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Anesthesia for labor and delivery in high-risk heart disease: Specific lesions

Anesthesia for labor and delivery in high-risk heart disease: Specific lesions
Literature review current through: Jan 2024.
This topic last updated: Jul 27, 2022.

INTRODUCTION — Management of the parturient with high-risk cardiovascular disease ideally involves a high-risk pregnancy heart team that includes cardiologists, obstetricians, and anesthesiologists to develop an individualized management plan [1-3]. Interdisciplinary communication and preparation are critically important, since peripartum obstetric and cardiac complications may require urgent intervention.

This topic will discuss considerations for anesthetic management of patients with specific acquired and congenital heart lesions during labor and delivery, focusing on methods to minimize peripartum risk while providing optimal anesthetic care. Patients with complex cardiac disease or multiple lesions may have opposing hemodynamic impact (eg, combined mitral stenosis and mitral regurgitation). In such cases, the care team should focus on the specific lesion that is most severe and/or most likely to lead to hemodynamic deterioration.

General considerations for anesthetic management of obstetric patients with high-risk heart disease, including preanesthetic evaluation, monitoring considerations, and risks and benefits of neuraxial and general anesthetic techniques are discussed separately [4]. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations".)

OBSTRUCTIVE LESIONS — For parturients with obstructive cardiac lesions (eg, aortic stenosis [AS], mitral stenosis [MS], aortic coarctation, pulmonic valve or conduit stenosis), hemodynamic goals are to maintain sinus rhythm with a normal heart rate (HR), as well as adequate preload and systemic vascular resistance (SVR) (table 1). Details are provided elsewhere. (See "Anesthesia for noncardiac surgery in patients with aortic or mitral valve disease", section on 'Aortic stenosis' and "Anesthesia for noncardiac surgery in patients with aortic or mitral valve disease", section on 'Mitral stenosis'.)

Specific considerations for anesthetic management during labor and delivery include:

Neuraxial anesthesia – An epidural is placed early in labor to attenuate pain-related catecholamine release that may increase HR and SVR. For cesarean delivery, a very slowly titrated epidural anesthetic or a low-dose sequential combined spinal-epidural is reasonable after insertion of an intra-arterial catheter for continuous blood pressure (BP) monitoring. A phenylephrine infusion is initiated at the time of needle placement and subsequently titrated to keep BP as close to baseline as possible. In patients with severe obstructive lesions, avoid spinal anesthesia due to the possibility of a rapidly induced sympathectomy leading to profound hypotension. (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'.)

General anesthesia – While intravenous (IV) premedication (eg, fentanyl or lidocaine) is generally avoided in obstetric anesthesia because of newborn depression, it is reasonable to administer these agents in selected patients to ensure a hemodynamically smooth induction. Notify the neonatology team if these agents are administered at induction. Avoid administering doses of induction agents that are likely to cause significant tachycardia (eg, ketamine) (see "General anesthesia: Intravenous induction agents", section on 'Ketamine') or vasodilation (eg, a standard bolus induction doses of propofol. (See "General anesthesia: Intravenous induction agents", section on 'Propofol'.)

While a titrated slow induction is generally avoided in obstetric anesthesia, it is reasonable to titrate the induction according to intra-arterial blood pressure monitoring in patients with obstructive cardiac lesions because the risks of hemodynamic alterations are greater than the risk of aspiration.

Notably, adequate anesthetic depth should be established to blunt the sympathetic response to laryngoscopy and intubation. If necessary, a vasopressor such as phenylephrine is administered to minimize or treat hypotension.

Management of complications:

Peripartum hemorrhage – If peripartum hemorrhage occurs, rapid treatment with fluid and blood resuscitation is appropriate, as well as cautious administration of appropriate uterotonic drugs when bleeding is related to atony. However, oxytocin causes a profound decrease in SVR, particularly if administered as an IV bolus dose. Therefore, oxytocin should not be bloused; rather, it is titrated via an infusion pump (eg, at 6 to 15 units/hour) with continuous BP monitoring, and immediate treatment of hypotension with a vasopressor [5]. (See "Anesthesia for the patient with peripartum hemorrhage" and "Postpartum hemorrhage: Medical and minimally invasive management", section on 'Administer additional uterotonic medications'.)

Postpartum pulmonary edema – Patients with severe MS are at particularly high risk for rapid onset of pulmonary edema (ie, "flash" pulmonary edema) immediately after delivery. Contributing factors include:

-Aorto-caval decompression, which allows blood from the lower body to return rapidly to the heart

-Autotransfusion from the contracting evacuated uterus

-Tachycardia and atrial arrhythmias

Although the increased venous capacitance caused by sympathetic blockade with neuraxial anesthesia helps to reduce this risk, the physiologic changes following delivery increase preload and left atrial pressure, which may result in pulmonary congestion. In the awake parturient, this may present as acute dyspnea, hypoxemia, orthopnea, and coughing. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Neuraxial anesthesia for cesarean delivery'.)

Treatment of flash pulmonary edema includes positioning the bed in the head-up position as much as possible and administering supplemental oxygen via mask. In patients with severe symptoms, an IV diuretic (eg, furosemide) is administered. Avoid nitroglycerin infusion in the immediate postpartum period since this may cause uterine atony with a significant increase in obstetric hemorrhage. If severe symptoms persist, it may be necessary to induce general anesthesia, intubate the trachea, and use positive pressure mechanical ventilation.

REGURGITANT LESIONS — For parturients with a regurgitant cardiac lesion (eg, mitral, aortic, tricuspid, or pulmonic valve, or conduit regurgitation), hemodynamic goals include maintaining a normal to fast heart rate (HR), maintaining or decreasing systemic vascular resistance (SVR), and maintaining preload (table 1). Details are provided elsewhere. (See "Anesthesia for noncardiac surgery in patients with aortic or mitral valve disease", section on 'Aortic regurgitation' and "Anesthesia for noncardiac surgery in patients with aortic or mitral valve disease", section on 'Mitral regurgitation'.)

Specific considerations for anesthetic management during labor and delivery include:

Hemodynamic management – The inodilator milrinone is typically used to maintain cardiac output (CO) through a combination of peripheral dilation and increased contractility (table 2). Acute increases in SVR are treated to avoid regurgitant flow and increase forward flow. Nifedipine (a calcium channel blocker), labetalol (a beta-blocker with alpha-blocking activity), hydralazine (a direct arteriolar vasodilator), and methyldopa (a centrally acting alpha-2 agonist) are typically used as these antihypertensive agents have been well-studied in clinical trials for parturients with preeclampsia (table 3). However, a more rapidly acting intravenous (IV) vasodilator is sometimes necessary (table 4). We typically titrate an IV infusion of the calcium channel blocker nicardipine to achieve vasodilation and lower blood pressure (BP) during the antepartum and intrapartum periods (table 5). We initiate the infusion at 5 mg/hour and titrate it up to 15 mg/hour, waiting approximately 10 minutes between dose changes to observe the effects. Infusion of the calcium channel blocker clevidipine is an alternative option if a more rapid and titratable calcium channel blocker is preferred, although this agent has not been well studied in pregnant patients.

Notably, nitroglycerin and nitroprusside are uterine relaxants; thus, discontinuation of these agents after delivery is necessary to avoid uterine atony with resultant postpartum hemorrhage. Furthermore, since nitroprusside may cause fetal cyanide toxicity if administered in high doses over a prolonged time period, this agent is avoided. (See "Acute aortic regurgitation in adults" and "Acute mitral regurgitation in adults".)

Careful afterload reduction with regional anesthesia is beneficial. (See "Overview of neuraxial anesthesia", section on 'Cardiovascular'.)

Considerations for chronic aortic or mitral regurgitation

Neuraxial anesthesia – Epidural labor analgesia is strongly recommended for labor and delivery to attenuate pain-related catecholamine release that may increase SVR. For cesarean delivery, patients with regurgitant lesions and normal ventricular function will usually tolerate either spinal or epidural anesthesia well because the decrease in afterload may beneficially reduce regurgitant volume and increase CO. (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'.)

General anesthesia – Careful titration of propofol is preferred for induction because of its ability to reduce SVR. Dosing of both induction and maintenance anesthetic agents should be adequate to avoid increased BP due to sympathetic stimulation during tracheal intubation and subsequent surgical stimulation.

Considerations for acute aortic or mitral regurgitation – Parturients with acute onset of severe aortic or mitral regurgitation (eg, due to endocarditis, papillary muscle rupture) are unstable and will require tracheal intubation, controlled ventilation, and inotropic and/or vasodilator support. These patients also typically require emergency cardiac surgery, which may be timed with cesarean delivery, depending on gestational age at presentation. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Emergency cardiac surgery with concomitant cesarean delivery'.)

RIGHT-TO-LEFT SHUNTING WITH CYANOSIS — For parturients with cyanotic congenital heart disease and right-to-left shunting (eg, palliated single ventricle, atrial septal defect with pulmonic stenosis, unrepaired tetralogy of Fallot [infundibular or pulmonic valve stenosis with a ventricular septal defect]), hemodynamic goals are to maintain or increase systemic vascular resistance (SVR) while avoiding increases in pulmonary vascular resistance (PVR) (table 1). Details are provided elsewhere. (See "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Right-to-left shunt with cyanosis'.)

Notably, patients with Eisenmenger's syndrome (ie, bidirectional intracardiac shunt and pulmonary arterial hypertension) are at particularly high risk for pregnancy-related morbidity and mortality [6]. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Initiation of extracorporeal membrane oxygenation' and "Pulmonary hypertension with congenital heart disease: Pregnancy and contraception".)

Specific considerations for anesthetic management during labor and delivery include:

Management of intravenous catheters – For any patient with cyanosis related to a right-to-left shunt, filters must be placed on all intravenous (IV) lines as a precaution to prevent venous air embolism and paradoxical arterial air embolism during right-to-left shunting.

Hemodynamic management to minimize right-to-left shunting:

Treat decreased SVR – If a vasoconstrictor is necessary to treat decreased SVR or hypotension, the alpha-adrenergic agonist phenylephrine is administered as boluses or an infusion; norepinephrine may be employed if phenylephrine is ineffective. Although vasopressin is typically preferred in patients with right-to-left shunting since it increases SVR without increasing PVR, this agent may be avoided during pregnancy due to lack of data regarding its effects on uteroplacental perfusion and its similar structure to oxytocin. However, after delivery of the fetus, vasopressin may be the agent of choice (table 2). (See "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Right-to-left shunt with cyanosis'.)

Notably, there is a particularly high potential for decreases in SVR and hypotension immediately after delivery, and during initiation of post-delivery oxytocin therapy, particularly if a bolus dose of oxytocin is administered (see "Anesthesia for cesarean delivery", section on 'Administration of uterotonics'). Therefore, this agent should be titrated via an infusion pump with continuous blood pressure (BP) monitoring, and hypotension is immediately treated with a vasoconstrictor [5]. (See "Pregnancy in women with congenital heart disease: Specific lesions", section on 'Cyanotic congenital heart disease' and "Pregnancy in women with congenital heart disease: General principles", section on 'Cyanosis'.)

Prevent and treat increases in PVR – PaCO2 is maintained at 28 to 30 mmHg during general anesthesia to maintain a low PVR. The uterotonic agent carboprost is avoided as it may cause significant increases in PVR.

Neuraxial anesthesia – An epidural is placed early in labor to attenuate pain-related catecholamine release that may cause tachycardia. For cesarean delivery, a slowly titrated epidural anesthetic or a low-dose sequential combined spinal-epidural is reasonable after insertion of an intra-arterial catheter for continuous BP monitoring. A phenylephrine infusion is initiated at the time of epidural needle placement and subsequently titrated to keep BP as close to baseline as possible. Avoid spinal anesthesia in patients with severe right-to-left shunting because the rapid onset of a sympathectomy and consequent decrease in SVR worsens right-to-left shunting and cyanosis. (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'.)

General anesthesia – Typically, induction is accomplished with etomidate or ketamine combined with an opioid (fentanyl or remifentanil) [7,8]. Avoid anesthetic agents or techniques that decrease SVR (eg, large bolus doses of propofol or high concentrations of an inhalation anesthetic. (See "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Choice of induction agent' and "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Maintenance'.).

LEFT-TO-RIGHT SHUNTNG — For parturients with predominantly left-to-right shunting (eg, atrial septal defect [ASD], ventricular septal defect [VSD], patent ductus arteriosus [PDA]), the emphasis is on maintaining systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR) at normal physiologic pressures (table 1). Details are provided elsewhere. (See "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Left-to-right shunt with pulmonary overcirculation'.)

Compared with cyanotic congenital heart disease with right-to-left shunting, lesions with predominantly left-to-right shunting are typically better tolerated during labor and delivery, particularly in patients without significant pulmonary hypertension or severe impairment of ventricular function. However, marked reductions in SVR should be avoided or treated to avoid reversal of shunt direction to become right-to-left shunting. Vasopressors (eg, phenylephrine or norepinephrine) are administered if necessary.

Specific considerations for anesthetic management during labor and delivery include:

Management of intravenous catheters – As noted above, for any patient with a shunt, filters must be placed on all intravenous lines as a precaution to prevent venous air embolism and paradoxical arterial air embolism since a temporary decrease in SVR could cause shunt reversal resulting in right-to-left shunting. (See 'Right-to-left shunting with cyanosis' above.)

Neuraxial anesthesia – An epidural should be used for labor and delivery. For cesarean delivery, mild reductions in SVR due to a slowly titrated epidural anesthetic or a low-dose sequential combined spinal-epidural are usually well-tolerated.

FONTAN PHYSIOLOGY (CAVOPULMONARY PALLIATION) — For parturients with Fontan physiology (a single ventricle that pumps blood to the systemic circulation, with passive nonpulsatile circulation to the pulmonary arterial system (figure 1) (see "Overview of the management and prognosis of patients with Fontan circulation")), hemodynamic goals are to maintain adequate cardiac output (CO) and forward flow by maintaining preload, myocardial contractility, coronary perfusion, and sinus rhythm, while decreasing pulmonary vascular resistance (PVR) (table 1) [9-14]. Details are provided elsewhere. (See "Anesthesia for adults with congenital heart disease undergoing noncardiac surgery", section on 'Fontan physiology (cavopulmonary palliation)'.)

Specific considerations for anesthetic management during labor and delivery include:

Hemodynamic management – Since Fontan patients are exquisitely sensitive to impaired venous return, it is important to [15]:

Avoid aortocaval compression. Patients should be positioned in left uterine displacement or on their side while laboring when possible [16].

Assess the effects of pushing and the Valsalva maneuver during the second stage of labor. Some obstetricians continuously monitor heart rate (HR), blood pressure (BP), and oxygenation throughout a trial of pushing to assess whether hemodynamic stability is maintained. If hemodynamic compromise is noted, pushing can be modified from typical closed glottic pushing to open glottic pushing.

Less commonly, the obstetrician may try to avoid decreases in venous return by minimizing use of the Valsalva maneuver, opting instead for a "passive second stage of labor." Prior communication with the obstetrician regarding this plan is essential because a passive second stage requires excellent sacral coverage via epidural analgesia.

Avoid hypovolemia. Although we rarely insert a central venous catheter during labor and delivery, if central venous pressure (CVP) is to be monitored, it is maintained at 10 to 15 mmHg to maintain the transpulmonary gradient and facilitate pulmonary blood flow [11,12,17].

If peripartum hemorrhage occurs, rapid treatment with fluid and blood resuscitation is appropriate, as well as administration of uterotonic drugs (eg, oxytocin, or misoprostol) if bleeding is related to atony. Avoid carboprost tromethamine in patients with Fontan circulation since this agent can cause a profound and sudden elevation in PVR. Likewise, methylergonovine is typically avoided since this agent can cause increases in PVR as well as systemic vascular resistance (SVR). (See "Anesthesia for the patient with peripartum hemorrhage" and "Postpartum hemorrhage: Medical and minimally invasive management", section on 'Administer additional uterotonic medications'.)

Avoid spinal anesthesia to avoid a rapidly induced sympathectomy resulting in decreased venous return.

Avoid positive end-expiratory pressure which may decrease venous return and increase PVR. In particular, avoid overinflation of the lungs; treat any evidence of bronchospasm promptly to avoid lung hyperinflation.

Neuraxial anesthesia – Epidural labor analgesia is recommended, particularly if the obstetrician plans to modify the second stage of labor through assisted vaginal delivery or by avoiding, minimizing, or modifying closed glottic pushing (ie, Valsalva maneuver) [15,18]. For cesarean delivery, a very slowly titrated epidural anesthetic or a low-dose sequential combined spinal-epidural is reasonable. Infusion of phenylephrine or norepinephrine is initiated at the time of epidural needle placement and subsequently titrated to keep blood pressure (BP) as close to baseline as possible. As noted above, spinal anesthesia is avoided. (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'.)

General anesthesia – To minimize risk of inducing hemodynamic instability, we slowly titrate the intravenous induction agent (typically etomidate or propofol), rather than proceeding with a rapid sequence induction and intubation. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'General anesthesia technique for cesarean delivery' and "Rapid sequence induction and intubation (RSII) for anesthesia".)

Postpartum complications – Postpartum hemorrhage is the most common obstetric complication in Fontan patients with Fontan physiology. This may be due to uterine atony with lesser use of uterotonic agents (see "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Effects of drugs commonly used in the labor unit'), or underlying chronic liver dysfunction [19,20]. Other common peripartum complications are arrhythmias and heart failure [19,21].

CARDIOMYOPATHIES — Hemodynamic and anesthetic management of parturients with cardiomyopathy varies depending on the type of cardiomyopathy.

Presence of a biventricular pacemaker and/or implantable cardioverter-defibrillator device is common in patients with cardiomyopathy (algorithm 1 and algorithm 2). In patients with a history of poorly tolerated arrhythmias, external defibrillator pads are positioned during labor and delivery in case rapid cardioversion or defibrillation becomes necessary. Further details are described separately. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Management of implantable cardioverter defibrillators and pacemakers' and "Perioperative management of patients with a pacemaker or implantable cardioverter-defibrillator".)

Dilated cardiomyopathy — For parturients with a dilated cardiomyopathy, including peripartum cardiomyopathy, hemodynamic goals are to maintain or increase cardiac output (CO) by maintaining contractility, a normal to fast heart rate (HR), and adequate preload and systemic vascular resistance (SVR) (table 6). Details are provided elsewhere. (See "Acquired heart disease and pregnancy", section on 'Cardiomyopathy' and "Peripartum cardiomyopathy: Etiology, clinical manifestations, and diagnosis" and "Management of heart failure during pregnancy".)

Specific considerations for anesthetic management during labor and delivery include:

Hemodynamic management

Avoid hypovolemia. Peripartum hemorrhage may significantly decrease preload and require fluid and blood resuscitation. However, in a patient with dilated cardiomyopathy, particular care should be taken to avoid fluid overload which exacerbates congestive heart failure (CHF). Administration of phenylephrine or ephedrine may be necessary to maintain adequate BP during volume administration.

Maintain SVR. Avoid spinal anesthesia due to the possibility of a rapidly induced sympathectomy. Notably, hypotension is particularly likely immediately after birth and during initiation of post-delivery oxytocin therapy; thus, oxytocin administration is titrated using an infusion pump [5]. (See "Anesthesia for cesarean delivery", section on 'Administration of uterotonics'.)

Neuraxial anesthesia – An epidural is optimal for labor. For cesarean delivery, a slowly titrated epidural anesthetic or a low-dose sequential combined spinal-epidural is reasonable. A phenylephrine infusion is initiated at the time of epidural needle placement and subsequently titrated to keep blood pressure (BP) as close to baseline as possible. As noted above, spinal anesthesia is avoided. (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'.)

General anesthesia – We prefer induction with etomidate 0.2 to 0.3 mg/kg supplemented with an opioid (eg, remifentanil 1 to 3 mcg/kg, fentanyl 2 to 8 mcg/kg) to avoid a sudden decrease in SVR and subsequent hemodynamic collapse. A reasonable alternative is induction with slowly and carefully titrated propofol administered in combination with norepinephrine or phenylephrine administered as needed to maintain adequate BP. Avoid agents and doses causing myocardial depression (eg, high doses of propofol or volatile anesthetic agents). It may be necessary to administer inotropic agent(s) in a patient with severe left ventricular (LV) systolic dysfunction (table 2). (See "Intraoperative management for noncardiac surgery in patients with heart failure", section on 'Use of vasoactive and inotropic agents' and "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'General anesthesia technique for cesarean delivery'.)

Transesophageal echocardiography (TEE) may be useful during general anesthesia to assess ventricular function as well as volume status. (See "Intraoperative transesophageal echocardiography for noncardiac surgery", section on 'Volume status' and "Intraoperative transesophageal echocardiography for noncardiac surgery", section on 'Ventricular function'.)

Cardiomyopathy associated with preeclampsia — Preeclampsia is associated with peripartum and idiopathic cardiomyopathy, often with diastolic dysfunction that limits the patient’s ability to increase stroke volume (table 7) [22,23]. If pulmonary edema develops, prompt vaginal or cesarean delivery is often indicated since this is the only cure for preeclampsia. Details regarding anesthetic management during labor and delivery are available in a separate topic. (See "Anesthesia for the patient with preeclampsia".)

Hypertrophic cardiomyopathy (HCM) — Hemodynamic goals for parturients with hypertrophic cardiomyopathy (HCM) differ from goals for other types of cardiomyopathy. To avoid worsening left ventricular outflow tract (LVOT) obstruction, increases in myocardial contractility are avoided, while sinus rhythm, preload, and SVR are maintained. Also, maintaining diastolic BP >70 mmHg ensures adequate coronary perfusion of the hypertrophied LV (table 8). Details are provided elsewhere. (See "Anesthesia for patients with hypertrophic cardiomyopathy undergoing noncardiac surgery", section on 'Hemodynamic goals and management'.)

Specific considerations for anesthetic management during labor and delivery include:

Hemodynamic management

Avoid hypovolemia. If peripartum hemorrhage occurs, rapid treatment with fluid and blood resuscitation is appropriate, as well as administration of uterotonic drugs if bleeding is related to atony. (See "Anesthesia for the patient with peripartum hemorrhage" and "Postpartum hemorrhage: Medical and minimally invasive management", section on 'Administer additional uterotonic medications'.)

Avoid decreases in SVR. Avoid spinal anesthesia due to the possibility of a rapidly induced sympathectomy. Hypotension is particularly likely immediately after birth and during initiation of post-delivery oxytocin therapy; thus, oxytocin administration is titrated using an infusion pump [5]. (See "Anesthesia for cesarean delivery", section on 'Administration of uterotonics'.)

Avoid administration of inotropic agents (eg, ephedrine, epinephrine, and norepinephrine (table 2)) to avoid increases in myocardial contractility; likewise, avoid sympathetic stimulation.

Terbutaline (a rapidly acting and short-acting beta-2 agonist) is contraindicated during labor since it may cause exacerbation of LVOT obstruction. Although obstetricians often administer an intramuscular dose of terbutaline 0.25 mg as a rescue treatment for uterine hyperstimulation or tachysystole if fetal distress is present, a hemodynamic crisis due to rapid increase in maternal HR and peripheral vasodilation may occur due to the sudden onset of beta-2 agonism caused by this typical dose of terbutaline.

Neuraxial anesthesia – An epidural is placed early in labor to attenuate pain-related catecholamine release that may increase contractility and HR. The specific technique used for the test dose should be carefully considered. Subsequently, the analgesic block should be sufficiently dense to avoid labor pain. Avoid spinal anesthesia as noted above. (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'.)

For cesarean delivery, a slowly titrated epidural anesthetic or a low-dose sequential combined spinal-epidural is reasonable (after an intra-arterial catheter has been inserted for continuous BP monitoring). However, at the time of intrathecal injection, a phenylephrine infusion is initiated at approximately 0.5 mcg/kg per minute and subsequently titrated to keep the BP as close to baseline as possible. If phenylephrine is ineffective, norepinephrine can be administered. Vasopressin is typically avoided during pregnancy due to absence of data regarding its effects on uteroplacental perfusion and also because of its similar structure to oxytocin. However, after delivery of the fetus, vasopressin can be considered an option (table 2). As noted above, spinal anesthesia is avoided. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Test dose administration'.)

General anesthesia – A reasonable approach is administration of an induction agent (eg, etomidate or propofol) together with low doses of lidocaine [24], and an opioid (eg, fentanyl, remifentanil) (see "Perioperative uses of intravenous opioids: Specific agents", section on 'Fentanyl' and "Perioperative uses of intravenous opioids: Specific agents", section on 'Remifentanil'). Avoid induction agents that cause tachycardia (eg, ketamine) or decrease SVR (eg, high doses of propofol), but establish adequate anesthetic depth to minimize sympathetic stimulation and tachycardia during laryngoscopy and intubation. Beta blockers (eg, esmolol, labetalol, metoprolol) are often used to control HR and beneficially decrease contractility, particularly during induction and intubation, and also during emergence and extubation [25]. (See "Anesthesia for patients with hypertrophic cardiomyopathy undergoing noncardiac surgery", section on 'Induction'.)

During general anesthesia, TEE monitoring is particularly useful for continuous assessment of volume status, LVOT obstruction, LV cavity size and function, presence of mitral regurgitation, and global or regional myocardial ischemia, particularly if hemodynamic instability develops. (See "Intraoperative rescue transesophageal echocardiography (TEE)", section on 'Left ventricular outflow tract obstruction'.)

Pulmonary hypertension and/or right heart failure — Severe pulmonary arterial hypertension (PAH) carries a high risk of maternal mortality because the increased CO required to sustain pregnancy may not be accommodated in a patient with fixed pulmonary vascular resistance (PVR) and right heart failure [26]. As CO increases during the first trimester, patients with severe PAH may begin to decompensate and become hypoxemic. In some cases, delivery may become necessary prior to completion of a full-term pregnancy (or dilation and curettage or dilation and evacuation procedures may be performed during the first trimester) [27]. The postpartum period is the highest risk for patients with severe pulmonary hypertension, and these patients require close monitoring following delivery. In patients with peripartum acute pulmonary hypertensive crisis, invasive monitoring is typically employed (eg, intra-arterial catheter, pulmonary artery catheter) to guide therapy. In rare cases, cardiopulmonary resuscitation and extracorporeal membrane oxygenation (ECMO) may become necessary during pregnancy or the peripartum period [27]. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Mechanical circulatory support in pregnancy' and "Pulmonary hypertension with congenital heart disease: Pregnancy and contraception", section on 'Management of pregnancy'.)

Details regarding anesthetic management of patients with pulmonary hypertension and right heart failure are discussed separately (table 1). (See "Anesthesia for noncardiac surgery in patients with pulmonary hypertension or right heart failure".)

ISCHEMIC HEART DISEASE — For parturients with ischemic heart disease, hemodynamic goals are to optimize myocardial oxygen supply and minimize myocardial oxygen demand by maintaining a normal heart rate (HR), as well as adequate preload and systemic vascular resistance (SVR) (table 9 and table 10). Details are provided elsewhere. (See "Anesthesia for noncardiac surgery in patients with ischemic heart disease", section on 'Anesthetic goals'.)

Specific considerations for anesthetic management during labor and delivery include:

Neuraxial anesthesia – An epidural is placed early in labor to attenuate pain-related catecholamine release that may increase HR. For cesarean delivery, either spinal or epidural anesthesia is usually tolerated if hypotension is avoided or treated promptly. (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'.)

General anesthesia – Avoid induction agents that cause tachycardia (eg, ketamine) or vasodilation (eg, high bolus doses of propofol), but establish adequate anesthetic depth to minimize tachycardia due to sympathetic stimulation during laryngoscopy and intubation. Beta blockers are often used to avoid or treat tachycardia and hypertension during laryngoscopy and subsequent surgical stimulation, and/or during emergence and tracheal extubation. Some clinicians use the short-acting opioid remifentanil as a supplemental agent during and after induction to avoid tachycardia without risk of causing prolonged neonatal respiratory depression. (See "Perioperative uses of intravenous opioids: Specific agents", section on 'Remifentanil' and "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'General anesthesia technique for cesarean delivery'.)

Management of complications:

Peripartum hemorrhage – If peripartum hemorrhage occurs, rapid treatment with fluid and blood resuscitation is appropriate, as well as cautious administration of uterotonic drugs if bleeding is related to atony. (See "Anesthesia for the patient with peripartum hemorrhage" and "Postpartum hemorrhage: Medical and minimally invasive management", section on 'Administer additional uterotonic medications'.)

Acute myocardial infarction – In rare cases, parturients with acute myocardial infarction may require emergency cardiac surgery and concomitant cesarean delivery. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Emergency cardiac surgery with concomitant cesarean delivery'.)

AORTIC ANEURYSM OR DISSECTION — For parturients with a dilated ascending aorta and those at risk for aortic dissection (eg, Marfan syndrome), hemodynamic goals are to maintain a normal heart rate (HR) and blood pressure (BP) and adequate preload, while avoiding increases in systemic vascular resistance (SVR) or myocardial contractility (table 11). Details are provided elsewhere. (See "Heritable thoracic aortic diseases: Pregnancy and postpartum care", section on 'Delivery'.)

Marfan syndrome — Specific considerations for anesthetic management during labor and delivery include:

Monitoring – Throughout labor and delivery, continuous monitoring of pulse oximetry with a visible waveform, 5-lead electrocardiogram, and BP via an intra-arterial catheter should be employed. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Monitoring' and "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Intravascular catheters'.)

Hemodynamic management – The priority is control of BP and HR throughout labor and delivery.

Continue beta blockers initiated during pregnancy to maintain slow to normal HR and to prevent increases in BP.

Treat increases in BP or myocardial contractility (eg, due to sympathetic stimulation) with:

-Intravenous (IV) beta blockers. Esmolol has the advantage of a short half-life, enabling titration to effect. Other reasonable choices include labetalol or metoprolol (table 5).

-If necessary for acute management to control BP, a vasodilator (eg, nitroglycerin, nicardipine, clevidipine) can be administered (table 5). However, nitroglycerin and nitroprusside are uterine relaxants that can cause uterine atony and resultant postpartum hemorrhage; thus, discontinuation after delivery is necessary. Furthermore, nitroprusside is typically avoided as it may cause fetal cyanide toxicity. (See "Heritable thoracic aortic diseases: Pregnancy and postpartum care".)

Neuraxial anesthesia – An epidural is placed early in labor prior to significant pain-related catecholamine release which would increase BP and aortic wall stress. Thereafter, the analgesic block should be sufficiently dense to avoid sympathetic stimulation from labor pain. Notably, the presence of dural ectasia in a patient with Marfan’s syndrome should be considered (image 1) [28]. Although dural ectasia is not a contraindication for epidural anesthesia, risk of dural puncture or inadequate anesthesia is more likely due to the increased volume of cerebrospinal fluid [29,30]. Performing the epidural technique in the lateral decubitus position may reduce risk for extension of the ectatic dural sac into the epidural space [29]. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders", section on 'Dural ectasia' and "Heritable thoracic aortic diseases: Pregnancy and postpartum care", section on 'Delivery'.)

Furthermore, the specific technique used for the test dose should be carefully considered. (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 'Test dose administration'.)

For cesarean delivery, spinal, epidural, or low-dose sequential combined spinal-epidural anesthesia can be used with continuous monitoring of intra-arterial BP. Allowing the BP to drop as much as 20 percent below baseline after neuraxial anesthesia is reasonable. However, lower BP may result in uteroplacental insufficiency and fetal distress. A phenylephrine infusion can be titrated to prevent and treat more severe decreases in BP. (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'.)

General anesthesia – Induction with propofol supplemented with adjuvant agents (eg, an opioid such as fentanyl or remifentanil plus lidocaine) is reasonable. Ketamine is contraindicated because of its sympathomimetic effects. Subsequently, adequate depth of anesthesia is maintained with a volatile anesthetic agent. Beta blockers and/or vasodilators may be used to control BP (table 5). (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'General anesthesia technique for cesarean delivery'.)

During general anesthesia, transesophageal echocardiography (TEE) monitoring is useful to localize any aortic intimal tear and resultant valvular dysfunction (ie, acute aortic regurgitation) or myocardial ischemia. (See 'Acute aortic dissection' below.)

During emergence and tracheal extubation, hypertension may lead to aortic dissection. Risk is lessened by optimizing perioperative analgesia and by administering beta blockers and/or vasodilating agents.

Acute aortic dissection — If ascending aortic dissection occurs during pregnancy, labor, or delivery, immediate cardiovascular surgical intervention is usually warranted because of the high risk of maternal mortality [31,32]. Management of the fetus depends on the stage of pregnancy; emergency cardiac surgery with concomitant cesarean delivery is often preferred due to the high risk of fetal loss during complex aortic surgery. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations", section on 'Emergency cardiac surgery with concomitant cesarean delivery'.)

Anesthetic management includes:

Ensure large-bore intravascular access and a functioning intra-arterial catheter. Also, plan insertion of a central venous catheter for administration of intravenous vasoactive drugs and to provide large-bore secure intravascular access for volume resuscitation.

Administer preoperative and intraoperative pharmacologic therapy to control BP until the patient is on cardiopulmonary bypass. Initial treatment is with IV beta blockers (eg, esmolol, labetalol, metoprolol) to reduce HR to <60 beats/minute, reduce systolic BP, and minimize aortic wall stress (table 5). If systolic BP remains >100 mmHg after beta blockade, vasodilators (eg, nitroglycerin, nicardipine, clevidipine) are administered to achieve control.

Further details regarding management of acute aortic dissection are discussed in other topics. (See "Overview of acute aortic dissection and other acute aortic syndromes", section on 'Acute medical management' and "Management of acute type B aortic dissection", section on 'Anti-impulse therapy' and "Heritable thoracic aortic diseases: Pregnancy and postpartum care", section on 'Management of aortic dissection' and "Management of acute type A aortic dissection", section on 'Acute management'.)

PREVIOUS HEART TRANSPLANTATION — For parturients who have undergone heart transplantation prior to becoming pregnant, loss of cardiac autonomic enervation results in pharmacologic and hemodynamic changes. Notably, these parturients are extremely dependent on adequate intravascular volume and preload. Furthermore, direct-acting vasopressor agents to treat hypotension (eg, phenylephrine) are effective, but indirect agents that exert their effects via the autonomic nervous system (eg, ephedrine) are ineffective in the denervated heart. In addition, ventricular dysfunction and ischemic heart disease are often present (see 'Ischemic heart disease' above). Details are provided elsewhere. (See "Anesthetic considerations after 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: Perioperative cardiovascular evaluation and management" and "Society guideline links: Obstetric anesthesia".)

SUMMARY AND RECOMMENDATIONS

Specific cardiac lesions – Symptomatic or severe acquired or congenital heart lesions requiring specialized anesthetic management during labor and delivery include:

Obstructive lesions – Examples include aortic stenosis, mitral stenosis, aortic coarctation, pulmonic valve or conduit stenosis (table 1). (See 'Obstructive lesions' above.)

Regurgitant lesions – Examples include acute or chronic mitral, aortic, tricuspid, or pulmonic valve regurgitation, or conduit regurgitation (table 1). (See 'Regurgitant lesions' above.)

Cyanotic congenital heart disease with right-to-left shunting – Examples include palliated single ventricle, atrial septal defect (ASD) with pulmonic stenosis, Eisenmenger syndrome, and unrepaired tetralogy of Fallot (table 1). (See 'Right-to-left shunting with cyanosis' above.)

Left-to-right shunting – Examples include atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA) (table 1). (See 'Left-to-right shuntng' above.)

Fontan physiology – Fontan physiology involves a single ventricle that pumps blood to the systemic circulation, with passive nonpulsatile circulation to the pulmonary arterial system (figure 1 and table 1). (See 'Fontan physiology (cavopulmonary palliation)' above.)

Cardiomyopathies – Hemodynamic and anesthetic management of parturients with cardiomyopathy varies depending on the type of cardiomyopathy:

-Dilated cardiomyopathy (table 6) – (See 'Dilated cardiomyopathy' above.)

-Cardiomyopathy associated with preeclampsia – (table 7) (See 'Cardiomyopathy associated with preeclampsia' above.)

-Hypertrophic cardiomyopathy (table 8) – (See 'Hypertrophic cardiomyopathy (HCM)' above.)

-Pulmonary hypertension with right heart failure (table 1) – (See 'Pulmonary hypertension and/or right heart failure' above.)

Ischemic heart disease (table 9 and table 10) – (See 'Ischemic heart disease' above.)

Aortic aneurysm or dissection – (table 11)

-Marfan syndrome – (See 'Marfan syndrome' above.)

-Acute aortic dissection – (See 'Acute aortic dissection' above.)

Previous heart transplantation (table 1) – (See 'Previous heart transplantation' above.)

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Topic 93919 Version 35.0

References

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