Anesthesia for adults with congenital heart disease undergoing noncardiac surgery

INTRODUCTION — Congenital heart disease is present in approximately 6 to 19 of 1000 live births [1-3]. In the United States, approximately 1,400,000 pediatric and adult patients have congenital heart disease, with a growing number surviving into middle age and beyond [4-7]. As survival and median age of patients being seen in centers across the country continues to increase, the prevalence of patients presenting with more complex cardiac lesions and associated comorbidities has also increased [8,9]. Many of these patients require anesthetic care for either cardiac or noncardiac surgery. Not surprisingly, risk of perioperative complications such as renal failure, respiratory failure, thromboembolic events, and mortality is increased in patients with adult congenital heart disease (ACHD) [10,11].

This topic will discuss the anesthetic management during noncardiac surgery for patients with ACHD. Preanesthetic assessment of these patients and perioperative hemodynamic goals for specific congenital heart lesions are discussed in a separate topic. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery".)

Anesthetic management during labor and delivery for women with ACHD and other high-risk heart disease is discussed separately. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations" and "Anesthesia for labor and delivery in high-risk heart disease: Specific lesions".)

PREINDUCTION CONSIDERATIONS

Premedication — Many patients with ACHD have had multiple prior surgical procedures. In those with significant preoperative anxiety, premedication may be helpful. As for adults without ACHD, we typically administer intravenous (IV) midazolam 1 to 2 mg. Distress in patients with cognitive impairment may be attenuated by allowing a caregiver to accompany the patient up until the time that induction of anesthesia commences.

Precautions to avoid air embolism — Since many ACHD patients have intracardiac and/or extracardiac shunts, IV lines must be carefully prepared and managed to avoid the possibility of paradoxical systemic air embolism that may cause ischemic stroke [12].

Risk of paradoxical systemic air embolism from IV lines is highest for patients with cyanosis due to right-to-left shunts [13]. Patients with unrepaired intracardiac cyanotic lesions and those with only partial repair or palliation (eg, Blalock-Thomas-Taussig-shunt [BT] shunt) are at the greatest risk. Those with predominant left-to-right shunting such as an atrial septal defect (ASD) are at lower risk, although transient right-to-left shunting can occur even if the ASD is small with normal intracardiac pressures) (see "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Intracardiac and extracardiac shunts'). Other ACHD lesions associated with risk of right-to-left shunting include Fontan patients with venovenous collaterals (which are ubiquitous in adult Fontan patients), and those who have undergone atrial switch operations (due to a high prevalence of baffle leaks) [14]. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

Risk of accidental air embolism in the perioperative setting can be effectively eliminated by meticulously flushing air bubbles from IV catheters prior to connection, using air-bubble filters for all IV lines, and de-airing syringes and ports. During administration of any IV medication, introduction of new bubbles is avoided by first attaching the syringe containing the medication to the IV line, then aspirating fluid from the line into that syringe to remove any bubbles in its hub or the attachment port. Only then is the new medication administered.

Endocarditis prophylaxis — Many patients with ACHD require prophylaxis for endocarditis during selected high-risk procedures. The specific cardiac lesions and procedures for which antibiotic prophylaxis is indicated are discussed separately (see "Prevention of endocarditis: Antibiotic prophylaxis and other measures"). For these indications, an IV antibiotic is administered as a single dose 30 to 60 minutes prior to the procedure (table 1).

Arrhythmia risk management — Arrhythmias are common in patients with ACHD due to abnormal cardiac structure and function, congenital anomalies of the conduction system, tissue injury, or sequelae of previous surgical interventions; these are a leading cause of morbidity and mortality [15]. Patients with single ventricle physiology, ventricular dysfunction, or severe obstructive lesions are particularly intolerant of arrhythmias. Perioperative precautions include placement of cardioversion/defibrillation pads prior to induction of anesthesia (figure 1).

Many patients with ACHD have a previously placed pacemaker or implanted cardioverter-defibrillator [15]. Perioperative management of these devices is discussed in detail elsewhere. (See "Perioperative management of patients with a pacemaker or implantable cardioverter-defibrillator".)

MONITORING

●Invasive cardiovascular monitors – Invasive cardiovascular monitors (eg, intra-arterial and central venous catheter) are used in selected patients with high-risk ACHD if a major surgical procedure is planned (see "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Anatomic and physiologic classification'). It is usually practical and/or necessary to obtain intra-arterial and/or central venous access before surgical positioning, prepping, and draping.

•Intra-arterial catheter – We typically insert an intra-arterial catheter for continuous blood pressure (BP) monitoring before anesthetic induction.

•Central venous catheter (CVC) – The CVC provides large-bore vascular access and central venous pressure (CVP) monitoring for patients with high-risk ACHD lesions and those with limited or difficult vascular access due to prior invasive monitoring or use of major blood vessels for palliative procedures. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Risk assessment and consultation' and "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Prior cardiac interventions and surgical procedures'.)

We use ultrasound guidance during insertion of a CVC in an internal jugular vein location or any site where the patient had prior vascular instrumentation or venous thrombosis. (See "Basic principles of ultrasound-guided venous access".)

After placement, patients with certain ACHD lesions have increased risk of thrombosis in an indwelling CVC catheter (eg, cavopulmonary connection) or for severe thromboembolic complications if thrombus forms on the catheter (eg, patients with right-to-left shunting) [16,17]. Thus, catheters are removed as soon as possible in the postoperative period.

•Pulmonary artery catheter (PAC) – Insertion of a PAC is rarely indicated. (See "Pulmonary artery catheterization: Indications, contraindications, and complications in adults", section on 'Indications'.)

●Transesophageal echocardiography (TEE) – TEE is often employed during major surgical procedures in patients with ACHD complicated by coexisting severe ventricular dysfunction (eg, heart failure in a patient with Fontan physiology), particularly for those undergoing a major surgical procedure [18].

Patients with Fontan physiology with advanced liver disease (FALD) may have esophageal varices, which may increase the bleeding risk associated with TEE probe placement. Fortunately, limited observational data in patients without Fontan physiology suggest that the risk of bleeding associated with TEE in patients with esophageal varices is low [19]. (See "Intraoperative transesophageal echocardiography for noncardiac surgery" and "Management of complications in patients with Fontan circulation", section on 'Liver disease'.)

●Other monitors

•Considerations for capnography – In patients with intracardiac or extracardiac shunts or a low cardiac output (CO), end-tidal carbon dioxide (ETCO₂) monitored with capnography may be significantly lower than the actual partial pressure of carbon dioxide in arterial blood (PaCO₂). This occurs due to the reduced amount of blood passing through the lungs for oxygen and carbon dioxide (CO₂) exchange [20,21]. In such patients, other available methods for ensuring adequacy of ventilation are closely monitored (eg, tidal volume readings, visualization of chest rise with each breath, and blood samples obtained from the intra-arterial catheter for PaCO₂ measurements).

•Cerebral oximetry – We use near-infrared spectroscopy (NIRS) in most patients with severe ventricular dysfunction and/or cyanosis to monitor regional cerebral oxygen saturation (rSO₂) during major surgical procedures with potential for large fluid shifts. This monitor provides an indirect assessment of the adequacy of CO or the possible need for red blood cell transfusion [22-24].

ANESTHETIC AGENTS AND TECHNIQUES

General anesthesia

Choice of induction agents — Most adult patients prefer intravenous (IV) induction of general anesthesia. Selection of induction agents and techniques is based on the expected hemodynamic effects of each anesthetic agent, the presence of ventricular dysfunction, and the hemodynamic goals for the patient's specific congenital cardiac lesion (table 2). (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Perioperative considerations for specific lesions'.)

●Preserved biventricular function – For most patients with preserved biventricular function and no significant shunt, we select a low to normal dose of propofol 0.5 to 2 mg/kg to induce general anesthesia (see "General anesthesia: Intravenous induction agents", section on 'Propofol'). The selected induction agent is typically administered in combination with small doses of fentanyl 0.5 to 2 mcg/kg or midazolam 0.5 to 1 mg as tolerated. (See "General anesthesia: Intravenous induction agents", section on 'Adjuvant agents'.)

However, we select a very low dose of propofol or a different anesthetic induction agent (eg, ketamine (see "General anesthesia: Intravenous induction agents", section on 'Ketamine'), or etomidate (see "General anesthesia: Intravenous induction agents", section on 'Etomidate')) for patients with the ACHD conditions that are noted below.

●Right-to-left shunt and cyanosis – Systemic vascular resistance (SVR) should be maintained or increased. Thus, we prefer ketamine 1 to 2 mg/kg because blood pressure (BP), heart rate (HR), and cardiac output (CO) will typically increase after administration of ketamine if the patient has an intact autonomic nervous system [25-27], while pulmonary vascular resistance (PVR) will be minimally affected if adequate oxygenation and normocarbia are maintained. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Right-to-left shunt with cyanosis' and "General anesthesia: Intravenous induction agents", section on 'Ketamine'.)

●Left-to-right shunt – SVR should be maintained or reduced. Thus, a reduced dose of propofol (eg, 0.5 to 1 mg/kg) is typically selected. However, if significant ventricular dysfunction is present we prefer ketamine 1 to 2 mg/kg to maintain CO. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Left-to-right shunt with pulmonary overcirculation'.)

●Fontan physiology (single-ventricle palliation) – We prefer ketamine 1 to 2 mg/kg to maintain or increase contractility and SVR, while minimally affecting PVR and HR. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

●Moderate or severe ventricular dysfunction (see "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Presence of ventricular dysfunction'):

•Moderate ventricular dysfunction – We typically administer a reduced propofol induction dose that is slowly titrated in 0.25 to 0.5 mg/kg increments up to approximately 1 mg/kg. We avoid a large bolus of propofol as this may decrease CO due to a combination of dose-dependent effects that include venous and arterial dilation, as well as decreased contractility [28-30]. (See "General anesthesia: Intravenous induction agents", section on 'Disadvantages and adverse effects'.)

•Severe ventricular dysfunction – We prefer either ketamine 0.5 to 2 mg/kg or etomidate 0.2 to 0.3 mg/kg for anesthetic induction in patients with reduced cardiopulmonary reserve because these agents are least likely to decrease contractility, SVR, and CO. (See "General anesthesia: Intravenous induction agents", section on 'Ketamine' and "General anesthesia: Intravenous induction agents", section on 'Etomidate'.)

If postoperative controlled ventilation in an intensive care unit (ICU) will be necessary, we typically employ a high-dose opioid technique to achieve anesthetic induction (eg, IV fentanyl 5 to 10 mcg/kg, incrementally titrated) since weaning and extubation will be delayed. This induction technique results in minimal direct myocardial depression and only a small decrease in arterial blood pressure, while minimizing tachycardia and/or hypertension during laryngoscopy and endotracheal intubation. Fentanyl may be combined with small doses of IV propofol, midazolam, or ketamine, as tolerated. (See "Perioperative uses of intravenous opioids in adults: General considerations", section on 'Induction'.)

●Inability to cooperate – An inhalation anesthetic induction may be employed to avoid severe agitation and increased sympathetic tone in response to the pain of a needle stick. We select sevoflurane to avoid coughing. As soon as the patient becomes unresponsive, IV access is obtained. Subsequently, the concentration of sevoflurane is quickly decreased to minimize myocardial depression and avoid hypotension and/or arrhythmias. For example, patients with trisomy 21 are especially prone to develop bradycardia during inhalational induction with sevoflurane [31,32]. (See "Induction of general anesthesia: Overview", section on 'Inhalation anesthetic induction' and "Inhalation anesthetic agents: Clinical effects and uses", section on 'Sevoflurane'.)

For rare patients who are combative during the preoperative period, intramuscular (IM) ketamine 2 to 4 mg/kg is a reasonable alternative. (See "General anesthesia: Intravenous induction agents", section on 'Ketamine'.)

Choice of maintenance agents — The choice of techniques and agents for maintenance of general anesthesia considers the hemodynamic goals for the patient's specific lesion (table 2), but is based primarily on the requirements for accomplishing the surgical procedure and the planned postoperative disposition (eg, outpatient status, hospital ward admission, ICU admission). (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Perioperative considerations for specific lesions'.)

In most patients with ACHD, we select an inhalational technique for primary maintenance of anesthesia (see "Maintenance of general anesthesia: Overview", section on 'Inhalation anesthetic agents and techniques'). Typically, the inhalation agent is supplemented with adjuvant IV agents (see "Maintenance of general anesthesia: Overview", section on 'Specific intravenous agents'). The goal is to keep the ratio of pulmonary blood flow (Qp) to systemic blood flow (Qs) as close to baseline as possible in patients with a shunt, pulmonary hypertension, or Fontan physiology (table 2). This shunt fraction is not altered at moderate concentrations of any of the potent volatile inhalation agents (ie, concentrations near the minimum alveolar concentration [MAC] value, defined as the MAC at 1 atmosphere preventing movement in 50 percent of patients exposed to a surgical incision) [33,34].

If total IV anesthesia (TIVA) is necessary or preferred for the surgical procedure, a combination of one or more infusions may be used. If propofol is selected, doses are typically reduced to 50 to 150 mcg/kg/minute and carefully titrated to minimize myocardial depression in any patient with ventricular dysfunction [35]. Titrated infusions of opioids or other anesthetic agents may be added and/or used instead of propofol. Typical combinations of anesthetic agents may include fentanyl, remifentanil, ketamine, or dexmedetomidine. (See "Maintenance of general anesthesia: Overview", section on 'Total intravenous anesthesia'.)

If a high-dose opioid technique was selected to induce anesthesia because the patient will require postoperative mechanical ventilation (see 'Choice of induction agents' above), this opioid technique may be continued during the maintenance period (eg, IV fentanyl 5 to 10 mcg/kg, incrementally titrated) (see "Perioperative uses of intravenous opioids in adults: General considerations", section on 'Maintenance'). Typically, supplementation with low doses of an inhalational agent (ie, less than the MAC value) or IV dexmedetomidine 0.2 to 1 mcg/kg/hour is employed as tolerated.

Management of ventilation — For most patients with ACHD requiring mechanical positive pressure ventilation (PPV) during general anesthesia, we maintain lung protective ventilation with a low mean airway pressure with tidal volume 6 to 8 mL/kg ideal body weight (IBW), short inspiratory time (ie, low inspiratory to expiratory [I:E] ratio of 1:3 to 1:4). These settings are similar for all patients who receive intraoperative mechanical ventilation, as described separately (see "Mechanical ventilation during anesthesia in adults", section on 'Lung protective ventilation during anesthesia'). We limit positive end-expiratory pressure (PEEP) to 5 to 8 mmHg. The goals are to prevent atelectasis and improve oxygenation while maintaining a low PVR to promote pulmonary blood flow and low intrathoracic pressure to promote venous return [33,36,37].

For patients with Fontan palliation (cavopulmonary connection) (figure 2), we prefer spontaneous breathing with pressure support rather than PPV. Pressure support (via either a supraglottic airway or an endotracheal tube [ETT]) is important to avoid hypoventilation and atelectasis, which could lead to hypercarbia, hypoxemia, and elevated PVR in a spontaneously breathing patient. If mechanical ventilation is necessary, we avoid or minimize PEEP. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

We maintain partial pressure of carbon dioxide in arterial blood (PaCO₂) in the normal range (approximately 40 mmHg) in most patients. However, in those with moderate to severe pulmonary arterial hypertension (PAH), we maintain PaCO₂ at 30 to 35 mmHg to lower PVR. (See 'Balancing circulation' below and "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Pulmonary arterial hypertension'.)

Emergence and extubation — During emergence, we extubate most ACHD patients awake (ie, conscious), particularly if PAH is present [38-40]. Extubation criteria are similar to those in patients without ACHD. (See "Extubation management in the adult intensive care unit".)

We minimize airway reactivity, coughing, straining, and sympathetic responses (eg, hypertension and tachycardia) to the ETT during extubation. Strategies include:

●Facilitating a calm, awake extubation by administering dexmedetomidine during and after extubation in the operating room (OR) to provide supplementary analgesia with minimal sedation or inhibition of respiratory drive, unless contraindicated due to history of sinus node dysfunction or bradycardia. Dosing is titrated to effect with an infusion of 0.2 to 0.7 mcg/kg/hour.

●Extubation while deep (ie, still fully anesthetized), allowing the patient to emerge from anesthesia without an ETT in place.

After extubation, patients are closely monitored for partial airway obstruction to avoid hypoxemia and/or hypercarbia, with consequent increased PVR.

Neuraxial anesthesia and analgesia — For selected patients undergoing appropriate surgical procedures, either a very slowly titrated epidural or a low-dose combined spinal-epidural (CSE) technique can be safely employed in most low- or moderate-risk ACHD patients with normal ventricular function. A neuraxial technique may be used as a primary anesthetic or as an adjunct during general anesthesia. (See "Epidural and combined spinal-epidural anesthesia: Techniques".)

●Advantages

•Continuous infusions of a local anesthetic and/or an opioid titrated to provide dense intraoperative analgesia minimize catecholamine release. Also, neuraxial anesthesia decreases afterload, which may be beneficial for patients with regurgitant lesions or left-to-right shunt. Furthermore, this technique facilitates spontaneous ventilation, which is preferable to positive pressure ventilation in patients with Fontan physiology (cavopulmonary connection):

-(See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Left-to-right shunt with pulmonary overcirculation'.)

-(See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Regurgitant lesions'.)

-(See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

•Provision of excellent postoperative analgesia

●Disadvantages

•Neuraxial analgesia or anesthesia may cause rapid onset of a sympathectomy with consequent decreases in SVR and systemic BP. Therefore, the technique is avoided or employed with extreme caution in patients with certain ACHD lesions such as severe left-sided obstructive lesions or failing Fontan physiology:

-(See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Obstructive lesions'.)

-(See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

●Precautions - If a neuraxial technique is employed, precautions to avoid hemodynamic collapse are necessary. These include:

•Continuous monitoring of systemic BP with an intra-arterial catheter.

•Fluid administration to maintain optimal intravascular volume.

•Very slow titration of the local anesthetic selected for an epidural, and/or use of very-low-dose CSE (eg, 3 mg isobaric bupivacaine combined with 15 mcg fentanyl or 0.15 mg preservative-free morphine or 50 to 100 mcg hydromorphone, administered into the intrathecal space).

•Prompt treatment of hypotension with fluid and phenylephrine boluses 40 to 100 mcg, followed by a phenylephrine infusion, if necessary. Norepinephrine should also be available in the event that phenylephrine is ineffective (table 3). Epinephrine is a reasonable alternative, particularly in patients with poor ventricular function.

Detailed discussion of these precautions is available in other topics addressing neuraxial analgesia for labor and delivery in patients with high-risk heart disease:

-(See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Neuraxial analgesia for labor'.)

-(See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Neuraxial anesthesia for cesarean delivery'.)

CONSIDERATIONS FOR SPECIFIC LESIONS — Key goals for managing specific ACHD lesions affect selection of anesthetic agents and vasoactive drugs, fluid management, decisions to use neuraxial techniques, and management of ventilatory support (table 2). Details for specific lesions are discussed separately. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Perioperative considerations for specific lesions'.)

OTHER CONSIDERATIONS

Balancing circulation — In patients with shunts, the magnitude and direction of the shunt depends upon the pressure gradient between left- and right-sided pressures (table 2) [41].

●Patients with right-to-left shunts – For patients with right to left shunts, the extent of shunting is maintained or reduced by maintaining or increasing systemic vascular resistance (SVR) with vasopressor use as needed to avoid systemic hypotension, and by maintaining or reducing pulmonary vacular resistance (PVR) by maintaining or increasing oxygenation and ventilation.

●Patients with left-to-right shunts – For patients with left to right shunts, the extent of shunting is maintained or reduced by maintaining or increasing PVR by avoiding hyperoxygenation and hyperventilation, and by maintaining or reducing SVR by minimizing vasopressor use. If a vasopressor is required to treat systemic hypotension, phenylephrine is generally preferred because it increases both SVR and PVR.

Standard vasoactive drugs for bolus dosing are always prepared in advance (eg, phenylephrine and ephedrine). Depending on the hemodynamic goals for the specific cardiac lesion, bolus doses and/or infusions of other inotropic, vasopressor, and vasodilator agents should also be readily available (table 2 and table 3 and table 4). For selected lesions, hypotension may be more effectively treated with vasopressin than with adrenergic agents. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Perioperative considerations for specific lesions'.)

Fluid management — Intraoperative fluid therapy is administered with the goal of maintaining optimal intravascular volume status and stroke volume (SV) to maintain adequate tissue perfusion. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Perioperative considerations for specific lesions'.)

Adequate preload is necessary to maintain adequate SV and cardiac output (CO), particularly in patients with certain ACHD lesions such as obstructive lesions, pulmonary arterial hypertension (PAH), and Fontan physiology (cavopulmonary connection) (table 2).

Decreased preload due to blood loss or vasodilation is particularly detrimental for patients with Fontan physiology because CO is dependent on an adequate driving pressure for blood across the pulmonary bed, maintained by an appropriate central venous pressure (CVP). During major surgery with bleeding or large fluid shifts, these patients may need significant volume resuscitation. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

Conversely, administration of large volumes of crystalloid solution may be detrimental for patients with certain ACHD lesions, particularly those at risk for pulmonary edema due to impaired systemic ventricular function or left-to-right shunting (table 2). For these patients, we limit the amount of crystalloid administered by using some colloid (albumin). (See "Intraoperative fluid management", section on 'Colloid solutions'.)

Notably, patients with frank cyanosis rely on a high hemoglobin level for oxygen delivery. For most cyanotic patients, hemoglobin thresholds for erythrocyte transfusion are 12 to 15 mg/dL (rather than 7 to 8 g/dL). An example is Eisenmenger syndrome with right-to-left shunting and PAH. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Right-to-left shunt with cyanosis' and "Medical management of cyanotic congenital heart disease in adults", section on 'Erythrocytosis and relative anemia'.)

Management of pulmonary hypertension — Intraoperative hemodynamic management of patients with pulmonary hypertension (including those with PAH) is discussed in detail separately. (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure", section on 'Hemodynamic management' and "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Pulmonary arterial hypertension'.)

Treatment of arrhythmias — Treatable causes of new arrhythmias should be identified and corrected. Electrolyte or acid–base disturbances may be present as side effects of chronically administered medications. High doses of anesthetics, especially volatile anesthetics, may induce a junctional rhythm. Increased sympathetic tone due to pain, anxiety, nausea, or hypothermia may result in a supraventricular tachycardia. (See "Arrhythmias during anesthesia", section on 'Potential contributing factors'.)

Any arrhythmia causing hemodynamic instability should be immediately treated. (See "Advanced cardiac life support (ACLS) in adults", section on 'Management of specific arrhythmias'.)

Considerations for laparoscopic surgery — Modern technology for laparoscopic surgery allows maintenance of low carbon dioxide (CO₂) insufflation pressures; thus, laparoscopic surgery is an option in ACHD patients if CO₂ insufflation pressure is kept <10 to 12 mmHg and hypercarbia is avoided [42-45]. (See "Anesthesia for laparoscopic and abdominal robotic surgery in adults".)

During laparoscopic surgery, cardiopulmonary risk is increased if the pneumoperitoneum that is created with CO₂ insufflation results in significantly increased abdominal pressure. The increased pressure will decrease preload and increase PVR due to both hypercarbia caused by CO₂ absorption and atelectasis caused by lung compression. These combined factors decrease CO and also worsen cyanosis in patients with right-to-left shunting. However, if CO₂ insufflation pressure is carefully controlled, laparoscopic surgery is not contraindicated even in patients with high-risk ACHD lesions (eg, right-to-left shunt lesions or Fontan physiology). (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Right-to-left shunt with cyanosis' and "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

EARLY POSTOPERATIVE MANAGEMENT — Postoperative pain and anxiety are treated to minimize sympathetic stimulation, encourage deep breathing to ensure adequate ventilation and oxygenation, and avoid increases in pulmonary vascular resistance (PVR). A neuraxial or regional technique may be appropriate to control pain after some procedures. If necessary, intravenous (IV) patient-controlled analgesia (PCA) is used to treat moderate-to-severe pain. (See 'Neuraxial anesthesia and analgesia' above and "Use of opioids for acute pain in hospitalized patients", section on 'Patient controlled analgesia'.)

Postoperative intensive care with or without a period of controlled ventilation may be necessary in some patients with high-risk ACHD, particularly after major surgery. During postoperative transport to an intensive care unit (ICU), we typically administer dexmedetomidine (loading dose of 0.25 mcg/kg, followed by infusion of 0.2 to 0.5 mcg/kg/hour) to provide sedation and analgesia for either intubated or extubated patients (see 'Emergence and extubation' above). Dexmedetomidine may be continued in the ICU, and in some cases, until the patient is mobile and taking oral pain medications. (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Anatomic and physiologic classification'.)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Congenital heart disease in adults".)

SUMMARY AND RECOMMENDATIONS

●Preanesthetic assessment – Patients with adult congenital heart disease (ACHD) are ideally managed at (or in consultation with) a medical center with expertise in ACHD for noncardiac surgical or interventional procedures. Preanesthetic assessment of and perioperative hemodynamic goals for specific congenital heart lesions are discussed in a separate topic (table 2). (See "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery".)

●Precautions to avoid air embolism – Risk of accidental air embolism can be effectively eliminated by meticulously flushing air bubbles from intravenous (IV) catheters prior to connection, using air-bubble filters for IV lines, and de-airing syringes and connectors. Embolic risk is highest in patients with cyanosis due to right-to-left shunts, although paradoxical air embolism is possible even in patients with small left-to-right shunts because transient right to left shunting can occur. (See 'Precautions to avoid air embolism' above.)

●Monitoring – Invasive cardiovascular monitors (eg, intra-arterial, central venous catheter (CVC), transesophageal echocardiography [TEE]) are used in high-risk patients undergoing a major surgical procedure. End-tidal carbon dioxide (ETCO₂) monitored with capnography may be significantly lower than the actual partial pressure of CO₂ in arterial blood (PaCO₂). (See 'Monitoring' above.)

●General anesthesia

•Selection of induction agents – Choices of induction agents are based on the expected hemodynamic effects of each anesthetic agent, the lesion-specific hemodynamic goals, and the presence of ventricular dysfunction (table 2).

-Patients with preserved biventricular function – We suggest a low to normal dose of propofol 0.5 to 2 mg/kg for most patients with preserved biventricular function without a shunt (Grade 2C). (See 'Choice of induction agents' above.)

Specific indications to select a different agent or to use a lower propofol dose include patients with:

-Right-to-left shunting and cyanosis – We suggest ketamine 1 to 2 mg/kg (Grade 2C). (See 'Choice of induction agents' above and "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Right-to-left shunt with cyanosis'.)

-Left-to-right shunting – We suggest low-dose propofol 0.5 to 1 mg/kg (Grade 2C). However, if ventricular dysfunction is present, we suggest ketamine 1 to 2 mg/kg (Grade 2C). (See 'Choice of induction agents' above and "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Left-to-right shunt with pulmonary overcirculation'.)

-Fontan physiology (single-ventricle palliation) – We suggest ketamine 1 to 2 mg/kg (Grade 2C). (See 'Choice of induction agents' above and "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

-Significant ventricular dysfunction – For mild to moderate ventricular dysfunction, we suggest slow titration of propofol in 0.25 to 0.5 mg/kg increments up to approximately 1 mg/kg, avoiding a large bolus of propofol (Grade 2C). For severe ventricular dysfunction, we suggest either ketamine 0.5 to 2 mg/kg or etomidate 0.2 to 0.3 mg/kg (Grade 2C). (See 'Choice of induction agents' above and "Preanesthetic assessment of adults with congenital heart disease undergoing noncardiac surgery", section on 'Presence of ventricular dysfunction'.)

-Inability to cooperate – We suggest an inhalational anesthetic induction with sevoflurane to avoid severe agitation and increased sympathetic tone in response to the pain of a needle stick (Grade 2C). (See 'Choice of induction agents' above and "Induction of general anesthesia: Overview", section on 'Inhalation anesthetic induction'.)

•Selection of anesthetic maintenance agents – Choice of techniques and agents for maintenance of general anesthesia considers the hemodynamic goals for the patient's specific lesion (table 2), but is based primarily on the requirements for accomplishing the surgical procedure and the planned postoperative disposition (eg, outpatient status versus intensive care unit [ICU] admission). (See 'Choice of maintenance agents' above.)

•Ventilation management – For most patients requiring mechanical positive pressure ventilation (PPV) during anesthesia, low intrathoracic pressure, mean airway pressure, and pulmonary vascular resistance (PVR) are maintained with lung protective ventilation with low tidal volume of 6 to 8 mL/kg and short inspiratory time (see "Mechanical ventilation during anesthesia in adults", section on 'Lung protective ventilation during anesthesia'). We limit positive end-expiratory pressure (PEEP) to 5 to 8 mmHg.

For patients with Fontan physiology, we prefer spontaneous negative pressure breathing with pressure support to avoid hypoventilation and atelectasis, which could lead to hypercarbia, hypoxemia, and elevated PVR, rather than PPV. If mechanical ventilation is necessary in a patient with Fontan physiology, we avoid or minimize PEEP. (See 'Management of ventilation' above.)

•Emergence and extubation – During emergence, we extubate most ACHD patients awake, particularly if pulmonary arterial hypertension (PAH) is present. After extubation, patients are closely monitored for partial airway obstruction to avoid hypoxemia, hypercarbia, and increased PVR. (See 'Emergence and extubation' above.)

●Neuraxial analgesia or anesthesia – Neuraxial techniques may be safely employed in selected ACHD patients if rapid onset of a sympathectomy with consequent decreases in SVR and systemic blood pressure (BP) is avoided. (See 'Neuraxial anesthesia and analgesia' above.)

●Fluid and hemodynamic management – Hemodynamic goals vary with the specific ACHD lesion (table 2) and guide selection of vasoactive drugs, as well as fluid and blood administration. Bolus doses and/or infusions of other inotropic, vasopressor, and vasodilator agents should be readily available (table 3 and table 4). (See 'Fluid management' above and 'Balancing circulation' above.)

●Laparoscopic surgery – During laparoscopic surgical procedures, carbon dioxide (CO₂) insufflation pressure is kept <10 to 12 mmHg and hypercarbia is avoided. (See 'Considerations for laparoscopic surgery' above.)

●Postoperative management – Postoperative pain and anxiety are treated to minimize sympathetic stimulation, encourage adequate ventilation and oxygenation, and avoid increases in PVR. (See 'Early postoperative management' above.)

ACKNOWLEDGMENT — We are saddened by the death of Kelly Machovec, MD, MPH, who passed away in March 2022. UpToDate acknowledges Dr. Machovec's past work as an author for this topic.