Anesthesia for adults with congenital heart disease undergoing noncardiac surgery

INTRODUCTION — Congenital heart disease (CHD) 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 CHD, with a growing number surviving into middle age and beyond [4-7]. Many of these patients require anesthetic care for either cardiac or noncardiac surgery. The anesthesiologist should understand the patient's native CHD lesion and prior palliation or repair, current cardiopulmonary reserve, and potential adverse effects of the planned surgical procedure in order to assess risk and develop an anesthetic plan appropriate for lesion-specific hemodynamic goals. As survival and median age of patients being seen in centers across the country continues to increase, this has been accompanied by the increase in prevalence of patients presenting with more complex cardiac lesions and associated comorbidities [8,9]. Not surprisingly, these patients are at an increased risk of perioperative complications such as renal failure, respiratory failure, and thromboembolic events that lead to increased length of stay and mortality [10,11].

This topic will discuss the anesthetic management of adult CHD patients undergoing noncardiac surgery. Anesthetic management during labor and delivery for women with congenital 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".)

PREANESTHETIC ASSESSMENT

General considerations — The history focuses on the native CHD lesion, prior palliative or reparative procedures, current functional status (eg, exercise tolerance and symptoms of heart failure), and cardiovascular and other sequelae associated with CHD [11-13]. The physical examination focuses on signs of cyanosis or heart failure.

Notes from previous surgical procedures are carefully reviewed, as well as cardiac catheterization and imaging studies, in order to delineate the current cardiac anatomy and abnormalities of great vessels or venous return. In addition, careful review of previous cardiovascular complications is important, particularly previous arrhythmias, which are the most common complication in patients with CHD.

High- and moderate-risk lesions — CHD patients with high and moderate risk are ideally managed at a center with expertise in the care of adult patients with CHD, particularly if a major surgical procedure is planned [14]. Such patients may require advanced monitoring and other specialized management during the procedure. (See 'Monitoring' below and 'Anesthetic management' below.)

Risk factors for perioperative morbidity and mortality include [14]:

●High risk

•Cyanotic CHD

•Pulmonary arterial hypertension (PAH)

•Prior Fontan procedure

•Severe systemic ventricular dysfunction (ejection fraction <35 percent)

•Severe left-sided heart obstruction

•History of complex ventricular arrhythmias

•Complex CHD with clinically significant comorbidities (eg, heart failure, significant valve dysfunction, need for anticoagulation)

•Poor overall health

•Urgent/emergent procedures

Patients with the combination of cyanotic CHD associated with PAH (such as Eisenmenger syndrome) are at particularly high risk [14,15]. (See 'Pulmonary arterial hypertension' below.)

●Moderate risk

•Prosthetic valve or conduit

•Intracardiac shunt

•Moderate systemic ventricular dysfunction

•Moderate left-sided heart obstruction

Even after completion of surgical or other interventions that result in optimal repair or palliation of a native CHD lesion, residual sequelae that confer high or moderate risk are often present (eg, ventricular dysfunction, PAH, residual shunting, cardiac valve dysfunction, arrhythmias, and the presence of prosthetic valves or stents) [1,6,13]. Full appreciation of an individual patient's perioperative risk thus requires assessment of the specific anatomy, type of intracardiac repair or palliation, current hemodynamic residua, and current functional status, as well as previous cardiovascular complications. Excellent interdisciplinary collaboration and communication between anesthesiologists and specialists in CHD is necessary for optimal patient care, including discussion of the type and severity of the planned surgical interventions [14,16]. For patients with high- or moderate-risk CHD who present with an indication for urgent or emergency surgery at a center without CHD expertise, communication with a specialized center is important to aid in management and help guide decisions regarding potential transfer.

Prior cardiac surgery or intervention — As a consequence of previous interventions (eg, surgical procedures, cardiac catheterization, peripheral cannulation) or the underlying anatomy (eg, bilateral superior vena cava), many patients with CHD had prior blood vessel injury or ligation (eg, femoral artery or vein, internal jugular vein) that may limit vascular access. Others have chronically occluded or otherwise distorted venous or arterial vessel anatomy.

Precise knowledge of the patient's abnormalities is important for planning invasive hemodynamic monitoring (eg, site of insertion of central venous or peripheral arterial catheters), particularly if emergency vascular access may be necessary (eg, transvenous temporary pacemaker, insertion of cannulas for extracorporeal membrane oxygenation [ECMO]). The notes should be specifically screened in the preoperative period for indications of chronic vessel occlusions or distortion (often noted in cardiac catheterization reports). In cases that are uncertain, assessment by ultrasound, magnetic resonance imaging (MRI), or computed tomography (CT) should be considered.

Sequelae of common surgical procedures in patients with CHD that may affect monitoring or other aspects of anesthetic management include:

●Blalock-Thomas-Taussig shunt – A classical Blalock-Thomas-Taussig shunt (also commonly called the Blalock-Taussig [BT] shunt) is a surgical connection between the subclavian artery and the ipsilateral pulmonary artery (figure 1). Patients with a prior BT shunt may have absent pulses (classic BT shunt) or lower blood pressure (BP; modified BT shunt) on the side of the shunt. A reasonable plan is to measure BP in both upper extremities. If there is discrepancy, measurement of pressures in the extremity without the shunt is generally best since this extremity will presumably have a higher pressure that better represents systemic organ perfusion pressure. For rare patients with bilateral BT shunts, lower extremity BPs are monitored.

●Coarctation repair – There may be residual narrowing of the aorta, resulting in decreased lower extremity BP and/or upper extremity hypertension. Upper extremity BP is always used, but the lower extremity BP is noted since this reflects perfusion pressure of the mesenteric, renal, hepatic, and spinal beds. If there is a marked discrepancy, it is reasonable to measure both upper and lower extremity pressures with either a noninvasive BP (NIBP) cuff or an invasive arterial line if the patient is undergoing major surgery.

In patients who had subclavian flap repair of coarctation (Waldhausen technique), ligation of the left subclavian artery mandates BP monitoring on the right arm.

●Previous ECMO – Patients who previously required ECMO undergo routine ultrasound screening for patency of vascular access prior to a scheduled cardiac or major noncardiac surgical procedure, particularly if emergency peripheral cannulation may become necessary.

Noncardiac conditions — The anesthesiologist also should be aware of common noncardiac sequelae and other comorbidities in adults with CHD [12,13]. Individuals with CHD commonly have conditions in other organ systems such as restrictive lung disease, renal dysfunction, hepatic dysfunction, neurologic sequelae, and hematologic abnormalities including iron deficiency anemia, secondary erythrocytosis, and coagulation abnormalities with tendencies toward bleeding and/or thrombosis [11-13].

●Coagulation abnormalities

•Erythrocytosis – Chronic hypoxemia (ie, cyanosis) leads to secondary erythrocytosis as a compensatory mechanism to maintain arterial oxygen content. Depending on the severity of hypoxemia, hemoglobin levels may be as high as 25 g/dL, and hematocrit is often >60 percent. Thrombocytopenia may also be present [17].

Perioperative management includes the following considerations:

-Symptoms due to hyperviscosity are unlikely if hematocrit is less than 65 percent; if symptoms are present, treatment is intravascular fluid repletion [16]. Dehydration due to prolonged fasting time or perioperative hypovolemia due to fluid shifts worsens hyperviscosity [18]. Hence, fasting time is minimized, IV fluid therapy is initiated in the preoperative period, and adequate intravascular volume status is carefully maintained during surgery. (See 'Fluid management' below.)

-Frequent phlebotomies are discouraged as these inevitably lead to iron deficiency, which may be detrimental, and may result in inadequate hemoglobin levels to maintain tissue oxygenation. However, in patients with preoperative hematocrit >65 percent, the American College of Cardiology/American Heart Association (ACC/AHA) guidelines suggest isovolemic phlebotomy to improve coagulation status [14]. In such cases, dilution to a hematocrit of approximately 45 percent is reasonable [19,20].

-Cyanotic patients are critically dependent on high hemoglobin levels to maintain tissue oxygenation. Thus, conventional transfusion thresholds do not apply to cyanotic patients and should be defined individually depending on cardiac conditions, severity of hypoxemia, and the extent of preoperative secondary erythrocytosis. For most cyanotic patients, hemoglobin thresholds for erythrocyte transfusion are 12 to 15 g/dL. (See "Medical management of cyanotic congenital heart disease in adults", section on 'Erythrocytosis and relative anemia'.)

-In patients with hematocrit >55 to 60 percent, the international normalized ratio (INR) will be falsely elevated if measured with standard citrate tubes; thus, it is important to use corrected citrate tubes in these patients.

•Acquired von Willebrand syndrome – Some CHD patients develop acquired von Willebrand syndrome [21,22]. Decisions regarding whether to use desmopressin (ie, DDAVP) or von Willebrand factor (vWF) concentrates for prophylaxis against bleeding are based on the patient's history of hemostatic challenges and severity of bleeding during and after surgery. (See "Acquired von Willebrand syndrome", section on 'Management'.)

●Airway abnormalities

•Upper airway abnormalities – Upper airway abnormalities (eg, vocal cord paralysis and subglottic stenosis) may be present in patients who had previous surgical procedure(s) complicated by prolonged postoperative tracheal intubation.

Damage to the recurrent laryngeal nerve causing chronic hoarseness or inspiratory stridor may be present after cardiac surgery, particularly after repair of aortic coarctation or the aortic arch. This cause of stridor does not cause airway obstruction but should be differentiated from stridor due to tracheomalacia (see below).

Some genetic abnormalities (Trisomy 21, DiGeorge syndrome, Cornelia de Lange syndrome) are associated with both CHD and abnormalities of the upper airway (eg, large tongue, retrognathia, difficult intubation). Note that some syndromes, including those associated with airway abnormalities may not have been diagnosed in childhood due to phenotypic variations [14].

•Tracheal abnormalities – Tracheomalacia with partial tracheal collapse during expiration may result in chronic episodes of stridor, which may worsen after extubation. Patients at risk include those with a large left atrium or pulmonary artery compressing the trachea (eg, absent pulmonary valve syndrome variant of tetralogy of Fallot), and those with prior repair of a congenital vascular ring that encircled the trachea. Rarely, airway obstruction due to tracheomalacia necessitates reintubation. (See "Tracheomalacia in adults: Clinical features and diagnostic evaluation".)

●Respiratory insufficiency – Chest wall abnormalities (eg, scoliosis) and/or restrictive lung development due to prior thoracotomy procedures may result in respiratory insufficiency [23,24]. Also, many patients with complex CHD have generalized muscle weakness including respiratory muscle weakness [25]. In addition, the phrenic nerve may have been injured during prior procedures such as aortic arch repair, causing chronic respiratory insufficiency due to limited chest expansion. Such neuromuscular and chest wall disease may necessitate invasive or noninvasive perioperative ventilatory support. (See "Noninvasive ventilation in adults with chronic respiratory failure from neuromuscular and chest wall diseases: Patient selection and alternative modes of ventilatory support".)

●Endocrine abnormalities – Adults with CHD have a high incidence of abnormalities in glucose and lipid metabolism, even in the absence of diabetes mellitus [26]. The incidence of diabetes in adults with CHD is similar to the general adult population. (See "Type 2 diabetes mellitus: Prevalence and risk factors".)

●Neurologic and psychiatric abnormalities

•Cognitive impairment – Delayed neurologic development may be present [13].

•Anxiety – Severe anxiety during interactions with medical personnel is common among patients with CHD. Thoughtful and unrushed communication during the preanesthesia consultation, as well as administration of appropriate premedication, are helpful. (See 'Premedication' below.)

•Tolerance to opioids – Tolerance to opioids is possible in CHD patients with significant previous exposure or current methadone use. (See "Management of acute pain in the patient chronically using opioids for non-cancer pain".)

Hemodynamic goals for specific lesions — Hemodynamic goals for specific CHD lesions affect selection of anesthetic agents and vasoactive drugs, fluid management, decisions to use neuraxial techniques, and management of ventilatory support (table 1). These recommendations refer to general principles according to the underlying pathophysiology of individual heart defects, although modifications may be necessary in individual patients. (See 'Anesthetic management' below.)

Right-to-left shunt with cyanosis — Intra- and extracardiac shunts alter the proportion of blood flow going to the systemic versus pulmonary circulations [27]. Physiologic changes occurring in the perioperative period due to anesthetic agents or techniques, surgical stimulation, or blood loss may adversely affect the balance of pulmonary blood flow (Qp) versus systemic blood flow (Qs).

For patients with a right-to-left shunt, hemodynamic goals are to maintain or increase systemic vascular resistance (SVR) while avoiding increases in pulmonary vascular resistance (PVR) (table 1). An example is unrepaired tetralogy of Fallot (infundibular or pulmonic valve stenosis with a ventricular septal defect) (figure 2).

In these patients, deoxygenated blood is shunted to the left, where it mixes with oxygenated blood before circulating through the systemic circulation. Decreased pulmonary blood flow (Qp:Qs <1) results in cyanosis. Chronic cyanosis (ie, chronic hypoxemia) results in secondary erythrocytosis, increased sympathetic tone, and decreased cardiovascular reserve. Also, patients with right-to-left shunting are at high risk of paradoxical systemic embolism by accidental air entry into intravenous (IV) lines or by paradoxical thromboembolic embolism).

Right-to-left shunting and cyanosis are worsened by decreasing SVR (eg, by administering a large bolus doses of propofol or high concentrations of an inhalational anesthetic (see 'Choice of induction agent' below and 'Maintenance' below)), or increasing PVR (eg, due to hypoxemia, hypercarbia, metabolic acidosis, sympathetic stimulation). If a vasoconstrictor is necessary to treat systemic vasodilation, vasopressin is preferred compared with phenylephrine because vasopressin increases SVR without increasing PVR (table 2) [28]. For major surgical procedures, invasive hemodynamic monitoring (eg, intraarterial and/or central venous access) is necessary to maintain optimal balance between PVR and SVR.

Left-to-right shunt with pulmonary overcirculation — For patients with a left-to-right shunt, hemodynamic goals are to maintain or decrease SVR while avoiding significant decreases in PVR (table 1). Examples of these lesions are atrial septal defect (ASD), ventricular septal defect (VSD), and patent ductus arteriosus (PDA).

In these patients, increased pulmonary blood flow (Qp:Qs >1) results in right atrial and right ventricular (RV) volume overload. If chronic, this leads to increased PVR, pulmonary hypertension, and eventual right heart failure. Left-to-right shunts also produce a significant volume burden on the lungs, particularly if Qp:Qs >3:1.

Left-to-right shunting and consequent pulmonary overcirculation are worsened by increasing SVR (eg, by administering a vasopressor) or by decreasing PVR (eg, due to hyperoxia with administration of a high fraction of inspired oxygen [FiO₂], hypocarbia caused by hyperventilation, or metabolic alkalosis). Increased SVR may be treated by administering additional sedative or anesthetic agents (see 'Choice of induction agent' below and 'Maintenance' below), while decreased PVR may be treated by reducing FiO₂ and minute ventilation.

Compared to cyanotic patients with right-to-left shunting, anesthesia in patients with predominant left-to-right shunting is typically better tolerated, particularly if PVR is not elevated and ventricular function is not markedly impaired. Anesthetic management of patients with pulmonary hypertension is discussed separately [29]. (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure".)

Fontan physiology (cavopulmonary palliation) — For patients with Fontan physiology, hemodynamic goals are to maintain preload, decrease PVR, and maintain myocardial contractility in order to maintain forward cardiac output (CO) (table 1) [30,31].

Fontan palliation (figure 3) is the most common palliative operation performed for patients with any type of univentricular physiology (ie, a single ventricle), including hypoplastic left heart syndrome, tricuspid atresia, or double-inlet left ventricle. (See "Management of complications in patients with Fontan circulation" and "Hypoplastic left heart syndrome: Anatomy, clinical features, and diagnosis".)

The resulting Fontan physiology has two components [30,32,33]:

●Presence of a single ventricle that pumps blood to the systemic circulation

●Passive pulmonary blood flow (ie, nonpulsatile circulation to the pulmonary arterial system)

Nonpulsatile pulmonary blood flow results in chronic loss of distal pulmonary vasculature, increased PVR, and chronically elevated central venous pressure (CVP). Adequacy of pulmonary blood flow and CO is dependent on preload and the transpulmonary gradient (TPG), which is the pressure difference between the CVP and the common atrial pressure. Typically, the CVP is maintained at 10 to 15 mmHg for an ideal TPG of 5 to 10 mmHg [30,32,34]. (See 'Fluid management' below.)

Patients with failing Fontan physiology have declining ability to maintain adequate CO despite chronically elevated CVP. This may be due to systolic or diastolic dysfunction of the single ventricle (see 'Presence of ventricular dysfunction' below), atrioventricular valve regurgitation, pulmonary hypertension, and/or arrhythmias [30,35]. These patients have a low CO state and chronically elevated CVP, with resultant liver and lymphatic sequelae (elevated transaminases, cirrhosis, protein losing enteropathy). Eventually, elevated ventricular end-diastolic pressure results in elevated common atrial pressure, necessitating an even higher CVP to drive blood through the pulmonary system to maintain CO. (See "Management of complications in patients with Fontan circulation".)

Adult Fontan patients are more likely to have complications after noncardiac surgery than either patients without heart disease or adults with biventricular congenital heart disease. Baseline oxygen saturation <90 percent is a risk factor for perioperative complications [36]. Fontan patients are particularly sensitive to any decrease in systemic venous return that may occur with positive pressure ventilation or due to insufflation of carbon dioxide for laparoscopic procedures (see 'Laparoscopic surgery' below). Whenever feasible, noncardiac surgery in Fontan patients (even for trivial procedures) should be performed at centers with interdisciplinary experience in the treatment of this high-risk patient group. Ventilation protocols that avoid high positive end-expiratory pressure (PEEP) and early extubation are important measures to decrease perioperative morbidity. (See 'Management of ventilation' below.)

Pulmonary arterial hypertension — For patients with pulmonary arterial hypertension (PAH), hemodynamic goals include decreasing PVR (table 1). Chronically administered medications for PAH should be continued without interruption, particularly chronic parenteral therapies, since any interruption in therapy may precipitate critical decompensation. (See 'Chronically administered medications' below and "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure", section on 'Management of chronic medications'.)

Also, adequate preload is required to maintain CO in patients with RV hypertrophy due to severe PAH since hypovolemia may cause RV underfilling. However, intravascular volume overload may cause RV distention that is often accompanied by worsening tricuspid regurgitation. CVP measurements, if available, are maintained at the patient's baseline, which varies depending on the cardiac lesion. (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure", section on 'Anesthetic management goals'.)

During the intraoperative period, administration of high FiO₂ and hyperventilation to produce mild hypocarbia (ie, partial pressure of carbon dioxide [PaCO₂] 30 to 35 mmHg) may further decrease PVR. Sympathetic stimulation is avoided by ensuring a smooth anesthetic induction and emergence without coughing or straining, and by maintaining excellent analgesia throughout the perioperative period. Hypovolemia is avoided if possible, whether absolute (eg, due to blood loss) or relative (eg, due to vasodilation). Treatment of hypovolemia is best accomplished gradually (eg, with administration of 5-mL/kg increments of isotonic crystalloid to increase intravascular volume). (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure", section on 'Intraoperative management'.)

Perioperative increases in PVR that do not appear to be due to hypoxemia, hypercarbia, or thromboembolism may be treated with inhaled nitric oxide (eg, 20 parts per million [ppm]), if available. (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure", section on 'Chronic targeted therapy for pulmonary hypertension: Patient selection' and "Inhaled nitric oxide in adults: Biology and indications for use".)

The presence of PAH confers increased risk for intraoperative complications (eg, arrhythmias, hypotension, and pulmonary hypertensive crises), as well as perioperative morbidity and death [14,37-46]. Particularly challenging patients include those with Eisenmenger syndrome, in which irreversible severe PAH is due to a significant left-to-right shunt with reversal of shunt direction to right-to-left resulting in chronic cyanosis. Also, pulmonary hypertension that is associated with severe right ventricular dysfunction and severe tricuspid regurgitation confers a high perioperative risk. Further details regarding anesthetic and perioperative management of such patients are available in separate topics. (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure" and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis", section on 'Management of procedures'.)

Obstructive lesions — For patients with an obstructive cardiac lesion (eg, aortic stenosis, mitral stenosis, aortic coarctation, pulmonary valve, or conduit stenosis), hemodynamic goals are to maintain sinus rhythm with a normal heart rate (HR), as well as adequate preload and SVR (table 1).

Obstructive lesions result in hypertrophy of the associated ventricle as a compensatory response, with decreased ventricular compliance, diastolic dysfunction, and decreased stroke volume. Perioperative risk depends on the type and severity of the obstruction and on ventricular function.

Adequate ventricular filling is partly dependent upon adequate preload (see 'Fluid management' below). Maintenance of the atrial contribution to ventricular filling ("atrial kick") is also very important. Sinus rhythm and a normal HR are optimal; a supraventricular tachyarrhythmia (SVT) can result in marked hypotension necessitating immediate cardioversion (see 'Treatment of arrhythmias' below). SVR must be maintained, with vasoactive agents if necessary (see 'Vasoactive drugs' below). Hypotension is avoided, particularly in patients with severe left heart obstruction, because the resulting reduction in coronary perfusion can lead to subendocardial ischemia of the hypertrophied ventricle and ventricular arrhythmias. (See 'Neuraxial anesthesia and analgesia' below.)

Regurgitant lesions — For a patient with a regurgitant valve lesions (mitral, aortic, tricuspid, or pulmonic valve, or conduit regurgitation), hemodynamic goals are to maintain a normal to fast HR (eg, 80 to 100 beats/minute [bpm]) and maintain or decrease SVR, particularly for left heart regurgitant lesions (table 1).

Regurgitant lesions result in ventricular volume overload and eventual ventricular dysfunction. Bradycardia or increases in SVR detrimentally increase regurgitation of a systemic atrioventricular valve, thereby decreasing forward CO. Likewise, a sudden increase in PVR may exacerbate tricuspid regurgitation with subsequent decrease in forward CO.

Presence of ventricular dysfunction — For patients with ventricular dysfunction, hemodynamic goals are to avoid bradycardia and myocardial depression (eg, due to high doses of propofol or high concentrations of a volatile anesthetic agent) (table 1). (See 'Choice of induction agent' below and 'Maintenance' below.)

CHD patients with severe ventricular dysfunction are at risk for heart failure with pulmonary edema, arrhythmias, and low CO syndrome. Since CO is dependent upon both HR and stroke volume, either bradycardia or decreases in myocardial contractility are detrimental.

During major surgery, these patients may benefit from invasive monitoring (eg, intraarterial catheter, central venous catheter [CVC], transesophageal echocardiography [TEE]) (see 'Monitoring' below). If decreased CO results in hypoperfusion, milrinone is often selected because of its inotropic, lusitropic (ie, myocardium-relaxant), and vasodilatory properties in both the systemic and pulmonary circulations (table 2). If hypotension or bradycardia are present, treatment typically includes selection of epinephrine or dopamine as an alternative or additional agent. (See "Inotropic agents in heart failure with reduced ejection fraction".)

Cardiac transplantation — For patients with a transplanted heart, hemodynamic goals are to maintain HR, myocardial contractility, and SVR (table 1).

Transplanted hearts have no sympathetic or parasympathetic innervation; thus, tachycardia and increased contractility do not occur as a reflex response to hypotension and/or hypovolemia [47,48]. For this reason, anesthetic depth is gauged by BP rather than HR. (See "Heart transplantation in adults: Arrhythmias", section on 'Sinus rate'.)

Direct-acting agents such as phenylephrine, vasopressin, epinephrine, or norepinephrine should be used to treat hypotension due to decreased SVR or poor contractility, and for treatment of bradycardia (table 2). Indirect-acting sympathomimetic drugs (eg, ephedrine) and vagolytic drugs (eg, atropine, glycopyrrolate) will not be effective.

Myocardial depressants (eg, high doses of propofol or high concentrations of a volatile anesthetic agent) are avoided. (See 'Choice of induction agent' below and 'Maintenance' below.)

PREANESTHETIC MANAGEMENT

Scheduling — Patients with CHD are scheduled as the first case of the day to minimize fasting time. This timing also ensures the presence of adequate personnel to assist if complications occur during or immediately after surgery and allows extra time in the post-anesthesia care unit (PACU) to determine whether admission to an intensive care unit (ICU) is necessary.

Chronically administered medications — Most chronically administered medications are continued throughout the perioperative period.

●Antithrombotic therapy – CHD patients may be receiving anticoagulant and/or antiplatelet agents. Management of these medications is coordinated with the surgical team, cardiologist, anticoagulation clinic, and/or consultant hematologist [14,49]. (See "Perioperative management of patients receiving anticoagulants".)

●Cardiovascular medications – Cardiovascular medications, including beta blockers, angiotensin-converting enzyme (ACE) inhibitors, and angiotensin II receptor blockers, are continued throughout the perioperative period. Specific details are discussed separately. (See "Perioperative medication management", section on 'Cardiovascular medications' and "Perioperative management of heart failure in patients undergoing noncardiac surgery", section on 'Preoperative management' and "Pulmonary hypertension in adults with congenital heart disease: Disease-specific management", section on 'Overview'.)

Patients receiving chronic ACE inhibitor or angiotensin receptor blocker (ARB) therapy are more likely to develop and require treatment for hypotension during anesthesia, particularly during induction of anesthesia. (See 'Vasoactive drugs' below.)

●Medications to minimize pulmonary vascular resistance (PVR) – All medications that minimize pulmonary vascular resistance (PVR) should be continued up to and during the surgical procedure (especially any continuous intravenous [IV] infusions). Examples are pulmonary vasodilators (eg, epoprostenol, iloprost, sildenafil, tadalafil), endothelin receptor antagonists (eg, bosentan, ambrisentan, macitentan), and calcium channel blockers (eg, amlodipine, diltiazem). (See "Pulmonary hypertension in adults with congenital heart disease: Disease-specific management", section on 'PAH-specific therapy'.)

●Antirejection medications – After cardiac transplantation, immunosuppressive therapy must be continued throughout the perioperative period.

ANESTHETIC MANAGEMENT

Premedication — Premedication is helpful for CHD patients with anxiety after multiple prior surgical procedures. In cooperative adults, we typically administer intravenous (IV) midazolam 1 to 2 mg. Intramuscular ketamine 2 to 4 mg/kg is a reasonable alternative for a combative patient. Patients with cognitive impairment may be at particular distress. This stress may be attenuated by allowing a caregiver to accompany the patient prior to the induction of anesthesia.

Precautions to avoid air embolism — Since many CHD patients have intracardiac and/or extracardiac shunts, IV lines must be carefully prepared to avoid the possibility of paradoxical systemic air embolism and ischemic stroke [50]. Risk of accidental air embolism in the perioperative setting can be effectively eliminated by using air-bubble filters for all IV lines in patients at risk. Also, air bubbles are meticulously flushed from all IV catheters, and air filters are used in all infusion lines. 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.

All patients with right-to-left shunting are at high risk of paradoxical systemic air embolism by accidental air entry into IV lines. Those patients with intracardiac shunts, particularly unrepaired cyanotic lesions, have the highest risk, while those with predominant left-to-right shunting (eg, atrial septal defects) are at lower risk. Other CHD lesions may also confer risk for right-to-left shunting, including Fontan patients with venovenous collaterals (which are ubiquitous in adult Fontan patients), patients who have undergone atrial switch operations (due to a high prevalence of baffle leaks) [51], and those with cyanotic lesions with only partial repair or palliation (eg, Blalock-Thomas-Taussig-shunt [BT] shunt). If right-to-left shunting is suspected (eg, due to oxygen saturation <96 percent at rest or during exercise), then preoperative bubble-contrast echocardiography is typically performed as a simple test to identify a high-risk patient.

Endocarditis prophylaxis — Many patients with CHD conditions require prophylaxis for endocarditis during selected high-risk procedures. The specific cardiac lesions and surgical 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 3).

Arrhythmia risk management — Arrhythmias due to abnormal cardiac structural anatomy, congenital anomalies of the conduction system, tissue injury, or sequelae of previous surgical interventions are common in patients with CHD, and are a leading cause of morbidity and mortality [52]. Patients with single ventricle physiology, ventricular dysfunction, or severe obstructive lesions are particularly intolerant of arrhythmias. Cardioversion/defibrillation pads are positioned prior to induction of anesthesia in these patients.

Many patients with CHD have a previously placed pacemaker or implanted cardioverter-defibrillator [52]. Perioperative management of these devices is discussed in detail elsewhere. (See "Perioperative management of patients with a pacemaker or implantable cardioverter-defibrillator".)

Monitoring

●Standard monitors – Standard American Society of Anesthesiologists (ASA) monitoring is required. This includes continuous pulse oximetry, capnography, electrocardiography, and temperature, as well as intermittent (eg, every three to five minutes) blood pressure (BP) monitoring.

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), because a reduced amount of blood passes through the lungs for oxygen and carbon dioxide (CO₂) exchange [53,54]. Thus, 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 for PaCO₂ measurements if an arterial line is present).

●Invasive monitors – Invasive monitors (eg, intra-arterial or central venous catheters) are employed in selected high-risk patients who are undergoing major surgical procedures (see 'High- and moderate-risk lesions' above). If intra-arterial and/or central venous monitoring is planned, it is usually practical and/or necessary to obtain access before surgical positioning, prepping, and draping.

•Intra-arterial catheter – For most patients with high-risk CHD, we insert an intra-arterial catheter for continuous BP monitoring during surgery. If possible, the intra-arterial catheter is inserted in the awake patient so that systemic arterial pressure may be closely monitored during induction of anesthesia.

•Central venous catheter (CVC) – Patients with high-risk CHD undergoing major surgery, and those with limited or difficult vascular access due to prior invasive monitoring or use of major blood vessels for palliative procedures, are appropriate candidates for a CVC. The CVC provides large-bore vascular access and central venous pressure (CVP) monitoring. We use ultrasound guidance during insertion of a CVC, particularly for the 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".)

Patients with certain CHD lesions have increased risk of thrombosis in an indwelling CVC catheter (eg, cavopulmonary connection), or have an increased risk for severe thromboembolic complications if thrombus forms on the catheter (eg, patients with right-to-left shunting) [55,56]. 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 in adolescent and adult patients with CHD complicated by coexisting severe ventricular dysfunction (eg, heart failure in a patient with Fontan physiology) undergoing major surgery [57]. (See "Intraoperative transesophageal echocardiography for noncardiac surgery".)

●Noninvasive monitors

•Cerebral oximetry – Near-infrared spectroscopy (NIRS) may be used to monitor mixed venous cerebral oxygen saturation, providing an indirect assessment of the adequacy of CO, or the possible need for red blood cell transfusion [58-60]. We employ this monitor in most patients with severe ventricular dysfunction and/or cyanosis, particularly during major surgical procedures with potential for large fluid shifts.

Choice of induction agent — Most adult patients prefer IV induction of general anesthesia.

Selection of induction agents and techniques is based on the expected hemodynamic effects of each anesthetic agent, the hemodynamic goals for the patient's specific CHD lesion, and the presence of ventricular dysfunction (table 1). (See 'Hemodynamic goals for specific lesions' above and "General anesthesia: Intravenous induction agents".)

In patients with good myocardial function, we typically select propofol 0.5 to 2 mg/kg to induce general anesthesia. Patient-specific reasons to select a different agent or to use a very low dose of propofol include the following:

●In patients with right-to-left shunting and cyanosis, systemic vascular resistance (SVR) should be maintained or increased. We prefer ketamine 1 to 2 mg/kg for induction in these patients since BP, heart rate (HR), and CO are typically increased if the patient has an intact autonomic nervous system [61-63] (see "General anesthesia: Intravenous induction agents", section on 'Ketamine'). Pulmonary vascular resistance (PVR) is minimally affected if adequate oxygenation and normocarbia are maintained. (See 'Right-to-left shunt with cyanosis' above.)

●In patients with left-to-right shunting, SVR should be maintained or reduced. We may select low-dose propofol 0.5 to 1 mg/kg; however, if depression of myocardial contractility is a concern, we prefer ketamine 1 to 2 mg/kg. (See 'Left-to-right shunt with pulmonary overcirculation' above and "General anesthesia: Intravenous induction agents", section on 'Propofol' and "General anesthesia: Intravenous induction agents", section on 'Ketamine'.)

●In patients with Fontan physiology (single-ventricle palliation), we prefer ketamine 1 to 2 mg/kg because contractility and SVR are maintained or increased, while PVR and HR are minimally affected. (See 'Fontan physiology (cavopulmonary palliation)' above and "General anesthesia: Intravenous induction agents", section on 'Ketamine'.)

●In patients with mild or moderate ventricular dysfunction, a reduced propofol induction dose may be slowly titrated (eg, 0.25- to 0.5-mg/kg increments up to approximately 1 mg/kg), since a large bolus of propofol may decrease CO due to a combination of dose-dependent effects that includes venous and arterial dilation, as well as decreased contractility [64-66]. (See "General anesthesia: Intravenous induction agents", section on 'Propofol'.)

●In patients with severe ventricular dysfunction and significantly reduced cardiopulmonary reserve, we prefer either ketamine 0.5 to 2 mg/kg or etomidate 0.2 to 0.3 mg/kg for anesthetic induction. (See "General anesthesia: Intravenous induction agents", section on 'Ketamine' and "General anesthesia: Intravenous induction agents", section on 'Etomidate'.)

The 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'.)

●In patients with severe ventricular dysfunction who will require postoperative controlled ventilation in an intensive care unit (ICU), we typically employ a high-dose opioid technique to achieve anesthetic induction (eg, IV fentanyl 5 to 10 mcg/kg, incrementally titrated). This opioid dose may be combined with small doses of IV midazolam administered in 0.5- to 1-mg increments, or ketamine 0.5 to 2 mg/kg, as tolerated. (See 'Presence of ventricular dysfunction' above and "Perioperative uses of intravenous opioids in adults: General considerations".)

●In uncooperative patients with CHD, as with young children, an inhalational anesthetic induction with sevoflurane may be employed to avoid severe agitation, coughing, and increased sympathetic tone in response to the pain of a needle stick. 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. Patients with trisomy 21 are especially prone to develop bradycardia during inhalational induction with sevoflurane [67,68]. (See "Induction of general anesthesia: Overview", section on 'Inhalation anesthetic induction'.)

Maintenance — The choice of techniques and agents for maintenance of general anesthesia is based primarily on the requirements for accomplishing the surgical procedure and the planned postoperative disposition (eg, outpatient status versus ICU admission), as well as the hemodynamic goals for the patient's specific lesion (table 1) [69,70]. (See 'Hemodynamic goals for specific lesions' above.)

In most patients with CHD, we select an inhalational technique for maintenance of anesthesia. In patients with a shunt, pulmonary hypertension, or Fontan physiology, the goal is to keep the ratio of pulmonary blood flow (Qp) to systemic blood flow (Qs) as close to baseline as possible (table 1). 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 minimum alveolar concentration at 1 atmosphere preventing movement in 50 percent of patients exposed to a surgical incision) [30,71].

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 [72]. Titrated infusions of opioids or other anesthetic agents may be added and/or used instead of propofol. Examples include fentanyl, remifentanil, ketamine, or dexmedetomidine. (See "Maintenance of general anesthesia: Overview", section on 'Intravenous anesthetic agents and techniques'.)

If a high-dose opioid technique was selected to induce anesthesia and the patient will require postoperative mechanical ventilation (see 'Choice of induction agent' above), this opioid technique (eg, IV fentanyl 5 to 10 mcg/kg, incrementally titrated) may be continued during the maintenance period. Typically, the opioid is supplemented with low doses of an inhalational agent (ie, less than the MAC value) or dexmedetomidine 0.2 to 1 mcg/kg/hour, as tolerated.

Emergence and extubation — During emergence, we extubate most CHD patients awake (ie, conscious), particularly if pulmonary arterial hypertension (PAH) is present [73-75]. We ensure that oxygenation and ventilation are adequate, thereby avoiding hypoxemia, hypercarbia, and large increases in PVR.

Dexmedetomidine may be administered 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. The goal is to minimize airway reactivity, coughing, and sympathetic responses to the endotracheal tube (eg, hypertension and tachycardia) to facilitate a calm, awake extubation.

An alternative strategy to avoid coughing and straining is extubation while deep (ie, still fully anesthetized), allowing the patient to emerge from anesthesia without an endotracheal tube in place.

Other considerations

Neuraxial anesthesia and analgesia — For 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 CHD patients with normal ventricular function. A neuraxial technique may be used as a primary anesthetic, adjunct during general anesthesia, and/or for postoperative pain management.

An advantage of employing neuraxial approaches is the ability to use continuous infusions of local anesthetic and/or opioid that are titrated to provide dense analgesia, thus minimizing 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 'Hemodynamic goals for specific lesions' above.)

Neuraxial analgesia or anesthesia is avoided or employed with extreme caution in patients with certain CHD lesions (eg, severe left-sided obstructive lesions or failing Fontan physiology (see 'Obstructive lesions' above and 'Fontan physiology (cavopulmonary palliation)' above)). Such patients will not tolerate rapid onset of a sympathectomy and the consequent decrease in SVR and systemic BP. Precautions to avoid hemodynamic collapse in these patients 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 2). Epinephrine is a reasonable alternative, particularly in patients with poor ventricular function.

Further details regarding these precautions are available in topics addressing neuraxial analgesia for labor and anesthesia for patients with high-risk heart disease who are undergoing labor and delivery. (see "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Neuraxial analgesia for labor' and "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Neuraxial anesthesia for cesarean delivery')

Fluid management — Intraoperative fluid therapy is administered with the goal of maintaining adequate tissue perfusion by maintaining optimal intravascular volume status and stroke volume. (See 'Hemodynamic goals for specific lesions' above.)

Adequate preload is necessary to maintain adequate CO in patients with certain CHD lesions, including obstructive lesions, PAH, and Fontan physiology (cavopulmonary connection) (table 1). 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 CVP (see 'Fontan physiology (cavopulmonary palliation)' above). During major surgery with bleeding or large fluid shifts, these patients may need significant volume resuscitation.

Conversely, administration of large volumes of crystalloid solution may be detrimental for patients with certain CHD lesions, particularly those with pulmonary edema due to poor ventricular function or left-to-right shunting (see 'Left-to-right shunt with pulmonary overcirculation' above and 'Presence of ventricular dysfunction' above). For these patients, we limit the amount of crystalloid administered by using some colloid (albumin). (See "Intraoperative fluid management".)

Since patients with frank cyanosis rely on a high hematocrit for oxygen delivery, we maintain hematocrit >30 percent with blood transfusions. An example is Eisenmenger syndrome with right-to-left shunting. (See 'Pulmonary arterial hypertension' above.)

Vasoactive drugs — Standard vasoactive drugs for bolus dosing should be 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 be readily available (table 1 and table 2 and table 4). (See 'Hemodynamic goals for specific lesions' above.)

Management of ventilation — Spontaneous negative pressure breathing with pressure support is preferable to positive pressure mechanical ventilation in patients with certain CHD lesions (eg, Fontan palliation [cavopulmonary connection] (see 'Fontan physiology (cavopulmonary palliation)' above) (figure 3)). Pressure support (via either a supraglottic airway or an endotracheal tube) is important in avoiding hypoventilation and atelectasis, which could lead to hypercarbia, hypoxemia, and elevated PVR in a spontaneously breathing patient.

If mechanical ventilation is necessary, we maintain a low mean airway pressure with tidal volume 6 to 8 mL/kg, short inspiratory time (ie, low inspiratory to expiratory [I:E] ratio of 1:3 to 1:4), and a low positive end-expiratory pressure (PEEP) of 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 [30,32,76].

We maintain PaCO₂ in the normal range (approximately 40 mmHg) in most patients. In patients with moderate to severe PAH, we maintain PaCO₂ at 30 to 35 mmHg in order to lower PVR. (See 'Pulmonary arterial hypertension' above.)

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

Treatable causes of new arrhythmias should be identified and corrected. Electrolyte or acid–base disturbances may be present as side effects of medications. Deep levels of anesthesia, especially volatile anesthetics, may induce a junctional rhythm. Increased sympathetic tone due to pain, anxiety, nausea, or hypothermia may result in supraventricular tachycardia.

Laparoscopic surgery — Modern technology for laparoscopic surgery allows maintenance of low CO₂ insufflation pressures; thus, laparoscopic surgery is an option in CHD patients if CO₂ insufflation pressure is kept <10 to 12 mmHg and hypercarbia is avoided [77-80].

During laparoscopic surgery, 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 in patients with high-risk CHD lesions (eg, right-to-left shunt or Fontan physiology). (See 'Right-to-left shunt with cyanosis' above and 'Fontan physiology (cavopulmonary palliation)' above.)

POSTOPERATIVE MANAGEMENT

●Post-anesthesia care unit (PACU) – Extubation criteria are similar to those in patients without CHD (see "Extubation management in the adult intensive care unit"). After extubation, patients are closely monitored for partial airway obstruction to avoid hypoxemia and/or hypercarbia, with consequent increased pulmonary vascular resistance (PVR).

Postoperative pain and anxiety are treated to minimize sympathetic stimulation, encourage adequate ventilation and oxygenation, and avoid increases in 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 postoperative pain control", section on 'Patient controlled analgesia'.)

●Intensive care unit (ICU) – Postoperative intensive care and a period of controlled ventilation may be necessary in patients with high-risk CHD, particularly after major surgery. (See 'High- and moderate-risk lesions' above.)

During postoperative transport to an ICU, dexmedetomidine may be administered (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 intubated or extubated patients. Dexmedetomidine is typically continued in the ICU, and in some cases, until the patient is mobile and taking oral pain medications.

SUMMARY AND RECOMMENDATIONS

●Preanesthetic assessment

•Congenital heart disease (CHD) patients with high or moderate risk – These patients are referred early for elective surgery to a center with multidisciplinary expertise in their care whenever possible. If urgent or emergency surgery is necessary at a center without CHD expertise, communication with a specialized center aids in management and helps guide decisions regarding potential transfer. (See 'High- and moderate-risk lesions' above.)

•Prior cardiac surgical interventions – Prior palliative or reparative procedures and the presence of noncardiac sequelae may influence anesthetic management. Potential restriction of vascular access due to anatomic variants or previous interventions are carefully assessed in the preoperative period. (See 'Prior cardiac surgery or intervention' above.)

•Noncardiac conditions – Assessment for respiratory insufficiency, airway pathology, or coagulation, neurologic, psychiatric, or endocrine abnormalities are common and may influence anesthetic care. (See 'Noncardiac conditions' above.)

●Preanesthetic management – Scheduling as the first case of the day minimizes fasting time. Most chronically administered medications are continued throughout the perioperative period. (See 'Preanesthetic management' above.)

●Precautions to avoid air embolism – All air bubbles are meticulously flushed from all IV catheters, particularly in patients with intracardiac shunts is avoided. Also, we use air filters in all infusion lines, and avoid introduction of new bubbles during administration of IV medications. (See 'Precautions to avoid air embolism' above.)

●Endocarditis prophylaxis - Some patients with CHD conditions require antibiotic prophylaxis for endocarditis during selected high-risk procedures. If indicated, the antibiotic is administered as a single intravenous (IV) dose 30 to 60 minutes prior to the procedure. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

●Selection of general anesthetic agents and techniques – Choices are based on the expected hemodynamic effects of each anesthetic agent, the lesion-specific hemodynamic goals, and the presence of ventricular dysfunction (table 1). (See 'Choice of induction agent' above and 'Maintenance' above.)

●Neuraxial analgesia or anesthesia – These techniques may be safely employed in selected CHD patients if rapid onset of a sympathectomy is avoided. Either a very slowly titrated epidural or a low-dose combined spinal-epidural (CSE) may be employed, with continuous monitoring of intra-arterial blood pressure (BP), administration of fluids to maintain optimal intravascular volume, and prompt treatment of hypotension with vasoactive agents. (See 'Neuraxial anesthesia and analgesia' above.)

●Hemodynamic management – Hemodynamic goals vary with the specific CHD lesion (table 1) and guide selection of vasoactive drugs (table 2 and table 4), as well as management of fluid and blood administration. Typical goals include maintenance of adequate preload, use of colloid rather than large volumes of crystalloid, and maintenance of hematocrit >30 percent in a cyanotic patient. (See 'Hemodynamic goals for specific lesions' above and 'Fluid management' above and 'Vasoactive drugs' above.)

●Ventilation management – Spontaneous negative pressure breathing with pressure support is preferable to positive pressure mechanical ventilation in patients with certain CHD lesions (eg, Fontan palliation [cavopulmonary connection]). During mechanical ventilation, low intrathoracic pressure, mean airway pressure, and pulmonary vascular resistance (PVR) are maintained with low tidal volume of 6 to 8 mL/kg, short inspiratory time, and low positive end-expiratory pressure (PEEP) of 5 to 8 mmHg. (See 'Management of ventilation' above.)

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

●Emergence and the early postoperative period – During emergence and in the postoperative period, increases in PVR are avoided by ensuring adequate ventilation and oxygenation to avoid hypoxemia and hypercarbia, and by treating postoperative pain and anxiety to minimize sympathetic stimulation. (See 'Emergence and extubation' above and '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.