and I will keep you posted when it is completed. Finally, if you haven’t registered for the upcoming SPA meeting I’d urge you to do it today! Myron Yaster MD
Original article
Nasr VG, Markham LW, Clay M et al. on behalf of the AHA Council on Lifelong Congenital Heart Disease and Heart Health in the Young and Council on Cardiovascular Radiology and Intervention. Perioperative Considerations for Pediatric Patients with Congenital Heart Disease Presenting for Noncardiac Procedure: A Scientific Statement From the American Heart Association. epub 21 january 2023 Circulation: Cardiovascular Quality and Outcomes PMID: 36519439
Technical advances coupled with innovations have led to multi-disciplinary improvements in the treatment of congenital heart disease (CHD). Now more than 90% of children born with CHD are surviving to adulthood. Improved survival superimposed on an increased incidence of indications for non-cardiac procedures results in more pediatric patients with CHD presenting for non-cardiac surgery, diagnostic and interventional procedures. CHD with its attendant co-morbidities comprises an infinite number of combinations of structural abnormalities, hemodynamic consequences, and severity whose pathophysiology interacts with the perturbations imposed by the non-cardiac intervention(s) and the sedation or anesthesia needed to accomplish the same. Recognizing that no guidelines existed for comprehensive care of this growing group of patients the American Heart Association formed a working group chaired by Nasr and Markham to formulate a Scientific Statement. The Scientific Statement synthesizes the existing data attempting to begin to fill the void.
Studies of cardiac risk factors (cardiac function, residual lesion burden, pre-operative inotropic support, mechanical ventilation and/or ICU admission) and outcomes have identified subsets of patients with minor, major or severe CHD (see table below for criteria). Other determinants of risk include: age, non-cardiac co-morbidities, type of non-cardiac procedure (tissue injury, mechanical deformation of organs, blood loss, fluid shifts, abdominal insufflation), urgency of procedure, race, and socio-economic status. This has led to an attempt to develop risk stratification scores specific to this population. Risk stratification, from low to high risk, predicts potential for peri-operative complications. Children with minor risk have similar outcomes to those without CHD. Patients with major or severe risk have increasing likelihood for adverse outcomes.
The authors review contemporary data on perioperative morbidity and mortality for non-cardiac procedures. Patients with CHD are at higher risk for morbidity (cardiac arrest, neurological and thrombotic complications, renal failure, infection/sepsis, re-intubation, re-operation, unplanned or re-admission) and mortality during and after non-cardiac procedures compared to children without CHD undergoing the same procedures. Contributing factors are: major or severe CHD, age less than 1 year, non-cardiac co-morbidities, and complex or emergency procedures. More than half of the cardiac arrests in this population occur in the non-cardiac OR, compared with 26% in the cardiac OR and 17% in the Cath Lab. The mortality rate for these children undergoing non-cardiac procedures is 2-10 times higher than in children without CHD undergoing the same procedures.
Perioperative care needs to be individualized for each unique patient and undertaken by a multi-disciplinary team familiar with managing children with CHD, in a venue that can handle any eventuality. To date there is no randomized control evidence that justifies all or which of these children should be cared for by a pediatric cardiac anesthesiologist. Children are more likely than adults to undergo non-cardiac surgery at a CHD center. However, children living further away from CHD centers are more likely to undergo non-cardiac procedures at local centers. In fact, their parents prefer it even though the risks are higher. Although the proceduralist at non-CHD centers may possess the expertise to perform the procedure, anesthesiologists and other consultants experienced in the care of these patients may not be available or feel comfortable providing care. If it is decided that a procedure be performed in a non-specialized center, preoperative consultation with a pediatric cardiac anesthesiologist and other consultants may be warranted.
Care begins with a thorough pre-operative assessment. Formulation of a comprehensive plan requires understanding of the native cardiac anatomy, the current physiology, the indications for the proposed non-cardiac intervention and its projected impact on the cardiac physiology. In addition, review of prior procedures and their anesthetics, all pertinent medications, imaging, and laboratory results are necessary to assess risk, determine if additional testing and/or cardiac interventions are needed before embarking on the procedure proposed. Postoperative recovery (outpatient vs inpatient) and the need for invasive monitoring depends on the severity of the congenital cardiac lesion, the non-cardiac procedure, co-morbidities and how the child tolerates the procedure.
Nasr and her colleagues review relevant aspects of several high-risk patients with CHD and discus the pathophysiologies that influence peri-operative management, including single ventricle and pulmonary hypertension. It is beyond the scope of the PAAD to review and summarize this and we would urge you to read the article in its entirely.
The Scientific Statement, a comprehensive review of the data currently available, is well written and well referenced and will serve a resource for practitioners caring for these children. A succinct road map of the integration of patient and procedural elements is found in Figure 3 (included below). It enables risk stratification that can be used to formulate a comprehensive peri-operative plan for the practitioner caring for the occasional child with CHD presenting for a non-cardiac procedure. Multiple branch points of the road map include consultation with a pediatric cardiac anesthesiologist – what does that look like in your practice? Can the ability to consult with pediatric cardiac anesthesiologists be built on a national collaborative scale? Should it? How are patients stratified and who cares for patients with congenital cardiac disease for non-cardiac procedures at your institution? Send your responses to Myron who will post them in future reader response.
This statement alerts pediatric practitioners to the high-risk nature of this growing heterogenous group of children. Meticulous attention to detail and formulation of a perioperative plan specific for each patient optimizes their chances to undergo successful non-cardiac procedures. A similar focus to what has been applied to the care of these children for their cardiac procedures needs to be adopted for their non-cardiac procedural care. Significant challenges currently preclude developing evidence-based care paradigms. The heterogeneity of the myriad of combinations of congenital heart lesions, co-morbidities, and non-cardiac procedures results in insufficient numbers of specific patient types at a single center for appropriately designed studies. Comprehensive multicenter databases with granular data are needed to have adequate numbers of specific subsets of patients to answer the clinical questions and determine best practices including optimal care providers and location of care. With the transformation toward standardization and inter-institutional collaboration, advancements are expected. Reiterative revisions of the AHA Scientific Statement as more data becomes available will continue to improve outcomes, including the quality of life, of the survivors of CHD.
