Anesthesia for surgical repair of congenital heart defects in adults: General management

INTRODUCTION — The number of adults living with congenital heart disease (CHD) has increased steadily due to advances in diagnosis and management of these conditions in children [1-3]. Some patients present as adults with CHD requiring surgical correction due to failure to detect a mild lesion in childhood or lack of resources for repair in some geographic regions. Also, adults with known CHD who had surgical procedures during infancy and childhood often require reoperation for management of residual lesions after a previous repair or for further palliation after previous palliative surgery.

This topic reviews general anesthetic management of adult patients presenting for surgical repair of CHD defects [4]. Management for adults undergoing primary surgical repair or reoperations for specific congenital lesions is discussed separately. (See "Anesthesia for surgical repair of congenital heart defects in adults: Management of specific lesions and reoperation".)

Other topics address anesthetic management for noncardiac surgery, or for labor and delivery, in adult patients who have repaired or unrepaired CHD. (See "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery" and "Anesthesia for labor and delivery in high-risk heart disease: Specific lesions".)

PREANESTHESIA CONSULTATION — An important aspect of the preanesthesia consultation is to ensure understanding and appropriate management of clinically relevant cardiac and noncardiac sequelae of the specific congenital heart disease (CHD) lesion [5].

General considerations for preanesthetic consultation before cardiac surgery are described separately. (See "Preoperative evaluation for anesthesia for cardiac surgery".)

Assessment of cardiovascular risk — Multidisciplinary communication before, during, and after surgical repair of CHD in an adult includes discussion among the cardiac surgical, cardiology, critical care, and anesthesiology teams [6,7].

Assessment of congenital lesion — The preanesthesia consultation includes a thorough review of the patient’s diagnosis, complications, cardiac surgical and catheter-based interventional history, current physiology, recent imaging studies (eg, cardiac catheterization, echocardiography, chest computed tomography), and laboratory data (table 1).

Risk stratification of adults with CHD undergoing cardiac surgery depends on several factors including the complexity of the lesion, physiological status, concurrent conditions and risk factors, and the planned surgical procedure (table 1) [8-10]. Mortality risk is determined by patient-specific factors (eg, univentricular physiology, cyanosis, presence of genetic syndrome, PH, arrhythmias, acute infective endocarditis, subaortic atrioventricular valve regurgitation, ventricular dysfunction, ischemic coronary disease, presence of aortic aneurysm), as well as overall CHD complexity and surgical repair options risk [7,11,12].

Few studies have examined risk-scoring systems for adults with CHD undergoing cardiac surgery. The existing risk scores have been validated in the pediatric population but not in adults. Several groups have attempted to derive risk scores to predict in-hospital mortality in adults undergoing congenital heart surgery.

●A US study looked at mortality associated with procedural groups based upon 12,513 procedures performed at 116 centers and found that the highest risk of operative mortality was associated with Fontan revision, followed by heart and lung transplants [13].

●A European study of patients >16 years old undergoing 1782 congenital heart surgery procedures developed a score predictive of in-hospital mortality. Worse outcomes were associated with New York Heart Association (NYHA) functional class III/IV, urgent surgery, more than two sternotomies, active endocarditis, glomerular filtration rate (GFR) <60, extremely high or low hemoglobin values, and high perioperative adult congenital heart surgery (PEACH) score (ie, in-hospital mortality following cardiac surgery in patients with a score >3 was 17.2 percent [14].

●A risk model for death within four years after surgery for adult CHD was developed in a prospective cohort of 602 patients with moderate/complex ACHD treated at a tertiary care center clinic in the Netherlands. The model was validated in a retrospective cohort of 402 ACHD patients treated in the Czech Republic. Variables including age, congenital diagnosis, New York Heart Association (NYHA) class, current medications, body mass index, N-terminal proB-type natriuretic peptide, and need for revision after initial corrective surgery [15].

●A retrospective study of postoperative complications (eg, cardiac, respiratory, or infectious complications, or acute kidney injury) of 16,841 adult CHD surgery admissions cited the following risk factors: not being from a White population, government insurance, high surgical complexity, non-elective admission, chronic kidney or liver disease, and heart failure [16]. Patients with these complications had a longer length of hospital stay (10 versus 5 days) and higher mortality (4.6 versus 0.9 percent; odds ratio [OR] 2.5, 95% CI 1.8-3.4) than patients without such complications.

Assessment of cardiovascular sequelae — The following common cardiovascular sequelae of CHD should be assessed:

●Heart failure (HF) – Left, right, or biventricular HF may be present in patients with long-standing CHD due to volume and/or pressure overload of one or both ventricles. In patients with HF, left and right ventricular ejection fractions may be reduced or preserved. Volume overload typically occurs due to uncorrected or residual shunts or cardiac valve incompetence. Pressure overload typically occurs due to residual outflow tract obstruction. Many patients with CHD have combined volume and pressure overload. Furthermore, myocardial damage may have occurred in patients who had a previous cardiac surgical repair due to prolonged duration of cardiopulmonary bypass (CPB) with poor myocardial preservation, or because of a large incisional scar or previous ventriculotomy [17].

•Right-sided HF – Patients with right-sided HF may have coexisting pulmonary hypertension (PH). The presence of tricuspid regurgitation (TR) is associated with progressive right ventricular (RV) dysfunction; TR may occur due to a congenital tricuspid valve abnormality, or as a result of RV dilation or dysfunction [2].

Anesthetic management goals include maintaining preload in an optimal range while avoiding fluid overload, and maximizing RV oxygen supply (ie, RV perfusion and subendocardial blood flow), while minimizing RV oxygen demand (ie, RV afterload, tachycardia) [18]. Since the failing RV is exquisitely afterload sensitive, a key goal is to maintain pulmonary arterial pressure (PAP) as low as possible to maintain forward flow. Factors that increase baseline pulmonary vascular resistance (PVR) are avoided (eg, hypoxemia, hypercarbia, acidosis). The figure shows various factors during anesthesia and surgery that can precipitate RV failure with rapid hemodynamic decompensation (figure 1). Further details regarding perioperative and anesthetic management are available in a separate topic. (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure".)

•Left-sided HF – Management of failure of the systemic left ventricle in adults with CHD is similar to management of left-sided HF due to other causes. The limited available evidence on medical therapy for systemic right ventricles is inconclusive [19]. Details are available in a separate topic. (See "Intraoperative management for noncardiac surgery in patients with heart failure".)

Patients with single-ventricle physiology or an RV functioning as the systemic ventricle have a high incidence of myocardial dysfunction with progressive HF. Examples include patients born with a hypoplastic left ventricle, those with levo-transposition of the great arteries (L-TGA), or dextro-TGA (D-TGA) with a Senning or Mustard atrial switch repair in childhood [2]. In such patients, management of RV failure chronically and in the perioperative period is particularly challenging. Diastolic dysfunction is common, often with preserved systolic function, and high filling pressures may be required to maintain oxygenation and cardiac output. (See "L-transposition of the great arteries (L-TGA): Anatomy, clinical features, and diagnosis" and "D-transposition of the great arteries (D-TGA): Management and outcome".)

●Pulmonary hypertension – PH is defined as an elevated mean PAP >20 mmHg at rest [20], and may be present in adults with CHD and long standing left-to-right shunts (eg, due to atrial septal defect [ASD], ventricular septal defect [VSD], or aortopulmonary window). Patients with PH and congenital heart disease (PH-CHD) are predominantly classified in group 1 (precapillary or pulmonary arterial hypertension [PAH]), but some are in group 2 (due to left heart disease), group 3 (due to lung disease and/or hypoxia), group 4 (chronic thromboembolic PH), or group 5 (unclear or multifactorial mechanisms) (table 2) [21]. (See "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis" and "Pulmonary hypertension in adults with congenital heart disease: General management and prognosis".)

It is particularly important to continue chronically administered medications that ameliorate PH and/or right-sided HF throughout the perioperative period. (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure", section on 'Management of chronic medications' and "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure", section on 'Chronic targeted therapy for pulmonary hypertension: Patient selection'.)

●Arrhythmias – The patient’s history of cardiac arrhythmias and current electrocardiogram (ECG) should be reviewed. Both atrial and ventricular arrhythmias are common in adults with unrepaired or palliated CHD defects, particularly in those who had late intracardiac repairs [22]. Many of these patients have a pacemaker with or without biventricular pacing capability, or an implantable cardioverter-defibrillator (ICD) device. Details regarding perioperative management of these devices are available in a separate topic. (See "Perioperative management of patients with a pacemaker or implantable cardioverter-defibrillator".)

•Atrial arrhythmias – During the perioperative period, supraventricular tachyarrhythmias (SVT) are most common, particularly intra-atrial reentrant tachycardia (IART) that originates from the right atrium and is paroxysmal in nature. The incidence of supraventricular arrhythmias is highest in patients who have had a Mustard, Senning, or Fontan procedure, and in those who presented for ASD closure later in life. These defects are associated with increased right atrial volumes [23-26].

In patients older than 50 years, atrial fibrillation is the most common SVT. Such atrial arrhythmias in older CHD patients are more resistant to treatment (eg, medical management, ablation), and are more likely to be permanent [27]. Prior to any elective procedure, there should be a discussion regarding potential for arrhythmias and potential management options.

•Ventricular dysrhythmias – Ventricular tachycardia (VT) with hemodynamic instability, syncope, or death may be encountered as a late complication in patients who have undergone ventriculotomy with or without a VSD patch (eg, tetralogy of Fallot [TOF] repair). Ventricular dysrhythmias also occur in patients with reduced left ventricular (LV) or RV function.

Treatment is similar to VT caused by myocardial ischemia; thus, many patients who have a history of sustained VT have a previously inserted ICD for secondary prevention. Patients may also take medications to control arrhythmias and reduce ICD shocks [28]. Preoperative assessment includes consultation with the cardiology or institutional cardiac implantable electronic device care team regarding ICD management throughout the perioperative period, as discussed in detail in a separate topic (algorithm 1). (See "Perioperative management of patients with a pacemaker or implantable cardioverter-defibrillator".)

•Bradyarrhythmias – Bradyarrhythmias in adults with CHD usually occur as a result of direct trauma to the sinoatrial (SA) node, atrioventricular (AV) node, or coronary arteries during previous surgical procedures. Many of these patients have an atrial or dual-chamber pacing system [2].

Assessment of noncardiac sequelae — CHD impacts various organ systems in addition to the heart, including the lungs, liver, kidneys, and coagulation [29].

Pulmonary abnormalities

●Restrictive lung disease – Many patients with CHD have restrictive lung disease due to increased pulmonary blood flow or obstructed pulmonary venous drainage leading to interstitial edema, reduced lung compliance, and increased work of breathing [29-31]. Other risk factors for restrictive mechanics include previous thoracotomies (resulting in chest wall deformities), cardiomegaly (leading to direct compression of lung parenchyma), chronic administration of amiodarone, diaphragmatic nerve palsy, scoliosis, and obesity [32]. Sleep apnea is more common in adults with CHD, and is often unrecognized and untreated. Details regarding anesthetic and perioperative considerations in patients with restrictive physiology are discussed separately. (See "Anesthesia for patients with interstitial lung disease or other restrictive disorders".)

●Respiratory effects of cyanosis – Cyanotic patients have chronic hypoxemia and may exhibit a blunted ventilatory response to worsening hypoxemia compared with their response to hypercarbia [33]. Also, end-tidal carbon dioxide (ETCO₂) values in patients with cyanotic CHD usually underestimate the partial pressure of arterial carbon dioxide (PaCO₂) due to anomalies in pulmonary blood flow.

Hematologic abnormalities

●Anemia – Anemia is common in adult patients with CHD, occurring in 20 percent of patients with acyanotic disease [9,29]. These patients typically have multifactorial etiologies for anemia including iron deficiency, bleeding, renal insufficiency, and anemia of chronic disease. In those with cyanotic CHD, iron deficiency is present in approximately one-third, although this deficiency is frequently missed because of elevated hemoglobin levels associated with cyanosis [34]. Ideally, patients with cyanotic CHD are screened annually for iron deficiency with measurements of iron, ferritin, and transferrin saturation levels [29].

Similar to other adult patients undergoing cardiac surgery, iron deficiency anemia should be treated with iron (algorithm 2). In some cases, it is appropriate to postpone surgery to diagnose the cause and correct anemia to minimize or avoid perioperative transfusion of red blood cells (RBCs). (See "Preoperative evaluation for anesthesia for cardiac surgery", section on 'Anemia' and "Perioperative blood management: Strategies to minimize transfusions", section on 'Iron deficiency anemia'.)

●Hyperviscosity with stasis due to erythrocytosis – Some patients with cyanotic CHD develop secondary erythrocytosis due to chronic hypoxemia causing overproduction of erythropoietin, in order to maintain oxygen delivery despite low blood oxygen saturation [9,29]. This leads to hyperviscosity with slow flow and stasis in arterioles and capillaries, and impaired tissue perfusion. Clinical manifestations of hyperviscosity include headache and fatigue and increased risk of thrombotic events such as stroke. Volume depletion can exacerbate hyperviscosity; thus, volume repletion is first-line therapy in patients with cyanotic heart disease and hyperviscosity symptoms. (See "Medical management of cyanotic congenital heart disease in adults", section on 'Erythrocytosis and relative anemia'.)

Patients are questioned about nonspecific symptoms of poor tissue perfusion (eg, fatigue, headache, and vision changes) during the preoperative workup. Coagulation studies are typically performed. However, an important caveat for preoperative coagulation testing is that increased red cell mass reduces the plasma volume contained in a given volume of whole blood in patients with erythrocytosis; thus, the coagulation laboratory should be contacted for instructions on proper specimen collection to appropriately adjust the amount of anticoagulant. Otherwise, test results will show elevated prothrombin time (PT) and activated partial thromboplastin time (aPTT), falsely suggesting a diagnosis of disseminated intravascular coagulation (DIC). (See "Clinical use of coagulation tests", section on 'Sources of interference'.)

Suspected hyperviscosity is managed with volume repletion as needed and assessment and treatment of iron deficiency. Overnight admission for intravenous (IV) hydration on the night before surgery may be beneficial for some patients with erythrocytosis. Treatment of hyperviscosity by phlebotomy has been associated with increased risk for cerebrovascular accident and, in nonsurgical settings, is indicated only for patients with documented severe symptoms such as headache, fatigue, or evidence of end-organ damage if hemoglobin is >20 g/dL (or hematocrit >65 percent). Similarly, preoperative phlebotomy to improve hemostasis is usually reserved for selected patients with hemoglobin >20 g/dL [29,35]. (See "Medical management of cyanotic congenital heart disease in adults".)

●Bleeding and thrombosis – Patients with CHD often have risk factors for both bleeding and thrombosis [9,29]:

•A bleeding diathesis may be caused by abnormal platelet counts and function, as well as increased peripheral platelet consumption. Also, megakaryocytes may be directly delivered into the systemic circulation via a right-to-left shunt that bypasses the lungs so that the megakaryocytes are not fragmented into functional platelets [36]. Furthermore, adults with cyanotic CHD have impaired fibrinogen function and deficiencies of von Willebrand factor (vWF) [37], as well as factor V and the vitamin K dependent factors (II, VII, IX, and X) [35]. (See "Medical management of cyanotic congenital heart disease in adults", section on 'Hemorrhage'.)

•Risk factors for thrombosis include atrial arrhythmias and/or atrial dilation and erythrocytosis; in addition, stents, grafts, baffles, and catheterization sites are potential sites of thrombus formation. A thrombotic tendency increases risk of stroke in a patient with an intracardiac or extracardiac shunt due to risk of paradoxical embolization from the right to the left side of the circulatory system. (See "Medical management of cyanotic congenital heart disease in adults", section on 'Thromboembolism'.)

Notably, preoperative hypercoagulability is associated with decreased responsiveness to heparin for systemic anticoagulation during cardiac surgery [38]. (See "Blood management and anticoagulation for cardiopulmonary bypass", section on 'Heparin resistance'.)

Renal dysfunction — Many adults with CHD have renal insufficiency, which may manifest as subclinical dysfunction. The prevalence is higher in those adults with cyanotic CHD. The etiology is typically multifactorial and may include chronic hypoxemia, polycythemia, hypercellular glomeruli with basement membrane thickening, interstitial fibrosis, and exposure to nephrotoxins. Multiple previous exposures to CPB also result in renal damage due to impaired renal flow and ischemic-reperfusion injury with acute postoperative kidney injury and eventual development of chronic kidney disease after multiple cardiac surgical procedures [29,39]. Ensuring optimal baseline renal function, including preoperative hydration and avoidance of nephrotoxic agents (eg, nonsteroidal anti-inflammatory drugs [NSAIDs]) is important, although no specific strategies are known to improve outcomes in adults with CHD and pre-existing renal insufficiency. (See "Medical management of cyanotic congenital heart disease in adults", section on 'Renal dysfunction'.)

Details regarding perioperative management of chronically dialyzed patients are discussed separately. (See "Anesthesia for dialysis patients".)

Gastrointestinal abnormalities

●Hepatic dysfunction – Data regarding hepatic dysfunction in adults with CHD is limited, and focuses primarily on adults with single ventricular physiology who have liver disease due to congestive hepatopathy, which can lead to fibrosis and even cirrhosis over time [9,29,40]. Heart failure also results in congestive hepatopathy and/or ischemic liver damage due to a low flow state. Patients at particular risk include those with severe tricuspid regurgitation (including Ebstein anomaly), left-to-right shunts, pulmonic stenosis or regurgitation, or systemic venous baffle stenosis. (See "Congestive hepatopathy".)

Patients with CHD may also have ischemic hepatic damage incurred during periods of hypotension and hepatic hypoperfusion. Many are receiving chronic medications with hepatotoxic side effects (eg, amiodarone). Furthermore, cholelithiasis may be present due to increased bilirubin production resulting from erythrocytosis. (See "Ischemic hepatitis, hepatic infarction, and ischemic cholangiopathy".)

Details regarding anesthetic and perioperative considerations in patients with hepatic disease are discussed separately. (See "Anesthesia for the patient with liver disease".)

●Protein-losing enteropathy – Protein-losing enteropathy, defined as abnormal loss of serum proteins from the gut, may present as chronic diarrhea or with secondary manifestations of hypoalbuminemia such as peripheral edema, ascites, clotting abnormalities, or recurrent infections [29].

●Esophageal varices – CHD patients with hepatic dysfunction are at risk for esophageal varices, which should be considered prior to insertion of a transesophageal echocardiography (TEE) probe. (See "Transesophageal echocardiography: Indications, complications, and normal views", section on 'Safety of TEE examination'.)

INTRAOPERATIVE MANAGEMENT: GENERAL APPROACH — The anesthetic plan for cardiac surgery in adults with congenital heart disease (CHD) should be individually designed considering patient-specific cardiac anatomy and function, as well as procedure-specific requirements.

Intravascular access

●Preparations must be made to avoid systemic air embolism if intracardiac and/or extracardiac shunts may be present. For this reason, each intravenous (IV) line must be meticulously flushed to remove all air bubbles and avoid the possibility of systemic air embolism. Also, air filters are used in all IV 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 medication administered. (See "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Precautions to avoid air embolism'.)

●Large-bore IV catheter insertion should also be accomplished prior to induction of anesthesia. Also, IV infusions of inotropic and vasopressor medications should be prepared in advance and ready for use before induction of anesthesia (table 3). Some patients may require vasoactive support prior to induction.

Monitoring

●In addition to standard American Society of Anesthesiology (ASA) monitors, before induction of anesthesia, multifunction external pacing/defibrillation/cardioversion pads attached to an external defibrillator with pacing capability are placed on most patients with CHD. This is particularly important for those undergoing redo sternotomy due to the high risk of arrhythmias leading to hemodynamic instability during sternotomy and subsequent surgical dissection. (See "Anesthesia for surgical repair of congenital heart defects in adults: Management of specific lesions and reoperation", section on 'Lesions requiring reoperation or redo sternotomy'.)

●An intra-arterial catheter is inserted prior to induction so that hemodynamic changes during and immediately after induction may be immediately identified and treated. This is particularly important in patients with pulmonary hypertension (PH) or poor ventricular function. (See 'Pulmonary abnormalities' above and 'Assessment of cardiovascular sequelae' above.)

Notably, the anatomy of the patient’s original cardiovascular defect or previous palliative procedures may affect site selection for arterial catheter insertion. For example, patients with a previous classic Blalock-Taussig (BT) shunt have an end-to-side anastomosis of the subclavian artery to the pulmonary artery (PA) (figure 2). Since blood pressure will be low on the side of the shunt, arterial access should be on the contralateral side.

●Following induction, a large central venous introducer sheath is inserted, which allows for administration of large volumes of blood and crystalloid if necessary, as well as infusion of vasoactive agents, and monitoring of central venous pressure (CVP). Again, it is important to understand the patient’s cardiovascular anatomy and to be aware of previous palliative procedures that may alter plans for central venous catheter (CVC) insertion and subsequent monitoring [9].

Patients who have had multiple previous procedures or history of extracorporeal membrane oxygenation may have ligated or permanently thrombosed central vessels, so cautious insertion of any central venous catheter is accomplished with ultrasound guidance after confirmation of vessel patency. Note that in patients with Glenn or Fontan shunts, the CVP value actually reflects the mean pulmonary artery pressure (PAP). In addition, patients with single ventricle physiology with a Glenn shunt or Fontan circulation are at risk for thrombotic complications and have a high risk for thrombosis of any indwelling central catheter. (See "Management of complications in patients with Fontan circulation", section on 'Thrombosis'.)

●Intraoperative transesophageal echocardiography (TEE) is indicated during repair of congenital cardiac defects unless there is a contraindication [9,41,42]. (See "Intraoperative transesophageal echocardiography for noncardiac surgery", section on 'Contraindications and precautions'.)

Shortly after induction of general anesthesia, a TEE probe is inserted for continuous monitoring. Also, complete TEE examinations are performed before and after cardiopulmonary bypass (CPB) [43]:

•The full prebypass examination is done to confirm the preoperative transthoracic echocardiography (TTE) examination findings, and also to identify any previously undiagnosed lesions such as patent foramen ovale (PFO), residual shunt, or intracardiac thrombus. Furthermore, assessment of current ventricular function and reexamination of valvular function are important.

•Postbypass TEE examination is performed to assess adequacy of the surgical repair, identify any residual shunt, obstruction, or new pathology, and reassess current ventricular function. TEE evaluation for any aortic pathology is also necessary after CPB.

●Many centers employ neurological monitoring with near infrared spectroscopy (NIRS) as a trend monitor for cerebral blood flow regulation since it is a real time monitor of cerebral oxygen supply and demand. While use in pediatric congenital cardiac surgery is widespread, data are limited regarding whether outcomes are improved, and no specific data support or refute its use in adults with CHD [44,45]. (See "Anesthesia for cardiac surgery: General principles", section on 'Brain monitors'.)

Anesthetic induction and maintenance — If anxiolytics are administered shortly before induction, only small carefully titrated doses are employed (eg, midazolam in 0.5 mg increments). Particular care is taken to maintain adequate ventilation and oxygenation since even moderate degrees of respiratory depression may precipitate sudden catastrophic increases in pulmonary vascular resistance (PVR) and acute right ventricular (RV) dysfunction.

Selection of anesthetic induction agents is based on the expected hemodynamic effects of each anesthetic agent, the hemodynamic goals for the patient's specific CHD lesion (ie, maintaining a balance between systemic and pulmonary blood flow), and the presence of ventricular dysfunction. The choice of anesthetic induction and maintenance agents is not as important as maintaining hemodynamic stability in the individual patient with a specific congenital cardiac lesion (table 4). There is no evidence supporting use of any individual IV or volatile anesthetic agent in patients with CHD. However, any agent with myocardial depressant effects (eg, propofol, volatile inhalation agents) is carefully titrated in order to maintain myocardial contractility. (See "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Hemodynamic goals for specific lesions'.)

Propofol (0.5 to 1 mg/kg), ketamine (0.5 to 2 mg/kg), or etomidate (0.3 mg/kg) are acceptable induction agents for most adult patients with CHD (see "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Choice of induction agent'). Patients are encouraged to hyperventilate with 100 percent oxygen during induction, and respiratory depressants (such as opiates) are best avoided. Patients should be deeply anesthetized prior to laryngoscopy to prevent excessive sympathetic stimulation. Once controlled ventilation is established, elevated peak airway pressures and high tidal volumes are avoided, as these settings may decrease venous return and/or increase PVR thereby worsening right-to-left shunting and cyanosis. Inspired concentration of oxygen should be quickly adjusted to maintain the patient's baseline oxygen saturation, as significant deviation from baseline affects PVR and pulmonary blood flow.

Maintenance of anesthesia is usually accomplished with a carefully titrated potent volatile inhalation agent, supplemented with judicious doses of an opioid and a neuromuscular blocking agent. (See "Anesthesia for cardiac surgery: General principles", section on 'Maintenance techniques' and "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Maintenance'.)

Management of intraoperative problems

●Hemodynamic management – General hemodynamic goals are to maintain optimal end organ perfusion and oxygen delivery by maintaining near-normal heart rate (HR), preload, afterload, and contractility, as well as avoiding exacerbation of preexisting PH. Specific hemodynamic goals depend on the underlying pathophysiology of the CHD lesion, as noted in the table (table 4), although modifications may be necessary in individual patients. Notably, intraoperative changes in patient positioning, ventilation, blood loss, and administration of anesthetic agents all cause changes in the systemic vascular resistance (SVR) and/or PVR that may adversely affect the balance of pulmonary blood flow (Qp) versus systemic blood flow (Qs).

Occasionally, patients with severe systemic ventricular failure will undergo insertion of a ventricular assist device (VAD). Details regarding anesthetic management for VAD placement are presented in a separate topic. (See "Anesthesia for placement of ventricular assist devices".)

●Management of arrhythmias – Most patients with CHD are predisposed to development of arrhythmias [9,46]. Most intraoperative arrhythmias are adequately managed with electrolyte repletion and pharmacologic agents (eg, amiodarone), while ensuring adequate inotropy if there is myocardial dysfunction. Patients with hemodynamic instability associated with an arrhythmia may require immediate cardioversion. Inotropes are used only as required given the proarrhythmic risk with these agents.

After CPB, temporary epicardial pacemaker leads are routinely placed. For patients with complex anatomy and an indication for permanent pacing, transvenous lead placement for pacing may not be an option. Preoperative multidisciplinary discussion should include determination of whether permanent epicardial lead placement is indicated after CPB.

●Management of bleeding and coagulation – Before CPB, preoperative hypercoagulability may be associated with decreased responsiveness to heparin administration for systemic anticoagulation during cardiac surgery; management is individualized with hemostatic monitoring and available point-of-care testing (algorithm 3) (see "Blood management and anticoagulation for cardiopulmonary bypass", section on 'Heparin resistance') [38,47]. After CPB, bleeding and coagulopathy are common in patients with various CHD defects. Management of anticoagulation, bleeding, and coagulation during cardiac surgical procedures is discussed in detail separately. (See "Blood management and anticoagulation for cardiopulmonary bypass".)

EARLY POSTOPERATIVE MANAGEMENT

●Intensive care unit admission – Patients are admitted to an intensive care unit (ICU) following cardiac surgery for continuous hemodynamic monitoring and management of vasopressor titration, fluid administration, mechanical ventilation, sedation, analgesia, and, in some cases, mechanical circulatory support.

●Planning for extubation – At our institution, many patients having repair of congenital heart disease (CHD) lesions are appropriate candidates for fast-track extubation (typically within six hours of surgery) or ultra-fast-track extubation (ie, in the operating room). We use the same extubation criteria for these patients as for other adult cardiac surgical patients. (See "Extubation management in the adult intensive care unit".)

In rare cases, prolonged intubation with controlled mechanical ventilation is necessary. Management of adult respiratory distress syndrome (ARDS) in patients with CHD includes use of lung-protective ventilation and conservative fluid management, similar to other patients with ARDS (see "Postoperative complications among patients undergoing cardiac surgery", section on 'Pulmonary dysfunction') [9]. Postoperative pulmonary complications are more likely to occur in certain patients with CHD due to factors that include:

•Pre-existing pulmonary hypertension (PH) — Useful adjuncts in patients with PH include nitric oxide (eg, 20 parts per million) and inhaled epoprostenol (eg, 50 ng/kg/min) [48]. Both can be administered via an endotracheal tube or noninvasively via nasal cannula or face mask. (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure", section on 'Chronic targeted therapy for pulmonary hypertension: Patient selection'.)

•Pre-existing restrictive lung disease – Perioperative respiratory management of these patients is described separately. (See "Anesthesia for patients with interstitial lung disease or other restrictive disorders", section on 'Mechanical ventilation' and "Anesthesia for patients with interstitial lung disease or other restrictive disorders", section on 'Management in the post-anesthesia care unit'.)

•Pulmonary edema may suddenly develop following certain procedures, such as branch pulmonary artery dilations resembling reperfusion injury. Worsening left ventricular (LV) dysfunction or inflammation due to exposure to CPB may also precipitate pulmonary edema.

•Bronchial compression – An enlarged pulmonary artery and/or left atrium may cause bronchial compression and inadequate ventilation of the left lung.

After extubation, patients remain carefully monitored for respiratory depression, which can lead to hypercarbia and hypoxemia with devastating increases in pulmonary vascular resistance (PVR) [49]. In general, patients with CHD are at risk for requiring additional respiratory support after early extubation (eg, high flow nasal cannula), and some require reintubation.

●Ensuring adequate analgesia – Postoperative pain and anxiety are treated appropriately to facilitate adequate oxygenation and ventilation, and to prevent increases in sympathetic tone. Similar to other cardiac surgical patients, those with CHD are candidates for multimodal analgesia.

•Opioids are the mainstay of treatment. Examples include fentanyl (administered in 25 to 50 mcg boluses as frequently as every 30 minutes) or hydromorphone (0.5 to 1 mg every two hours). These opioids may be administered via a patient-controlled analgesia (PCA) technique if bolus dosing is insufficient. Once extubated and taking oral liquids, patients are typically transitioned to an oral opioid such as oxycodone.

•To minimize opioid dosing, nonopioid analgesic adjunct agents administered as part of a multimodal regimen may include:

-Dexmedetomidine administered as an infusion at 0.1 to 1 mcg/kg per hour. Intravenous (IV) dexmedetomidine can be safely used during postoperative transport in either an intubated or an extubated patient, and may also be continued in the ICU until the patient is able to take oral medications [9]. Its use may minimize the risk of tachycardia and delirium [9]. It is typically used as a first-line agent.

-Ketamine administered as an infusion at 0.15 to 0.35 mg/kg/hour. It is typically used as a second-line agent when IV opioids and dexmedetomidine are inadequate.

-Ketorolac administered as a 15 to 30 mg dose every six hours, if chest tube output is low and renal and platelet function is normal.

-Parenteral acetaminophen is employed as an adjunct analgesic agent for all cardiac surgical patients at our institution, except for those with severe liver disease. For patients >50 kg, the parenteral dose is 1000 mg IV every six hours or 650 mg IV every four hours. For patients <50 kg, the parenteral dose is 15 mg/kg every six hours or 12.5 mg/kg every four hours.

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

●Preanesthesia consultation – The preanesthesia consultation ensures understanding of the clinically relevant cardiac and noncardiac sequelae of the specific congenital heart disease (CHD) lesion (see 'Preanesthesia consultation' above):

•Assessment of congenital lesion – This includes a thorough review of the patient's diagnosis (table 1), complications, cardiac surgical and catheter-based interventional history, current physiology, recent imaging studies, and laboratory data to understand the complexity of the lesion, physiological status, concurrent conditions and risk factors, and the planned surgical procedure. (See 'Assessment of congenital lesion' above.)

•Assessment of cardiac and noncardiac sequelae – Common cardiovascular sequelae in adults with CHD include heart failure, pulmonary hypertension, and arrhythmias. Common noncardiac sequelae include pulmonary disorders (eg, restrictive lung disease, respiratory effects of cyanosis), hematologic abnormalities (eg, anemia, hyperviscosity, bleeding or thrombosis), renal dysfunction, and gastrointestinal abnormalities (eg, hepatic dysfunction, protein-losing enteropathy). (See 'Assessment of cardiovascular sequelae' above and 'Assessment of noncardiac sequelae' above.)

●Intraoperative management approach – The anesthetic plan for cardiac surgery in adults with CHD should be individually designed considering patient-specific cardiac anatomy and function, as well as procedure-specific requirements:

•Intravascular access – Preparations to avoid systemic air embolism include meticulous flushing to remove all air bubbles and use of air filters for all intravenous (IV) lines. Large-bore IV catheter insertion should also be accomplished prior to induction of anesthesia. (See 'Intravascular access' above.)

•Monitoring – Invasive cardiovascular monitors include an intra-arterial catheter inserted prior to anesthetic induction, and a large central venous introducer sheath that allows for administration of large fluid volumes and monitoring of central venous pressure (CVP), as well as a transesophageal echocardiography (TEE) probe inserted after induction. Many centers also employ neurological monitoring with near infrared spectroscopy (NIRS). (See 'Monitoring' above.)

•Anesthetic induction and maintenance – Selection of anesthetic induction agents is based on the expected hemodynamic effects of each anesthetic agent, the hemodynamic goals for the patient's specific CHD lesion (ie, maintaining a balance between systemic and pulmonary blood flow), and the presence of ventricular dysfunction. The choice of anesthetic induction and maintenance agents is not as important as maintaining hemodynamic stability in the individual patient with a specific congenital cardiac lesion (table 4). (See 'Anesthetic induction and maintenance' above.)

•Management of intraoperative problems – Common intraoperative problems include hemodynamic instability, arrhythmias, bleeding, and hypercoagulability. (See 'Management of intraoperative problems' above.)

●Postoperative management – Conditions that increase the risk of postoperative pulmonary complications include pre-existing pulmonary hypertension, pre-existing restrictive lung disease, conditions that increase the risk of pulmonary edema, and bronchial compression. (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.