Perioperative Care of Pediatric Patients with Congenital Heart Disease Undergoing Non-Cardiac Procedures: A Survival Guide

Last Updated: December 15, 2022

Disclosure: None
Pub Date: Thursday, Dec 15, 2022
Author: Susan C. Nicolson, MD
Affiliation: Josephine J. Templeton Chair, Division of Cardiothoracic Anesthesia, The Cardiac Center at The Children's Hospital of Philadelphia, Professor of Anesthesia and Critical Care Medicine, Perelman School of Medicine at The University of Pennsylvania

Technical advances coupled with innovations have led to multi-disciplinary improvements in the treatment of congenital heart disease (HD). Now more than 90% of children born with congenital HD are surviving to adulthood. Improved survival superimposed on an increased incidence of indications for non-cardiac procedures results in more pediatric patients with congenital HD presenting for non-cardiac surgery, diagnostic and interventional procedures. Congenital HD 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. To date no guidelines for comprehensive perioperative care of these children exist. The Scientific Statement by Nasr et al. synthesizes existing data in an attempt to begin to fill the void (ref).

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 congenital HD. 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 congenital HD. 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 congenital HD 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 congenital HD undergoing the same procedures. Contributing factors are: major or severe congenital HD, 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 congenital HD 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 congenital HD, in a venue that can handle any eventuality. To date there is no randomized control evidence that justifies all or which of these children be cared for by a pediatric cardiac anesthesiologist. Children are more likely than adults to undergo non-cardiac surgery at a congenital HD center. However, children living further away from congenital HD centers are more likely to undergo noncardiac procedures at local centers. Although the proceduralist at non-congenital HD centers may possess the expertise to perform the procedure, anesthesiologists and other consultants experienced in the care of these patients may not be available. 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, comorbidities and how the child tolerates the procedure. Nasr and her colleagues review relevant aspects of several high-risk pathophysiologies that influence peri-operative management, including single ventricle and pulmonary hypertension.

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. It enables risk stratification that can be used to formulate a comprehensive peri-operative plan for the practitioner caring for the occasional child with congenital HD presenting for a non-cardiac procedure.

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 congenital HD.


Nasr VG, Markham LW, Clay M, DiNardo JA, Faraoni D, Gottlieb-Sen D, Miller-Hance WC, Pike NA, Rotman C; on behalf of the American Heart Association Council on Lifelong Congenital Heart Disease and Heart Health in the Young and Council on Cardiovascular Radiology and Intervention. Preoperative considerations for pediatric patients with congenital heart disease presenting for noncardiac procedures: a scientific statement from the American Heart Association [published ahead of print December 15, 2022]. Circ Cardiovasc Qual Outcomes. doi: 10.1161/HCQ.0000000000000113


  1. Nasr VG, Markham LW, Clay M, DiNardo JA, Faraoni D, Gottlieb Sen D, Miller-Hance WC, Pike NA, Rotman C. Perioperative Considerations for Pediatric Patients with Congenital Heart Disease Presenting for Noncardiac Procedures: a Scientific Statement from the American Heart Association. Circulation: Cardiovascular Quality and Outcomes. 2022 doi: 10.1161/HCQ.0000000000000113

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