Anesthesia for surgical repair of congenital heart defects in adults: Management of specific lesions and reoperation

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

This topic reviews specific considerations in the anesthetic management for adults with selected CHD defects requiring primary cardiac surgical repair or reoperation. General management of anesthesia for adults requiring cardiac surgical repair of CHD defects is discussed in a separate topic. (See "Anesthesia for surgical repair of congenital heart defects in adults: General management".)

Other topics address anesthetic management for noncardiac surgery or for labor and delivery, in adult patients with 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".)

ANESTHETIC MANAGEMENT OF SPECIFIC LESIONS — Key hemodynamic goals during anesthetic management of adults undergoing repair of specific congenital heart disease (CHD) lesions are noted in the table (table 1).

Left-to-right shunt lesions — This section discusses the most common congenital defects causing left-to-right shunts.

Lesion types and clinical manifestations — Congenital defects producing left-to-right shunting include atrial and ventricular septal defects (ASD, VSD) and patent ductus arteriosus (PDA). With left-to-right shunting, some oxygenated blood returns to the lungs instead of being pumped to the systemic circulation; thus, the ratio of pulmonary-to-systemic flow (Qp:Qs) is >1:1 and, in severe cases >3:1. Pulmonary hypertension (PH) may develop in adults with these defects due to a long-standing excess pulmonary blood flow, and may be further exacerbated by concurrent conditions such as lung disease, left heart dysfunction, or obstructive sleep apnea. (See "Pulmonary hypertension with congenital heart disease: Clinical manifestations and diagnosis".)

●Atrial septal defects (figure 1) – ASD types include the secundum type, primum type (nearly always associated with atrioventricular [AV] valve abnormalities), sinus venosus defect (most associated with anomalous pulmonary venous return), and unroofed coronary sinus (the rarest type). Physiologically, flow across an ASD occurs in both systole and diastole, and the direction of flow in diastole depends on the atrial pressure difference, which is related to the relative compliance of each ventricle. Such increased pulmonary blood flow results in right ventricular (RV) volume overload and possible, though uncommon, development of progressive pulmonary vascular obstructive disease and PH, which may cause right heart failure (HF). An ASD may come to clinical attention in adulthood due to symptoms of dyspnea caused by an increase in left-to-right shunting with increased pulmonary blood flow as the left ventricle (LV) becomes less compliant with age [4]. As the atria enlarge, a previously asymptomatic ASD may come to clinical attention after onset of an atrial arrhythmia or after a stroke caused by paradoxical embolism across the ASD [4]. (See "Clinical manifestations and diagnosis of atrial septal defects in adults".)

●Ventricular septal defects (figure 2) – VSD types include infundibular VSD (type 1), membranous VSD (type 2), inlet VSD (type 3), muscular VSD (type 4), and Gerbode defect (the least common type, which causes an LV to right atrial shunt). Most shunting across a VSD occurs in systole, with the volume of shunted blood determined by the size of the defect and, in the absence of RV outflow obstruction, the relative resistances in the pulmonary and systemic arterial circulations. (See "Clinical manifestations and diagnosis of ventricular septal defect in adults".)

In adults with uncorrected VSD, the spectrum of clinical manifestations ranges from small restrictive VSDs that generally remain asymptomatic and rarely require closure, to moderately restrictive defects that may cause symptoms of LV volume overload or PH, to large nonrestrictive VSDs that generally cause progressive PH. An uncorrected VSD may come to clinical attention when left atrial and pulmonary venous congestion cause symptoms of dyspnea as the LV becomes less compliant with aging and is no longer able to accommodate the volume load of the shunt. In some cases, a VSD is diagnosed in adulthood when aortic regurgitation develops due to aortic cusp prolapse into an infundibular or membranous VSD, or when infective endocarditis is diagnosed.

●Patent ductus arteriosus – The ductus arteriosus connects the aorta and the pulmonary artery (PA) and occasionally remains patent into adulthood. As with a VSD, the volume of shunted blood across a PDA depends on the size of the connection and the relative resistances in the pulmonary and systemic circulations. The PDA generally places a volume load on the LV, as blood from the aorta returns to the PA and eventually to the left atrium and LV, resulting in left heart enlargement, and sometimes result in elevated pulmonary pressures. (See "Clinical manifestations and diagnosis of patent ductus arteriosus (PDA) in term infants, children, and adults".)

Management — The anesthetic management of surgical repair of congenital defects causing left-to-right shunts is discussed here. Further discussion regarding surgical repair of an ASD, VSD, or PDA is available in other topics:

●(See "Surgical and percutaneous closure of atrial septal defects in adults", section on 'Surgical closure'.)

●(See "Management and prognosis of congenital ventricular septal defect in adults", section on 'VSD closure'.)

●(See "Management of patent ductus arteriosus (PDA) in term infants, children, and adults", section on 'Surgical ligation'.)

●Prebypass management – A midline sternotomy is most often used for open cardiac surgical repair of an ASD or VSD. A PDA is generally closed percutaneously; rarely, it may be closed via left thoracotomy or sternotomy. Minimally invasive and robot-assisted approaches have become increasingly common for selected procedures, with demonstrated safety and efficacy [5,6]. These newer approaches have implications for anesthetic management. In particular, use of lung isolation and one lung ventilation can increase pulmonary vascular resistance (PVR).

Hemodynamic management goals for left-to-right shunt lesions are noted in the table (table 1). In particular, a normal to slightly low systemic vascular resistance (SVR) is maintained to minimize left-to-right shunting. However, vasodilation with very low SVR is avoided in patients with a PDA, as this may result in diastolic run-off that compromises coronary perfusion.

Also, a normal to slightly high PVR is maintained to minimize left-to-right shunting. The fraction of inspired oxygen (FiO₂) concentration is set at 21 percent (or the lowest concentration that produces near-normal oxygen saturations), and mild hypoventilation is employed to maintain partial pressure of carbon dioxide (PaCO₂) at 40 to 45 mmHg, thereby avoiding low PVR that may exacerbate left-to-right shunting. Hyperoxia and hyperventilation are avoided.

●Postbypass management – Pulmonary hypertension, ventricular dysfunction, and/or arrhythmias may be observed during attempts to wean from cardiopulmonary bypass (CPB) or in the postbypass period. After closure of a defect causing a left-to-right shunt, patients with long-standing increased pulmonary blood flow and PH are at risk for LV failure. This occurs because the affected ventricle loses its original "pop-off" into a circulation with lower vascular resistance, and may not be able to accommodate a normal ventricular volume [4].

In most cases, an intravenous inotrope infusion (eg, epinephrine) is administered to treat LV or RV dysfunction and achieve separation from CPB and postbypass hemodynamic stability (table 2). Milrinone may also be administered if the patient has RV dysfunction with significant PH. (See "Intraoperative problems after cardiopulmonary bypass", section on 'Left ventricular dysfunction' and "Intraoperative problems after cardiopulmonary bypass", section on 'Right ventricular dysfunction'.)

Arrhythmias, most commonly supraventricular tachycardias, are promptly treated with the appropriate pharmacologic agents. Beta blocker therapy started prior to or immediately after surgery may reduce the risk of postoperative atrial fibrillation. Temporary AV pacing is often necessary to manage bradycardia or complete heart block. Management of these perioperative arrhythmias is discussed in other topics. (See "Intraoperative problems after cardiopulmonary bypass", section on 'Arrhythmias' and "Surgical and percutaneous closure of atrial septal defects in adults", section on 'Perioperative management' and "Management and prognosis of congenital ventricular septal defect in adults", section on 'Postoperative course' and "Atrial fibrillation and flutter after cardiac surgery", section on 'Beta blockers'.)

Coarctation of the aorta

Lesion and clinical manifestations — Aortic coarctation is a narrowing in the lumen of the aorta usually located at or just distal to the insertion of the ductus arteriosus (image 1). Aortic coarctation is associated with long-term cardiovascular sequelae even after repair, particularly hypertension and associated cardiovascular risk (including coronary artery disease, stroke, and HF), as well as risks for development of recoarctation, intracranial aneurysms, and aortic disease (aneurysm, dissection, and rupture) [7]. Presentation in adulthood depends on the degree of aortic obstruction and the extent of collateral blood flow (which may delay symptoms and diagnosis). (See "Clinical manifestations and diagnosis of coarctation of the aorta" and "Management of coarctation of the aorta", section on 'Long-term cardiovascular complications'.)

Management — Anesthetic management during the intraoperative and early postoperative periods are discussed here. Further discussion regarding initial surgical management of coarctation, recoarctation, and associated aortic complications is available in another topic. (See "Management of coarctation of the aorta".)

●Prebypass management – Blood pressure (BP) is typically monitored in both upper and lower extremities in patients with aortic coarctation since hypertension is usually present in the arm(s) while BP in the legs is reduced. Depending on specific coarctation and subclavian arterial anatomy, diminished BP can occur in one or both upper extremities. Preoperative arterial line placement is planned based on the patient's anatomic features. Generally, an intra-arterial catheter for continuous BP monitoring is inserted in an upper extremity before induction of general anesthesia, and a noninvasive BP cuff is positioned on a lower extremity. For patients with severe coarctation, we insert a second intra-arterial catheter in a lower extremity (typically the femoral artery). Key goals for hemodynamic management of patients with coarctation of the aorta are noted in the table (table 1).

Cardiac surgical repair is often accomplished via left thoracotomy; rarely, median sternotomy may be required for long segments of coarctation and in those patients who have concomitant cardiac disease that requires intervention (eg, aortic valve disease, ascending aorta aneurysm, coronary artery disease). Median sternotomy with CPB is employed if an extended aortic cross-clamp time is anticipated, or if the cross-clamp is likely to compromise collateral flow [8]. If lower extremity BP decreases to <50 mmHg upon initial placement of the aortic cross-clamp, CPB will probably be necessary [9]. Other surgical and anesthetic considerations include the need to minimize the aortic cross-clamp time, avoid hemorrhage, and avoid or promptly treat hypotension to minimize risk for postoperative complications, particularly paraplegia.

Notably, patients with extensive collateral vasculature are at risk for vessel rupture and hemorrhage. In particular, those with aortic friability or presence of an aortic aneurysm have increased risk for intraoperative aortic dissection or rupture [9].

Similar to intraoperative management of other thoracic aortic disease, moderate hypertension may be tolerated while the aorta is cross-clamped to ensure adequate perfusion distal to the clamp. Also, mild hypothermia (34 to 35°C) is typically maintained during aortic cross-clamping and continuing into the immediate post-repair period to lessen the risk of paraplegia. (See "Anesthesia for open descending thoracic aortic surgery", section on 'Review of the surgical plan'.)

●Postbypass management – In the immediate postoperative period, some patients develop paradoxical hypertension after coarctation repair, typically managed with nicardipine, esmolol, nitroglycerin, or nitroprusside (table 3). Administration of a dexmedetomidine infusion at 0.2 to 0.5 mcg/kg per hour during the intraoperative and early postoperative periods may also be helpful to control hypertension, and to augment analgesia and sedation.

However, patients who had severe preoperative LV dysfunction may have hypotension after coarctation repair, and an inotrope infusion (eg, epinephrine) may be necessary to maintain adequate BP (table 2). Other early post-repair complications include left recurrent laryngeal nerve paralysis, phrenic nerve injury, or subclavian steal. Paraplegia and mesenteric arteritis with bowel infarction are rare complications. (See "Management of coarctation of the aorta", section on 'Surgery'.)

Recurrent aortic coarctation — Recurrence of coarctation may occur after original aortic repair, and may be associated with other congenital abnormalities, such as hypoplasia of the aortic arch, aneurysmal dilation of the ascending aorta, or aortic valve disease (with bicuspid aortic valve). (See "Management of coarctation of the aorta", section on 'Recoarctation' and "Management of coarctation of the aorta", section on 'Aortic aneurysm, dissection, and rupture'.)

●For aortic aneurysmal dilation – Dilation at the site of the previous coarctation can usually be managed with an endovascular approach. Since the stent is deployed through the femoral artery using fluoroscopic guidance, large-bore central venous access is achieved by the proceduralist with placement of a femoral venous introducer. Anesthetic management of this procedure is generally similar to that for other aortic endovascular procedures, as described in detail in a separate topic. (See "Anesthesia for endovascular aortic repair".)

●For enlarging thoracic aortic aneurysms – Large descending thoracic aortic aneurysms that are not amenable to endovascular repair may require an open thoracoabdominal approach. A double-lumen endotracheal tube is inserted to achieve lung isolation. Both a right radial and a femoral intra-arterial catheter are necessary. Monitoring for spinal cord ischemia is typically employed (eg, motor evoked potentials [MEPs], somatosensory evoked potentials [SSEPs]), as well as cerebrospinal fluid (CSF) pressure monitoring and CSF drainage as necessary to treat spinal cord ischemia (table 4).

In some patients, partial left heart bypass may be used to maintain perfusion to the spinal cord during proximal aortic cross-clamping. In others, cardiac surgical management with a redo sternotomy and full CPB may be necessary (eg, recurrent coarctation with associated lesions in the ascending aorta or those that involve the aortic valve). Additional procedures such as a carotid-subclavian bypass may also be performed in selected patients depending on the location of the aortic lesion.

Further details regarding anesthetic management of descending thoracic aortic procedures are described in a separate topic. (See "Anesthesia for open descending thoracic aortic surgery".)

Rarely, a period of elective deep hypothermia and circulatory arrest may be necessary to complete the repair. (See "Anesthesia for aortic surgery with hypothermia and elective circulatory arrest in adult patients".)

Sinus of Valsalva aneurysm or fistula

Lesion and clinical manifestations — A sinus of Valsalva aneurysm may occur as a congenital or acquired defect at the junction of the aortic media and the surrounding fibrous tissue. Sinus of Valsalva aneurysms are generally asymptomatic. Rarely, they can cause compression of adjacent cardiac chambers, the conduction system, or coronary arteries.

Sinus of Valsalva aneurysm rupture into the cardiac chambers results in continuous shunting from aorta to the receiving chamber. Depending on the size of the defect, patients generally present with acute onset of symptoms that include dyspnea and, in some cases, acute HF [10]. The precise location of the aneurysm determines which chamber is affected. Right or noncoronary sinus aneurysm rupture results in left-to-right shunting through a fistula between the aorta and right atrium or RV, while a left sinus aneurysm rupture creates a fistula between the aorta and the left atrium or LV [11]. (See "Echocardiographic evaluation of the thoracic and proximal abdominal aorta", section on 'Sinus of Valsalva aneurysms'.)

Dilated right sinus of Valsalva commonly occurs in patients with outlet ventricular septal defect. A VSD should always be sought and should be closed at the time of surgical intervention. Aortic valve regurgitation can also occur as a result of distortion of the aortic annulus and disruption of aortic valve coaptation. Aortic valve repair, or less commonly replacement, may be required. (See "Management and prognosis of congenital ventricular septal defect in adults", section on 'Aortic regurgitation' and "Management and prognosis of congenital ventricular septal defect in adults", section on 'Sinus of Valsalva aneurysm' and 'Aortic and mitral valve disease' below.)

Upon presentation for surgical repair, an adult patient may exhibit symptoms of increased pulmonary blood flow, coronary ischemia due to aneurysm compression of a coronary artery, conduction abnormalities, or aortic regurgitation.

Management — Surgical repair includes resection of the aneurysm and closure of any fistula, as well as closure of the sinus of Valsalva defect to prevent aneurysm recurrence, and repair of associated anomalies.

●Prebypass management – Hemodynamic management goals for patients with sinus of Valsalva aneurysm or rupture are noted in the table (table 1). In particular, it is important to maintain a normal HR and normal SVR to support coronary perfusion in patients with coronary compression or evidence of myocardial ischemia. Also, a normal to slightly high PVR is maintained to decrease left-to-right shunting.

●Postbypass management – An inotrope infusion (eg, epinephrine) may be necessary during and after weaning from CPB if the patient had preoperative HF symptoms or if signs of ventricular dysfunction are noted on postbypass transesophageal echocardiography (TEE) examination (table 2). Aortic regurgitation may be present if the aortic valve was affected and not adequately repaired.

Anomalous origin of a coronary artery

Lesion and clinical manifestations — Coronary arteries may arise variably from the aorta, or from the pulmonary artery [12-14]. A coronary artery that arises from the opposite sinus or other aortic location may lead to symptoms or be asymptomatic, depending on its course or location (see "Congenital and pediatric coronary artery abnormalities"). Anomalous coronary artery origin from the pulmonary artery (PA) instead of the aorta is usually symptomatic and requires repair early in life. Diagnosis is made in infancy if the left (rather than the right) coronary artery originates from the PA, though presentation in an adult occasionally occurs if sufficient collateral circulation has developed from the right to the left coronary artery [15]. Eventually, even in the presence of collaterals, coronary steal and subendocardial ischemia develop due to flow of coronary blood to the PA [12,16]. Ischemia may manifest as angina, arrhythmias, or sudden death. Poor function of the affected ventricle may also be present.

Management — Cardiac surgery is the treatment of choice for certain congenital coronary anomalies. The operation performed to repair the coronary anomaly depends on the type of anomaly, presentation, and patient characteristics. Unroofing is preferred for anomalous origin of the coronary artery from the opposite sinus with an intramural course. Reimplantation into the aorta is preferred for patients who have an anomalous coronary artery arising from the pulmonary artery. Notably, coronary artery bypass is generally avoided as this results in competitive coronary artery flow. Mitral regurgitation (MR) due to a structural or functional abnormality of the mitral valve may be present; repair or replacement of the valve should be considered at the time of coronary surgery since MR severity is unlikely to improve in the postoperative period [15]. Other aspects of surgical management of congenital coronary artery anomalies are discussed in a separate topic. (See "Congenital and pediatric coronary artery abnormalities".)

●Prebypass management – Anesthetic management of adult patients with congenital coronary anomalies is similar to that for adults undergoing coronary artery bypass grafting (CABG) for coronary artery disease of other etiologies (see "Anesthesia for coronary artery bypass grafting surgery"). Hemodynamic management goals for these patients, who may have coronary ischemia and poor ventricular function, are noted in the table (table 1). In particular, a normal to slow HR is maintained since tachycardia reduces time in diastole which reduces coronary perfusion and exacerbates myocardial ischemia (figure 3). A normal SVR is maintained to support coronary perfusion. However, in patients with an anomalous coronary arising from the PA, BP should not be so high that shunting of blood from the aorta to the PA is increased. Also, a normal to high PVR is maintained in these patients to reduce aortic-to-PA shunting. (See "Anesthesia for coronary artery bypass grafting surgery", section on 'Avoidance and treatment of ischemia'.)

●Postbypass management – TEE is employed during and after weaning from CPB to determine whether significant MR and/or significant ventricular dysfunction are present. An inotrope infusion (eg, epinephrine) is typically required to achieve weaning from CPB and hemodynamic stability in the postbypass period. Infusion of vasopressin may also be necessary to maintain adequate coronary perfusion (table 2). Persistent functional MR usually improves over time with reperfusion of the ventricle and is managed in the postbypass phase by supporting contractility and lowering afterload [17].

Valve surgery — Congenital defects resulting in cardiac valve stenosis or regurgitation may present for primary surgical repair or replacement in adulthood. These congenital valve lesions are generally physiologically similar to acquired valve diseases, but some (such as Ebstein anomaly) are associated with other cardiac lesions.

Aortic and mitral valve disease — Details regarding anesthetic management for surgical repair or replacement of cardiac valve lesions are presented in the table and in a separate topic (table 5). (See "Anesthesia for cardiac valve surgery".)

Pulmonic valve disease

●Pulmonic stenosis – Pulmonic stenosis (PS) is a relatively common lesion, occurring in an estimated 7 to 12 percent of patients with CHD [18]. PS is most commonly an isolated lesion, but it also occurs in combination with other malformations such as VSD, ASD, patent ductus arteriosus (PDA), or tetralogy of Fallot (TOF). Adult patients with PS may present with RV hypertrophy and dysfunction, or with arrhythmias. While balloon valvuloplasty is typically the first line of treatment for PS, pulmonic valve replacement may be necessary for complex lesions or if associated defects are present (such as hypoplastic pulmonary annulus, severe pulmonic regurgitation, subvalvular pulmonic stenosis, supravalvular pulmonic stenosis, severe tricuspid regurgitation, and most dysplastic pulmonic valves) [19]. (See "Pulmonic valve stenosis in adults: Management".)

Preanesthetic consultation includes assessment for ventricular dysfunction (particularly the RV), and for the presence of arrhythmias with or without previous insertion of an implantable cardioverter-defibrillator (ICD) or a pacemaker.

Hemodynamic management goals for patients with PS are generally similar to those for patients with Tetralogy of Fallot (table 1).

●Pulmonic regurgitation – Since intervention for pulmonic regurgitation (PR) in patients with CHD is commonly performed after prior cardiac surgery (eg, surgery for tetralogy of Fallot), this is discussed below. (See 'Pulmonic valve intervention' below.)

Tricuspid valve disease — Since many adults with CHD requiring tricuspid valve surgery have undergone prior cardiac surgery, management for tricuspid valve surgery is discussed below. Ebstein anomaly is one congenital lesion that causes tricuspid regurgitation and RV dysfunction as described below. (See 'Tricuspid valve surgery' below and 'Ebstein anomaly' below.)

Ebstein anomaly

Lesion and clinical manifestations — Ebstein anomaly is a rare CHD defect (approximately 1 in 2000 live births), which is characterized by apical displacement of the septal and posterior leaflets of the tricuspid valve, resulting in tricuspid regurgitation (TR) [20]. The morphology of the tricuspid valve and associated clinical presentation is highly variable. The tricuspid valve leaflets demonstrate variable degrees of direct fibrous attachment to the RV endocardium due to failed delamination (separation of valve tissue from the myocardium). Reduced size and dysfunction of the RV result from "atrialization" of the RV. These changes in RV geometry may also result in reduced LV function. The clinical spectrum ranges from severe HF with death in utero or shortly after birth, to severe tricuspid regurgitation and right HF in children and adults, to mild tricuspid regurgitation in adults. Mild forms of Ebstein anomaly may not present until adulthood, usually with new onset of atrial flutter in the setting of an acute illness or pregnancy. (See "Ebstein anomaly: Clinical manifestations and diagnosis".)

Supraventricular arrhythmias are common due to associated accessory conduction pathways. Syncope and sudden death may be caused by atrial fibrillation with rapid ventricular response due to conduction through an accessory pathway, or from ventricular arrhythmias. Other associated congenital defects may include a secundum ASD or patent foramen ovale (in up to 90 percent of patients with Ebstein anomaly), VSD, PDA, pulmonary outflow obstruction, mitral valve prolapse, or bicuspid aortic valve [21]. (See "Ebstein anomaly: Clinical manifestations and diagnosis", section on 'Associated cardiovascular defects'.)

Cardiac surgical procedures to repair Ebstein anomaly include a variety of procedures, such as tricuspid valve repair or replacement, plication of the atrialized RV, closure of an ASD or other intracardiac shunt, and arrhythmia surgery (surgical ablation of accessory pathways or a Maze procedure to prevent atrial arrhythmias) with or without implantation of pacemaker. In rare instances when RV dysfunction is severe, a bidirectional cavopulmonary anastomosis (ie, Fontan procedure) may be performed. (See "Ebstein anomaly: Management and prognosis", section on 'Surgical or catheter intervention'.)

Management — Intraoperative anesthetic management is discussed here. Surgical management of Ebstein anomaly is discussed further in a separate topic. (See "Ebstein anomaly: Management and prognosis", section on 'Surgical or catheter intervention'.)

●Prebypass management – Due to the likelihood of arrhythmias, it is particularly important to place transcutaneous cardioverter-defibrillator pads on the patient and ensure immediate availability of an external defibrillator before anesthetic induction and sternal incision.

Hemodynamic management goals for patients with Ebstein anomaly are noted in the table (table 1). In particular, a normal to low PVR is maintained to facilitate forward flow in the pulmonary arterial circulation. Hypoxia, hypercarbia, and acidosis are avoided to prevent increases in PVR that may exacerbate RV dysfunction. An inotrope infusion (eg, epinephrine) may be necessary to maintain ventricular contractility and hemodynamic stability after induction of anesthesia since most patients have some degree of RV dysfunction (table 2).

●Postbypass management – An inotrope infusion may also be necessary to achieve weaning from CPB and postbypass hemodynamic stability. Owing to its pulmonary vasodilating effects, milrinone is a good supplemental inotropic agent to support RV function in the postbypass period in patients who do not have hypotension (table 2).

In patients with a newly created cavopulmonary anastomosis, it is particularly important to maintain a low PVR by avoiding hypoxia, hypercarbia, and acidosis. Use of a continuous inhaled pulmonary vasodilator such as nitric oxide or epoprostenol may also be necessary in such patients. (See "Intraoperative problems after cardiopulmonary bypass", section on 'Right ventricular dysfunction'.)

LESIONS REQUIRING REOPERATION OR REDO STERNOTOMY — Adults with congenital heart disease (CHD) often require reoperation to manage residual cardiac lesions or complications. The most common reasons for reoperation include pulmonic regurgitation (treated with surgical or percutaneous valve replacement), right ventricular (RV) outflow tract obstruction, including RV-to-pulmonary artery conduit dysfunction (stenosis of the conduit or stenosis or regurgitation of the valve), pacemaker or defibrillator placement, left ventricular (LV) outflow tract obstruction, or regurgitation (including aortic valve or regurgitation due to root replacement or aneurysm repair), and mitral valve repair or replacement [22].

General considerations for redo sternotomy — Reoperation after previous cardiac surgery requires safe performance of redo sternotomy without damage to underlying cardiac structures that may result in massive bleeding. Computed tomography (CT) scanning or magnetic resonance imaging (MRI) of the chest is typically performed to determine the relationship between the sternum and the underlying cardiovascular structures, including the coronary arteries. If there are adhesions or no space between the sternum and underlying structures, the surgical team typically inserts cardiopulmonary bypass (CPB) cannulae in peripheral blood vessels (eg, a femoral vein and axillary artery) so that CPB can be initiated immediately if necessary. In some cases when CT or MRI imaging predicts a high risk of catastrophic hemorrhage (eg, retrosternal RV aneurysm or dilated ascending aorta adherent to the sternum), CPB is electively initiated via peripheral cannulation in order to decompress the cardiac chambers and permit safer sternal reentry.

The anesthesiology team's preparations and management include:

●Establishing large-bore central venous catheter (CVC) access as well as large-bore peripheral venous access. Notably, obtaining intravascular (IV) access may be challenging in patients who have had multiple previous surgical procedures and other interventions.

In emergency cases, a large-bore IV may be placed after induction for resuscitation. In such cases, large-bore central access is not typically performed by the anesthesia team; rather, the femoral veins are cannulated by the surgical team, and the side port of the venous access sheath is connected to the anesthesiologist's IV lines for infusions.

●Ensuring that blood is present in the operating room and checked prior to surgical incision (eg, four units of packed red blood cells [PRBC] and four units of fresh frozen plasma [FFP]).

●Ensuring immediate availability of a rapid infuser for administration of blood products in case of hemorrhage and emergency need for massive transfusion.

●Preparation of heparin 400 units/kg before surgical incision, so that anticoagulation can be rapidly achieved if emergency initiation of CPB becomes necessary.

●Ensuring immediate availability of the surgeon and perfusionist before induction of anesthesia.

●Performing anesthetic induction and maintaining general anesthesia. Hemodynamic considerations for elective reoperation are similar to those for first-time cardiac surgical procedures in adults with specific CHD lesions, as discussed above. (See "Anesthesia for surgical repair of congenital heart defects in adults: General management", section on 'Anesthetic induction and maintenance'.)

●Maintaining stable hemodynamics with appropriate inotropic and/or vasopressor infusions as needed (table 2). (See "Anesthesia for surgical repair of congenital heart defects in adults: General management", section on 'Management of intraoperative problems'.)

Notably, after completion of a complex redo cardiac surgical procedure, closure of the chest may not be possible due to persistent postbypass bleeding or exacerbation of hypotension when sternal closure is attempted. If necessary, delayed sternal closure can be performed on a subsequent postoperative day. Benefits of this approach include allowing time for myocardial recovery, resolution of tissue edema, and avoiding the risk of postoperative cardiac tamponade. (See "Intraoperative problems after cardiopulmonary bypass", section on 'Inability to close the sternum'.)

Specific redo cardiac surgical procedures

Valve surgery after prior cardiac surgery

Pulmonic valve intervention — Pulmonic valve replacement to treat pulmonic regurgitation (PR) or pulmonic stenosis (PS) is the most frequently performed cardiac surgical reoperation in adults with CHD. Patients with severe symptomatic native pulmonic valve regurgitation generally require surgery, as there are currently limited transcatheter valve replacement options (more commonly performed for prosthetic pulmonic valve regurgitation). By contrast, severe prosthetic pulmonic valve stenosis is commonly treated with transcatheter valve-in-valve implantation, while surgery is generally reserved for patients with complex lesions or associated defects. (See 'Pulmonic valve disease' above and "Pulmonic regurgitation", section on 'Intervention' and "Clinical manifestations and diagnosis of pulmonic stenosis in adults" and "Transcatheter pulmonary valve implantation".)

Further discussion regarding pulmonic valve replacement to treat PR or PS is available in other topics. (See "Pulmonic regurgitation", section on 'Intervention' and "Pulmonic valve stenosis in adults: Management" and "Transcatheter pulmonary valve implantation".)

●Pulmonic regurgitation – PR may cause volume overload of the RV after previous surgery involving the RV outflow tract such as repair of Tetralogy of Fallot (TOF). Chronic RV volume overload due to PR leads to progressive RV failure and consequently impaired LV filling. This progression may be accelerated if coexisting lesions such as tricuspid regurgitation (TR) are present.

Hemodynamic management goals for patients with PR are noted in the table (table 1). In particular, a normal to fast HR is maintained, since a faster HR minimizes regurgitant volume through an insufficient valve. Also, a normal to low pulmonary vascular resistance (PVR) is maintained to facilitate forward flow in the pulmonary arterial circulation. Increasing fraction of inspired oxygen (FiO₂) and mild hyperventilation to maintain a partial pressure of carbon dioxide (PaCO₂) of approximately 30 to 35 mmHg will lower PVR. Hypoxia, hypercarbia, and acidosis are avoided to prevent increases in PVR that may exacerbate RV dysfunction.

●Pulmonic stenosis – Intervention for PS is discussed above. (See 'Pulmonic valve disease' above.)

●Ross procedure – Prosthetic pulmonic valve abnormalities are also common in adult patients who have had a Ross procedure (native pulmonary valve excised and used to replace the native aortic valve [autograft], pulmonary valve generally replaced with homograft) because the homograft valve that was implanted in the pulmonic position, and the autograft implanted in the aortic valve position, usually eventually require replacement for valve dysfunction (regurgitation or stenosis) [23]. Other complications post-Ross procedure include ascending aorta/autograft dilatation and coronary ostial stenosis. (See "Pulmonic regurgitation", section on 'Causes and associated conditions'.)

●Management after pulmonic valve replacement

•After open pulmonic valve replacement – During weaning from CPB, transesophageal echocardiography (TEE) is used to assess for proper positioning of the replaced pulmonic valve and for any paravalvular leak. If present, RV dysfunction requires treatment with an inotrope infusion (eg, epinephrine) to achieve separation from CPB and hemodynamic stability in the postbypass period (table 2). If necessary to treat hypotension, a vasopressin infusion is also employed to increase systemic vascular resistance (SVR) with minimal effects on PVR.

After surgical pulmonic valve intervention, all patients are observed in the intensive care unit for at least 12 to 24 hours to detect development of complications such as pulmonary congestion or edema, hemoptysis, or bradycardia. Some patients manifest lung injury in the postbypass and postoperative period that is similar to reperfusion injury. This is likely due to the sudden increase in pulmonary blood flow that occurs after implantation of a normally functioning pulmonic valve prosthesis. Such patients typically remain intubated with controlled ventilation for a longer time in the postoperative period. In some, noninvasive ventilatory (NIV) support with high-flow nasal cannula (HFNC) oxygenation or bilevel positive airway pressure (BPAP) may be sufficient for recovery.

•After transcatheter pulmonic valve implantation – As noted above, there are limited data on use of transcatheter pulmonic valve implantation for native pulmonic regurgitation. If performed, the position and integrity of the replaced pulmonic valve are checked using fluoroscopy and/or intracardiac, transesophageal, or transthoracic echo after deployment. Although large paravalvular leaks are rare, the valve can be balloon dilated or rarely a second valve-in-valve can be deployed to treat this problem.

Extubation of the trachea can typically be accomplished at the end of a transcatheter procedure, after heparin neutralization and ensuring hemostasis [24]. However, patients must lie supine for up to four hours following femoral sheath removal to avoid bleeding and hematoma formation at the catheter access site. A low-dose dexmedetomidine infusion at 0.2 to 0.5 mcg/kg per minute may be useful to prevent agitation during this early post-procedure period.

Replacement of a conduit — Conduits or prosthetic valves with patch reconstructions performed in childhood, such as those used to reconstruct the RV outflow tract, may need revision in adulthood. For example, development of conduit valve stenosis or regurgitation may result in RV dilation and dysfunction, arrhythmias, or sudden death [25]. Some patients are candidates for a transcatheter procedure such as pulmonic valve replacement, thereby avoiding the need for redo sternotomy. However, other patients require open cardiac surgical intervention due to anatomy that is not favorable for a transcatheter procedure, or the presence of other cardiac abnormalities that require repair or coronary anatomy that predisposes to complications such as coronary compression.

Anesthetic considerations are similar to those for pulmonic valve replacement after other types of previous cardiac surgical procedures. (See 'Pulmonic valve intervention' above.)

Tricuspid valve surgery — Tricuspid valve pathology may be primary (eg, Ebstein anomaly with congenital valve leaflet dysplasia with prolapse (see 'Ebstein anomaly' above)) or secondary (eg, due to RV dilation and dysfunction) [26,27]. Most adults with CHD presenting for tricuspid valve repair or replacement have had previous heart surgery. In particular, patients with Ebstein anomaly may require reoperation for recurrent tricuspid valve disease or to address associated cardiac defects [28]. Hemodynamic goals for patients undergoing tricuspid valve replacement are similar to those for Ebstein anomaly (table 1), and are influenced by concomitant congenital heart lesions.

Reoperation after repair of D-transposition of great arteries

●Reoperation after atrial switch procedures – The Mustard and Senning atrial switch operations were previously the standard interventions to treat complete transposition of the great arteries (TGA). The Mustard operation redirects systemic and pulmonary venous blood using a pericardial baffle, while the Senning procedure uses the atrial septum and wall as a baffle (figure 4) (see "D-transposition of the great arteries (D-TGA): Management and outcome", section on 'Mustard and Senning procedures'). Complications typically develop following these atrial switch procedures including systemic ventricular failure (which is failure of the anatomic RV), arrhythmias, baffle obstruction, and baffle leaks. Reoperation in adulthood may be required for baffle revision for obstruction or leak, systemic atrioventricular (AV) valve regurgitation, and rarely for LV outflow tract obstruction. (See "D-transposition of the great arteries (D-TGA): Management and outcome", section on 'Complications after Mustard and Senning procedures'.)

●Reoperation after arterial switch procedures – The arterial switch (ie, Jatene) procedure, originally performed in 1975, has largely supplanted the atrial switch procedures. For patients who had an arterial switch operation, the LV is the systemic ventricle, and AV synchrony is preserved (figure 5) (see "D-transposition of the great arteries (D-TGA): Management and outcome", section on 'Arterial switch operation (ASO)'). Complications after arterial switch operation may include pulmonary artery stenosis (at the site of previous anastomosis), RV outflow obstruction, coronary artery stenosis, or neo-aortic root dilation with neo-aortic regurgitation. A catheter-based dilation with stent placement may be possible for a patient with pulmonary artery stenosis at the site of the anastomosis, thereby avoiding the need for redo sternotomy. However, selected patients may require open surgical reoperation to treat coronary lesions, RV outflow obstruction, or, in rare cases, neo-aortic regurgitation [29]. Furthermore, regurgitation of the tricuspid valve may necessitate its repair or replacement. In addition, placement or revision of a pacemaker is often needed. (See "D-transposition of the great arteries (D-TGA): Management and outcome", section on 'Complications after ASO'.)

During reoperations after repair of TGA, considerations for anesthetic management include:

●Central venous access may be difficult, and femoral veins are typically used. Notably, it may be difficult to place a pulmonary artery catheter (PAC) due to the anatomy imposed by the original repair.

●TEE is always used during the procedure to assess ventricular function, valvular abnormalities, and baffle anatomy. This should be done by a clinician experienced in imaging patients with complex CHD.

●Maintenance of RV function and stable hemodynamics is achieved with appropriate inotropic and/or vasopressor infusions as needed (eg, epinephrine plus vasopressin if hypotension is present, or milrinone if hypotension is absent (table 2)). Such vasoactive support is usually necessary to achieve separation from CPB.

Cardiac transplantation — Some adult patients with unrepaired or repaired CHD with lesions not amenable to corrective or palliative surgery may be candidates for heart transplantation (see "Heart transplantation in adults: Indications and contraindications", section on 'Indications for transplantation'). These patients should be managed at centers with experience in caring for adult CHD. Anesthetic management is discussed separately. (See "Anesthesia for heart transplantation".)

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

●Repair of congenital heart disease (CHD) lesions in adults – Some individuals with CHD present for surgical correction as adults due to failure to detect a lesion in childhood, lack of resources for repair earlier in life, or more recent development of criteria for repair in adulthood. (See 'Anesthetic management of specific lesions' above.)

●Anesthetic management of specific CHD lesions – Key hemodynamic goals during anesthetic management of adults undergoing repair of specific CHD lesions are summarized above and in the table (table 1):

•(See 'Left-to-right shunt lesions' above.)

•(See 'Coarctation of the aorta' above.)

•(See 'Sinus of Valsalva aneurysm or fistula' above.)

•(See 'Anomalous origin of a coronary artery' above.)

•(See 'Ebstein anomaly' above.)

•(See 'Valve surgery' above and "Anesthesia for cardiac valve surgery".)

Patients with ventricular systolic dysfunction may require infusion of an intravenous inotrope to achieve weaning from cardiopulmonary bypass (CPB) and for postbypass hemodynamic support (table 2). In the immediate postoperative period after coarctation repair, some patients develop paradoxical hypertension, requiring management with nicardipine, esmolol, nitroglycerin, or nitroprusside (table 3).

●Reoperation for CHD lesions – Adults with known CHD often require reoperation to perform a definitive repair or to manage residual cardiac lesions or complications. Reoperation after previous cardiac surgery requires preoperative planning to facilitate safe redo sternotomy without damage to underlying cardiac structures that may result in massive bleeding and acute decompensation. (See 'General considerations for redo sternotomy' above.)

Examples of specific reoperation procedures are:

•Valve surgery after prior cardiac surgery:

-(See 'Pulmonic valve intervention' above.)

-(See 'Tricuspid valve surgery' above.)

•Conduit replacement. (See 'Replacement of a conduit' above.)

•Previous repair of D-transposition of the great arteries. (See 'Reoperation after repair of D-transposition of great arteries' above.)

●Recurrent aortic coarctation. (See 'Recurrent aortic coarctation' 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.