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Wednesday, April 27, 2011

Management of the Parturient With Cardiovascular Disease


Management of the Parturient With Cardiovascular Disease
Lisa M. Councilman, M.D. Temple, Texas

Introduction
The incidence of clinically significant cardiac disease in the pregnant population ranges from 0.1-4%,
unchanged in decades; however, the most frequently seen etiology is now congenital heart disease (70-80%)1 due in part to advances in surgical techniques for these patients and advances in medical therapy, allowing these women to survive into childbearing age.2 Ischemic heart disease is also seen more commonly today due to both the increasing number of women of advanced maternal age who are electing to undergo pregnancy and childbirth as well as advances in medical therapy for ischemic heart disease, allowing women with this condition to carry a pregnancy to term. While the incidence of cardiac disease in pregnant patients has remained relatively unchanged, the maternal mortality from cardiac disease has decreased from 6% in the 1930s to 0.5-2.7% today.1 The last decade has shown a decline in maternal mortality from congenital heart disease, and now acquired heart disease has risen to be the leading cardiac cause of maternal death, with myocardial infarction, aortic dissection, and cardiomyopathy as the main processes.3 Unfortunately, the  cardiovascular changes of pregnancy may place additional stress on patients with underlying cardiac disease, increasing the risk of peripartum morbidity and mortality when compared with the general population, with the actual risk depending on the underlying cardiac disease process.1,2 Taking the altered physiologic processes into account, women with congenital heart disease and those with a history of ischemic heart disease require special attention and a multidisciplinary cooperation for optimal outcome during vaginal delivery or
cesarean section. The decision to perform regional or general anesthesia will ultimately depend on a thorough
understanding of the cardiac condition and condition-specific hemodynamic goals.2



In the United States between 1991 and 1999, 34% of maternal deaths that were attributable to medical
conditions other than embolism, hemorrhage, and pregnancy-induced hypertension (the leading causes of death) were from cardiovascular problems.4 During this same time frame the percentage of maternal deaths attributable to cardiomyopathy increased from 6% to 9%.4 Seventy percent of maternal deaths from cardiomyopathy between 1991 and 1997 were from peripartum cardiomyopathy (PPCM), a form of dilated cardiomyopathy.5 PPCM may present itself unexpectedly in the otherwise healthy parturient and may quickly become life-threatening, with exceedingly high rates of morbidity and mortality. Early recognition of the signs and symptoms of PPCM is critical, with rapid diagnosis and treatment crucial. Even in the best of circumstances maternal mortality is a very real concern in the presence of PPCM.
Congenital Heart Disease
Congenital heart disease is now the major etiology of cardiac disease in pregnant women in the United
States, occurring in 60-80% of cases.6 Many more women with congenital heart disease are now reaching childbearing age, giving rise to the need for novel approaches to labor analgesia and anesthesia for cesarean section in this high-risk population. There are now over one-half million American adult women with congenitally malformed hearts.7 Many of the women with congenital heart disease have undergone successful surgery to correct the cardiac defects, however some women may present with uncorrected or only partially-corrected lesions, putting them at risk for serious adverse events during labor and delivery.6 Management of these patients may present some interesting challenges for both the obstetrician and the anesthesiologist, and it is imperative to have the involvement of an adult congenital cardiologist and neonatologist, as well.6,7
Patients with existing small left-to-right shunts often tolerate pregnancy quite well. This category of
patients includes those with small atrial septal defects, small ventricular septal defects, or patent ductus arteriosus.
Anesthetic management of patients with left-to-right shunts needs to include attention to several important factors. Care must be taken to remove all air bubbles from the intravenous tubing to avoid systemic air embolization and epidural needle placement should be performed using a loss-of-resistance to saline rather than air to avoid systemic air emboli.6 Epidural anesthesia is preferred in these patients to avoid maternal hypoxemia, pulmonary hypertension, and right heart failure that can occur with an increase in the left-to-right shunt fraction. Increased shunting may be seen with the increased maternal systemic vascular resistance (SVR) that occurs with increased maternal catecholamine production in response to painful contractions.6 Early epidural analgesia may help to blunt this response by preventing the initial increase in catecholamine secretion.6 The epidural must be titrated slowly to prevent a rapid decrease in SVR which could reverse the shunt and cause maternal hypoxemia.6 Maneuvers to prevent an increase in pulmonary vascular resistance (PVR) should be implemented such as providing supplemental oxygen and avoiding hypercarbia and acidosis. An increase in PVR may lead to a reversal of the shunt, forming a right-to-left shunt that will lead to a worsening of the hypoxemia.6
Tetralogy of Fallot accounts for 5% of congenital heart disease in pregnant women.6,8 The uncorrected
lesions produce a right-to-left shunt which leads to cyanosis. Most patients will have undergone definitive surgical correction prior to their pregnancy but may have various anatomic and physiologic alterations requiring early recognition such as right ventricular dysfunction, pulmonary stenosis and/or regurgitation, and both atrial and ventricular dysrhythmias.6,8,9,10 Although these women may appear healthy, they are still considered a high-risk
group of parturients. These anatomic and physiologic alterations including right ventricular dysfunction and
tachydysrhythmias may present themselves symptomatically during pregnancy as the cardiovascular changes of
pregnancy progress. Patients with a tetralogy of Fallot repair should have echocardiograms performed prior to and
during pregnancy to identify evolving ventricular dysfunction. Anesthesia for asymptomatic patients with a
definitive tetralogy of Fallot repair will most likely not differ from those patients without this condition with the
exception of the need for an antepartum 12-lead electrocardiogram (ECG) and continuous ECG monitoring during labor and delivery to enable rapid identification of dysrhythmias.6,9 Fernandez and Kuczkowski describe the safe use of spinal anesthesia for cesarean delivery in a patient with a corrected tetralogy of Fallot.9 Patients with uncorrected or partially corrected tetralogy of Fallot require much greater attention. Pregnancy in these patients carries a major risk of heart failure, arrhythmia, and endocarditis.10 It is important to maintain adequate intravascular volume, venous return, and high right ventricular filling pressures to ensure adequate right ventricular and pulmonary blood flow.12 Effective labor analgesia is important to prevent exacerbations of the right-to-left shunt, caused by an increase in PVR such as pulmonary hypertension, right ventricular dysfunction, and cyanotic shunting.8 Neuraxial anesthesia containing local anesthetics must be used with extreme caution in these patients since the decrease in SVR associated with neuraxial administration of local anesthetic will worsen the right-to-left shunt, leading to hypoxemia.6 A single-shot spinal anesthetic for cesarean section has been considered relatively contraindicated because a precipitous decrease in SVR from the local anesthetic may lead to a worsening of the right-to-left shunt and progressive hypoxemia.8 Langesaeter et al demonstrated the reduction in SVR associated with spinal anesthesia could be blunted by cohydration with crystalloids and a concurrent infusion of phenylephrine guided by invasive monitoring and recommend this technique be considered for parturients with high-risk cardiac disease rather than expose them to the hazards of general anesthesia.11
Coarctation of the aorta is a congenital lesion more common in males than females and does not require
any special anesthetic management if surgically corrected and arm-to-leg blood pressure gradient is less than 20mm Hg.6,13 Having an uncorrected coarctation of the aorta during pregnancy places the patient at risk for left ventricular failure, aortic rupture or dissection, and endocarditis.6 Aortic rupture is more likely to occur during the third trimester.1,14 The added stress of the physiologic changes of pregnancy result in a maternal mortality rate of 3% in patients with an uncorrected aortic coarctation.1,14 These patients have a fixed obstruction to aortic outflow and distal hypoperfusion, manifested by a difference between left-sided and right-sided blood pressures, upper extremity and lower extremity blood pressures, and may present with hypertension of an unknown etiology.8 Hypotension will compromise not only the maternal myocardium, but also the placental blood flow to the fetus.14 The hemodynamic goals in the patient with an uncorrected aortic coarctation include maintaining normal to slightly elevated SVR, normal to slightly increased heart rate, and adequate intravascular volume, with invasive blood pressure monitoring being helpful in maintaining these goals.8 Postductal arterial pressures (left radial) may be a better indicator of uterine perfusion pressure than preductal arterial pressures (right radial).8 Postductal systolic blood pressure should be maintained greater than 100 mmHg in order to avoid compromising uterine blood flow.13
Neuraxial anesthesia for labor should be administered with great caution since agents leading to a decrease in SVR can be devastating.12 Fetal mortality approaches 20% due to the inability to provide adequate uterine perfusion.12
General anesthesia is generally recommended for cesarean section, however several reports in the literature describe the safe use of epidural and combined spinal-epidural techniques for cesarean section in patients with aortic coarctation, both with and without prior palliative surgery.13,14 Remifentanil has been used successfully for general anesthesia in parturients with aortic coarctation, facilitating the maintenance of hemodynamic stability with minimal neonatal respiratory depression.8 Ephedrine and dopamine are the vasopressors of choice in patients with uncorrected aortic coarctation with their ability to maintain SVR and heart rate.8,12
Marfan’s syndrome is an autosomal dominant condition affecting the skeletal, ocular, neurologic,
pulmonary, and cardiovascular systems.15 Ninety-five percent of mortality in patients with Marfan’s syndrome is cardiovascular in origin and the mean survival age is 32 years.15 All parturients with Marfan’s syndrome are considered high-risk, as aortic dilatation and dissection can occur with the increase in blood volume and cardiac output as the pregnancy progresses. Aortic dissection occurs even in the absence of preconception aortic root dilatation, mainly during the third trimester.15 Neuraxial analgesia for labor has been shown to be safe, as well as general anesthesia for cesarean section. Of greatest importance is reducing the shear force from blood ejected by the left ventricle, which may be attenuated by ß-adrenergic blockade therapy in order to decrease the cardiac rate and contractility.15 Phenylephrine would appear to be the vasopressor of choice in parturients with Marfan’s syndrome, as ephedrine produces the untoward side effect of tachycardia.15
Ischemic Heart Disease
The incidence of myocardial infarction (MI) during pregnancy is on the rise as more women with multiple
risk factors for ischemic heart disease are becoming pregnant.8 The incidence is now estimated at 1:10,000
deliveries, accounting for nearly 2000 deaths annually in women under age 45.8 Risk factors for ischemic heart disease in parturients are: 1) increasing maternal age, as the number of women finishing their education or beginning their careers before starting a family is increasing, 2) increasing number of women smokers, 3) increasing incidence of cocaine use in women of childbearing age, 4) experiencing stress in the workplace, 5) oral contraceptive use after the age of 35, and 6) increased prevalence of obesity and type II diabetes. 1,6,8,16 The maternal mortality rate of peripartum MI has decreased in recent years to 5.1-7.3%.6 If the MI occurs within two weeks of delivery, mortality may be as high as 45%-50%.16,17
Myocardial ischemia during pregnancy may be attributable more to coronary vasospasm than to coronary
artery disease.1 Coronary atherosclerosis is found in less than half of patients who have an MI during pregnancy.8
Other etiologies of ischemia in this population include coronary artery injury from vasospasm, dissection, aneurysm, or hematoma; severe hypertension from pregnancy-induced hypertension, pheochromocytoma or cocaine use; severe tachycardia in patients with left ventricular hypertrophy, hypotension, and anemia; and severe aortic stenosis.6,8 Svanstrom et al18 studied the iatrogenic etiology of myocardial ischemia after injection of intravenous oxytocin in otherwise healthy women undergoing cesarean section. Symptoms they observed included hypotension,
tachycardia, chest pain, flushing, dyspnea, and ECG changes consistent with myocardial ischemia. Kulka et al19 describe a 31-year-old patient who developed an MI following induction of spinal anesthesia for a cesarean section, after aggressive treatment of hypotension led to significant hypertension and tachycardia. Coronary angiography in  that patient revealed normal coronary vessels, however intravascular ultrasound demonstrated an atheroma in the left main coronary artery with a ruptured fibrous cap.
Diagnosis of myocardial ischemia in pregnant patients may be challenging since the symptoms of ischemia
mimic complaints seen in the normal pregnant patient. These presenting complaints may include dyspnea,
diaphoresis, poor exercise tolerance, chest pain, and syncope. Electrocardiogram changes seen in myocardial
ischemia mimic ECG changes which occur in normal pregnancy including sinus tachycardia, a left axis deviation, ST-segment depression, flattened or inverted T waves, and a Q wave in lead III, making a diagnosis of myocardial ischemia by ECG difficult, with only serial ECG changes meaningful.6 Holter monitoring and echocardiography are noninvasive diagnostic tools that remain useful during pregnancy.6
The anesthetic management of patients who have experienced an MI during pregnancy is challenging,
requiring careful control of cardiovascular parameters utilizing invasive monitoring and optimizing the myocardial oxygen supply-to-demand ratio. Supplemental oxygen should be provided throughout labor and delivery.6,8
Tachycardia both decreases the oxygen supply to the myocardium and increases the oxygen demand, and should be prevented or aggressively treated with adequate pain control and ß-adrenergic blockade as necessary. Neuraxial anesthesia, if no contraindications exist, will provide excellent pain relief, prevent hyperventilation, and reduce maternal concentrations of catecholamines, the goal of which is preventing coronary artery vasoconstriction. 6,17 A dense epidural block for labor analgesia has the benefits of providing excellent pain relief during the first stage of labor, minimizing the maternal expulsive efforts during the second stage of labor (which otherwise greatly increases myocardial oxygen demand) and allowing for a comfortable forceps-assisted delivery, and allowing the rapid extension of anesthesia should the need arise for an urgent or emergent cesarean section.6,8 Epinephrine should be avoided in the epidural test dose and anesthetic solutions to avoid hypertension and tachycardia if inadvertent intravascular injection occurs. Phenylephrine is the vasopressor of choice for maternal hypotension in patients with ischemic heart disease.6 Ephedrine should be avoided to prevent tachycardia, undesirable in patients at risk for myocardial ischemia. If the patient with ischemic heart disease requires a cesarean section, care must be taken during the history and physical examination to document all medications and treatments the patient has received during the pregnancy. Drug-eluting stents have been used in the treatment of parturients with critical coronary heart
disease. These particular stents require the patient to be placed on combination clopidogrel and aspirin therapy, and it is imperative that the patient not discontinue use of these medications for a period of up to twelve months, depending on the type of stent used. Concomitant or recent use of anticoagulants or antiplatelet drugs impacts the anesthetic options available for patients in labor or requiring a cesarean section, possibly contraindicating neuraxial anesthesia.16,20 Neuraxial anesthesia is absolutely contraindicated with ongoing clopidogrel use. Intravenous opioids for labor and general anesthesia for a cesarean section are the extent of options available in patients currently anticoagulated. Cuthill et al21 describe such a case of a parturient who received a sirolimus-eluting stent for severe ostial left main coronary artery stenosis. The patient was placed on combination anticoagulant therapy consisting of clopidogrel, aspirin, and enoxaparin, and labetalol was prescribed. A nitroglycerine patch was applied preoperatively and general anesthesia was performed for her cesarean section without any adverse events utilizing an arterial line and central venous catheter for intraoperative hemodynamic monitoring. Careful use of oxytocin is necessary post-delivery to avoid profound vasodilatation and compensatory tachycardia, which results in reduced coronary diastolic filling, leading to myocardial ischemia.20
Peripartum Cardiomyopathy
Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy that has an onset during a six-month
time frame including the last month of pregnancy to five months postpartum.5,8,22,23 Preexisting heart disease must be excluded, no determinable etiology can be present, and echocardiographic criteria must be met including a dilated cardiomyopathy with a decreased left ventricular ejection fraction (LVEF) of less than 45% or a fractional shortening of less than 30%, and a left ventricular end-diastolic dimension of greater than 2.7 cm/m2. 5,8,23
Echocardiography is the gold standard for the diagnosis of PPCM.24 Risk factors for PPCM include advanced maternal age, multiparity, multiple gestations, black race, obesity, gestational hypertension, preeclampsia, cesarean section, alcohol, cocaine, and tobacco abuse.22 Initial presenting symptoms for PPCM may be limited to symptoms of a mild upper respiratory infection, dyspnea, chest congestion, palpitations, and fatigue.6,8,22,23 These early symptoms may emulate the normal changes seen with pregnancy and may be difficult to distinguish as abnormal. These symptoms can rapidly progress to florid cardiac failure with biventricular hypokinesis, low cardiac output, elevated filling pressures, and ventricular ectopy.6 Fussell et al describe a five-week postpartum patient who presented with fulminant hepatic failure one week after becoming symptomatic from an unrecognized PPCM.25
PPCM is a significant cause of maternal morbidity and mortality. Recent studies have shown a mortality
rate of 9% and a cardiac transplantation rate of 14%, with a normalization of ejection fraction within six months in 50% of surviving women; however if no substantial improvement is observed during the initial six months, the mortality rate is 85% in five years. having.6 Women with a history of PPCM and normal systolic function will have a relapse rate of 20% with a subsequent pregnancy, whereas women with a history of PPCM and residual left ventricular dysfunction have a relapse rate of 50% and a mortality rate of 8% to 17% with a subsequent pregnancy.8,22 Sliwa et al26 followed six patients through subsequent pregnancies after having had PPCM with their prior pregnancy. They found a reduction in the ejection fraction by greater than 10% in five of the six patients at one month postpartum and two women of the five who had impaired left ventricular function at the onset of pregnancy died within three months postpartum despite optimal medical therapy, confirming that mortality during subsequent pregnancies is high, especially in patients with persistent left ventricular dysfunction. Whitehead et al27 reviewed 171 deaths related to PPCM from 1991 through 1997 and noted that African American women were 6.4
times as likely to die from PPCM than Caucasian women, and women 35-years-old and older had a risk of death 2.8
times greater than that of women aged 19-years and younger. Other risk factors for mortality from PPCM noted in
their study included twin or greater gestations and parity greater than three.
Medical management in the parturient with PPCM does not differ from the management of other patients
with severe cardiomyopathy. Diuretics, vasodilators, and digoxin, as needed, should be initiated with careful
attention paid to fetal safety and to excretion of the drug or its metabolites in breast milk.22,28 Angiotensinconverting
enzyme inhibitors, although contraindicated during pregnancy because of teratogenicity, should be
considered a mainstay of treatment postpartum.28,29 Hydralazine and nitrates are safe alternatives during
pregnancy.23,28,29 The addition of bromocriptine appeared to improve LVEF in one recent small study by Sliwa et
al.30 Amlodipine may have a role in the treatment of PPCM as it has been shown to improve survival in
nonischemic cardiomyopathy patients.21 Beta-adrenergic antagonists may be used in the postpartum period in
patients who have been refractory to other therapy and continue to have left ventricular dysfunction for more than
two weeks after standard heart failure therapy.28 Thromboembolic events are not uncommon, therefore
anticoagulation may become necessary, especially in patients with a LVEF less than 35% or bedridden patients with
atrial fibrillation, mural thrombi, obesity, or a history of thromboembolism.22,29 Rarely, patients with PPCM fail
medical therapy and require an intra-aortic balloon pump or ventricular assist device for cardiovascular support.23
These mechanical devices are considered a bridge until cardiac transplantation is able to be performed in suitable
transplant candidates.23,24,31 The survival after cardiac transplant for PPCM is 75% at four years and does not differ
from the survival rate of women who undergo cardiac transplantation for other forms of cardiomyopathy,23 therefore
cardiac transplantation is a successful option for the treatment of severe, refractory PPCM.24
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Anesthetic management in the parturient with PPCM is not significantly different from that of other
patients with a severe cardiomyopathy. Invasive monitoring is warranted in the acute setting until cardiac function
has stabilized. Coagulation status should be normalized prior to the performance of neuraxial anesthesia. Cesarean
sections have been performed safely in parturients with PPCM utilizing both general and regional anesthesia
techniques. Continuous neuraxial anesthesia is usually preferred as it decreases preload and afterload but not
contractility, improving myocardial performance and reducing myocardial work.8 Velickovic and Leicht describe
the successful use of continuous spinal anesthesia in a parturient with severe recurrent PPCM.32 When general
anesthesia is necessary, extreme caution must be exercised in choosing drugs which do not depress the myocardium,
as myocardial depression may precipitate cardiac arrest and death.8 A propofol and remifentanil combination has
been described for use in general anesthesia for cesarean section in a patient with PPCM, providing good
cardiovascular stability throughout the procedure.33
Summary
Encountering a patient with cardiovascular disease in the labor and delivery unit can be a harrowing
experience. The patient’s likelihood of developing complications including death is increased, something we do not
deal with on a frequent basis in most labor and delivery units. However, understanding the underlying physiologic
processes will assist the anesthesiologist in the preparation and delivery of safe anesthetic care for the parturient
with cardiovascular disease. Additional non-invasive monitoring including electrocardiography and pulse oximetry
should be considered for high-risk patients undergoing vaginal delivery. Invasive monitoring with an arterial line
may be warranted and slowly titrated neuraxial analgesia or anesthesia may be prudent; however, central venous
monitoring and pulmonary artery catheters are infrequently used. Certain cardiac conditions mandate general
anesthesia with invasive monitoring techniques including transesophageal echocardiography. In any event,
preparedness and vigilance, as well as a multidisciplinary approach with good communication between the
anesthesiologists, obstetricians, and cardiologists, are paramount for the safe management of the parturient with
cardiovascular disease.
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