Noncardiac Surgery in Those with Adult Congenital Heart Disease

Noncardiac Surgery in Those with Adult Congenital Heart Disease

Dana Irrer

Joseph D. Kay


Advances in surgical and medical treatments for children with congenital heart disease (CHD) have led to a steady increase in the number of adults with congenital heart disease (ACHD).1 The combination of late sequela from the congenital cardiac defects, progressive noncardiac comorbidities (sometimes related to their congenital cardiac defects), and gaps in surveillance cardiac care places many of these patients at higher risk for anesthesia and noncardiac surgery than the general population.2,3,4,5,6,7,8,9,10,11,12,13

Although most noncardiac procedures can be performed without significant risk, minor procedures generally considered low risk in the general population may be associated with a higher risk in certain ACHD patients.7 Patients with complex congenital cardiac lesions such as moderate to severe pulmonary hypertension or Eisenmenger syndrome have a high risk for adverse outcomes during noncardiac surgery, with mortality ranging from 4% to 10%.3,14,15 Those with Fontan palliation for single-ventricle anatomy have also been shown to have a higher than normal complication rate, with rates as high as 31%.8 Up to 48% of adverse outcomes may have been modifiable and are attributed to inadequate understanding of CHD physiology.13

In general, patients with CHD are at increased risk for hypotension, respiratory failure, acute kidney disease, pneumonia, and thromboembolic events.16 Surgical risk relates to the complexity of CHD, the type of surgical procedure, urgency of the intervention, and availability of trained specialists and resources. Additionally, procedures in ACHD patients are associated with longer length of stays and higher hospital charges than patients with CHD across a spectrum of noncardiac procedures.7,17 Thus, the ACHD population has unique preoperative imaging recommendations and perioperative monitoring considerations compared to the general population.

Guidelines have been established for perioperative cardiovascular evaluation and management of patients undergoing noncardiac surgery in the general population.18 However, these guidelines have been developed primarily from evidence gathered from adults with acquired heart disease. Although recommendations may generally apply to most patients with CHD, these evaluation and management strategies are insufficient to adequately risk stratify many in this complex and heterogeneous group. Specifically, the guidelines do not account for the variable nonischemic mechanisms for ventricular dysfunction and arrhythmia seen in the ACHD population.19 More recently, the American Heart Association (AHA) released a scientific statement on the diagnosis and management of noncardiac complications in ACHD patients and outlined the preoperative assessment of these patients before noncardiac surgery.17

In addition to the unique management concerns posted by complex congenital heart physiology, ACHD patients often have multi-organ involvement placing them at increased risk for perioperative complications.5,6 These include lung and airway abnormalities, pulmonary hypertension, coagulopathy, neurocognitive deficits, mood disorders, liver and renal disease, immunodeficiency, infection risk, genetic syndromes, and malignancy. This increased incidence of comorbid disease requires additional scrutiny during preoperative assessment and perioperative management.

Cardiac Risk Stratification

Perioperative cardiovascular risk assessment for the general population is outlined in the “2014 ACC/AHA Guideline on Perioperative Cardiovascular Evaluation and Management of Patients Undergoing Noncardiac Surgery.”18 Although the principles outlined in these guidelines are helpful in assessing risks for the ACHD population as well, they fall short of identifying many specific risk factors present in this population based on surgical history. Compared to patients with acquired heart disease, ACHD patients have increased operative risk owing to nonischemic ventricular dysfunction and a higher incidence of pulmonary hypertension, residual intracardiac shunts, arrhythmia, and multi-organ dysfunction.20,21,22 To date, there is not a specific tool in use for cardiac risk assessment with invasive procedures specific to ACHD patients, largely owing to the heterogeneity of the population. Hence, a stepwise, meticulous approach to perioperative cardiac assessment in ACHD was developed to guide workup and management (Algorithm 111.1).17 Factors associated with perioperative mortality and morbidity in this population include cyanosis, congestive heart failure, poor general health, older age, pulmonary hypertension, operations on the respiratory and nervous systems, complex CHD, and urgent or emergent procedures.10


Whenever possible, a comprehensive preoperative assessment by an ACHD expert is recommended for all ACHD patients undergoing noncardiac surgery in order to anticipate and prevent complications. Up to 48% of adverse outcomes in this population may be attributed to lack of adequate knowledge of CHD, previous treatments, and current physiology, and up to 40% of overall adverse events of noncardiac surgeries in ACHD patients are owing to inadequate preoperative assessment.13 Current AHA and American College of Cardiology (ACC) guidelines outline the frequency of routine follow-up and preoperative evaluation based on both the anatomic complexity (A) and physiologic class (P) of the congenital heart lesion. According to expert consensus, even adult patients with “simple” CHD—such as a small or repaired atrial or ventricular septal defect, or mild pulmonic stenosis—should have at least a onetime follow-up with an ACHD cardiologist or possibly every 3 to 5 years or more as determined by the physiologic stage.19

Cardiologists with expertise in ACHD have extensive experience with this heterogeneous population, leading to better anticipation and identification of long-term complications related to underlying disease and interventions. Prior to undergoing any elective surgery, ACHD AP classification should be ascertained and providers should ensure patients are following guidelines regarding ACHD outpatient follow-up and outpatient testing including electrocardiography, echocardiography, functional testing, and cross-sectional imaging. Lapses in adult congenital cardiology care are unfortunately common and should be identified as they are associated with higher morbidity in the ACHD population.23 Working with an adult congenital cardiologist, physiologic stage should be optimized prior to any invasive procedure.3,7,13,24

Location of Care

Ideally, noncardiac surgery risk stratification for ACHD patients should take place well before the planned elective surgical procedure. Those considered at increased risk for cardiovascular complications with surgery should have their elective procedures performed in an ACHD facility with the availability of cardiac and noncardiac subspecialists who are familiar with the management of this patient population. The 2018 ACC/AHA ACHD guidelines outline key personnel and services recommended for ACHD accreditation.19 This includes ACHD board-certified or eligible cardiologists including those trained in electrophysiology, interventional cardiology, heart failure, congenital cardiac imaging, congenital cardiac surgery, cardiac anesthesiologists with CHD expertise, as well as knowledgeable subspecialty care including high-risk obstetrics, pulmonary hypertension, genetics, hepatology, pulmonology, nephrology, infectious disease, neurology, and mental health. Patients with moderate and complex CHD have better outcomes when cared for by an integrated multidisciplinary team. Improved survival has similarly been demonstrated when patients are managed at regional CHD centers.25 This is related to several nuances in ACHD patients best addressed by cardiologists, anesthesiologists, and subspecialists with experience in ACHD care.

In reality, up to 74% of noncardiac surgeries in the ACHD population take place at non-ACHD centers.26 Although this may be appropriate for many of these patients with simple congenital lesions with normal to near-normal functional capacity (ACHD AP classification 1A and 1B), patients should at least have preoperative consultation by an ACHD cardiologist, given the potential for long-term complications. Even simple congenital defects such as small ventricular septal defect can cause progressive changes leading to the development of right or left ventricular outflow tract narrowing, which could significantly increase anesthetic risk. In more complex cardiac lesions, remote practice locations and emergent procedures may preclude transfer prior to noncardiac surgery. In these situations, collaboration with a knowledgeable multi-disciplinary ACHD team should be sought out early. ACHD cardiologists also frequently need to assist in the coordination of care between adult and pediatric trained providers with varied experience in adult congenital cardiology care. If the ACHD patient is managed in a pediatric center, access to adult subspecialty care is essential and may require formalized relationships between centers.27 Similarly, patients cared for in adult centers should be done so in conjunction with an ACHD cardiologist.

Physical Examination

A detailed physical examination is particularly important in this population in gauging operative risk and providing perioperative monitoring. Skin assessment including evidence of prior sternotomy or thoracotomy scar may provide valuable information regarding prior interventions, which may have implications for intraoperative monitoring. As outlined above, right or left thoracotomy scars may indicate surgically sacrificed subclavian arteries in the setting of classic Blalock-Thomas-Taussig shunt or subclavian flap repair of aortic coarctation. Blood pressure and pulse assessment in all four limbs may also aid in this determination and guide which are inappropriate for invasive or noninvasive monitoring or line placement. Additionally, a discrepancy in upper and lower extremity blood pressure or radio-femoral delay in pulse may indicate recurrent stenosis in those with prior aortic coarctation repair. Hence, these patients may be at particularly high risk for decreased renal perfusion during periods of hypotension if the anesthesia team is relying on upper extremity blood pressure alone. All patients should have pulse oximetry testing. Cardiac auscultation may indicate severity of disease as valvar and ventricular dysfunction may decline over time related to underlying disease or prior repair or palliation. This may need to be addressed prior to general anesthesia or surgical intervention. As mentioned elsewhere, patients with CHD often have long-term multisystem complications related to their underlying disease or intervention, and care should be taken to complete a thorough physical examination.

May 8, 2022 | Posted by in CARDIOLOGY | Comments Off on Noncardiac Surgery in Those with Adult Congenital Heart Disease

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