Top Things to Know: Perioperative Considerations for Pediatric Patients With CHD
Published: December 15, 2022
- Continuous advances in pediatric cardiology, surgery, and critical care have significantly improved survival rates for children and adults with congenital heart disease (CHD). Consequently, the resulting increase in longevity has expanded the prevalence of both repaired and unrepaired CHD and has escalated the need for diagnostic and interventional cardiac and noncardiac procedures.
- Patients with CHD are at high risk for mortality, complications, and reoperation after noncardiac procedures. In 2019, the mortality rate in patients with CHD at children’s hospitals was 1.06%, compared with 0.12% in non-CHD patients undergoing the same noncardiac procedures.
- The rigorous study of risk factors and outcomes has identified subsets of patients with minor, major and severe CHD who may have higher-than-baseline risk when undergoing noncardiac procedures and this has led to the development of risk prediction scores specific to this population.
- Using the National Surgical Quality Improvement Program Pediatric, a multivariable analysis identified 8 preoperative predictors that were used to create a 0–10 risk stratification score, which showed very good calibration and discrimination.
- Race and socioeconomic factors also have been found to impact outcomes in CHD patients after noncardiac surgery. A 2021 propensity-matched analysis revealed that Black children with minor and major CHD experienced higher complication and postoperative mortality rates after noncardiac procedures than white children.
- Patient comorbidities and the severity of the cardiac lesion at the time of the noncardiac surgical procedure appear to be the overwhelming, predominant determinants of 30-day mortality. During procedures associated with significant physiological responses prompted by direct surgical tissue injury, the mechanical deformation of organs, blood loss, core temperature variations, and fluid shifts, it is prudent to consider using more invasive monitoring, such as intra-arterial and central venous catheters.
- An important component of preoperative risk stratification is functional status, defined according to cardiac function, residual lesion burden, requirements for inotropic support, the need for preoperative mechanical ventilation, and preoperative intensive care unit admission at the time of surgery. Beyond these factors, the type of cardiac lesion also has been shown to be a significant predictor of risk.
- Although most heart defects occur in isolation, genetic and other extracardiac anomalies (e.g., tracheoesophageal fistulas, anorectal anomalies, genitourinary and musculoskeletal pathologies) are present in about a third of cases, and additional diagnostic and therapeutic procedures may be required to address these noncardiac ailments. Thus, preoperative evaluation is critical for assessing potential risks from anesthesia related to the patient’s clinical symptoms and current health status, the cardiac diagnosis, any altered physiology associated with noncardiac anomalies or illnesses, and the possible implications of the noncardiac procedure.
- Even in patients with a favorable intraoperative course, post-procedure observation is key to ensure early identification or prevention of acute decompensation in the postoperative period. Postoperative care involves many of the same principles as intraoperative management. The level of postoperative recovery (e.g., outpatient ambulatory versus inpatient) and need for invasive monitoring will depend on the type of noncardiac procedure, the severity of the congenital heart lesion with underlying hemodynamics, other coexisting medical conditions, and clinical condition after surgery.
- It is important to differentiate patients with Single Ventricle Physiology (SVP) such as those pre or post Stage 1 single-ventricle palliation, from patients at more advanced stages of single-ventricle palliation, such as those with a superior cavopulmonary anastomosis (SCPA, also known as a bidirectional Glenn shunt), or total cavopulmonary anastomosis (Fontan palliation). Although the risk associated with noncardiac surgery is known to be elevated in patients with single-ventricle palliation, the risks associated with each stage of palliation (SVP, SCPA, Fontan) are less well characterized.
- When possible, noncardiac surgery should be deferred until the cardiac lesion has been treated and the patient’s functional status has been optimized. However, this approach is not always feasible, and many patients require noncardiac procedures before their definitive or palliative intervention or before functional status can be improved. The risk should always be weighed against the benefits of the procedure, in consultation with a multidisciplinary care team.
- While there is no randomized control evidence that justifies the care of these children exclusively by a pediatric cardiac anesthesiologist for every anesthetic procedure, the anesthesia care clinician must have appropriate understanding of the anatomy and physiology coupled with experience to provide safe care. Practitioners, including cardiologists, intensivists, and primary care physicians who care for patients with CHD, must be able to identify the best location for procedures, specify a qualified team (e.g., cardiac anesthesiologists, surgeons), and procure experts in noncardiac subspecialties (e.g., nephrology, hematology, pediatrics). A detailed algorithm is included in the statement.
Citation
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