Congenitally corrected transposition of the great arteries (CCTGA) is an uncommon congenital heart disease characterized by inversion of the ventricles resulting in both atrioventricular (AV) and ventricular-great artery discordance (Fig. 1). As a consequence, deoxygenated blood flows from the right atrium, through the left ventricle, and into the pulmonary artery (PA). In contrast, oxygenated blood flows from the left atrium, through the right ventricle, and into the aorta. Because the morphologic right ventricle and tricuspid valve are in the systemic circulation, most patients will develop systemic (morphologic right) ventricular dysfunction and varying degrees of systemic AV (morphologic tricuspid) valve regurgitation with increasing age.1–3 Similarly, the hemodynamic stress of pregnancy, labor, and delivery may also lead to ventricular failure and valvular dysfunction.
Previous investigations examining pregnancy outcomes in women with CCTGA have demonstrated that although successful pregnancies can be achieved, complications often occur.4,5 As a result, these patients may require specialized anesthetic care during labor and delivery. The current literature examining the anesthetic management of patients with CCTGA during labor and delivery is limited to single case reports.6–8 We describe the anesthetic management during labor and delivery of three parturients with CCTGA and review the relevant literature on this uncommon cardiac disorder.
A 34-yr-old acyanotic woman, gravida 1 para 0, presented at 39 wks’ gestation for elective cesarean delivery for breech presentation. At the age of 9 yrs, she was diagnosed with CCTGA. Her history included a tissue systemic (morphologic tricuspid) valve replacement for Ebstein’s-like malformation, an episode of endocarditis, permanent pacemaker placement for complete heart block, and paroxysmal atrial fibrillation and flutter. Her medications included prenatal vitamins and aspirin. A first trimester echocardiogram demonstrated CCTGA, a left AV valve replacement with no residual stenosis or regurgitation, and a systemic ventricular ejection fraction of 47%. At 19 wks’ gestation, palpitations led to Holter monitoring which showed AV pacing with underlying sinus rhythm, multiple premature ventricular complexes, and a single 8-beat run of supraventricular tachycardia. At 20 wks, uterine contractions led to a short hospitalization for fluid administration and subsequent at-home modified bed rest. Mild shortness of breath was noted at 30 wks’ gestation, and her pacemaker rate was increased from 70 to 80 bpm.
Immediately before cesarean delivery, the patient was hemodynamically stable with an arterial blood pressure of 120/80, a heart rate of 80 (AV paced), a respiratory rate of 21 breaths/min, and an oxygen saturation of 100% on 4 L/min oxygen via nasal cannula. Peripheral IV access was established, standard noninvasive monitoring initiated, and endocarditis prophylaxis administered. The pacemaker was interrogated and a magnet was placed at the bedside to permit conversion from a synchronous to an asynchronous mode. A lumbar epidural catheter was placed and tested with 3 mL of lidocaine 20 mg/mL with epinephrine 5 μg/mL. Additional lidocaine/epinephrine (17 mL) with fentanyl 100 μg was incrementally injected and a T-3 sensory level obtained. Chloroprocaine 30 mg/mL (3 mL) was administered every 15 min to maintain surgical anesthesia. A low transverse cesarean delivery was performed with a breech extraction of a vigorous 3600-g infant. Oxytocin 20 U diluted in 1 L of lactated Ringer’s (LR) solution was administered. Her estimated blood loss was 850 mL and a total of 2.1 L of LR solution was administered during the case. Her systolic blood pressure remained between 125 and 140 mm Hg except for two occasions: 98/65 mm Hg after the initial epidural bolus (treated successfully with ephedrine 5 mg) and 150/110 mm Hg immediately after delivery of the fetus. The patient was monitored in the intensive care unit for 24 h with no postpartum cardiovascular complications.
A 35-yr-old acyanotic woman, gravida 3 para 0, was admitted at 34-6/7 wks’ gestation with shortness of breath, pedal edema, and breech presentation for suspected congestive heart failure. Her arterial blood pressure was 110/70, heart rate 82 bpm in normal sinus rhythm, and a respiratory rate 16 breaths/min. She was acyanotic with no oxygen saturation recorded. CCTGA was diagnosed at 19 yrs of age. Her medical history was significant for alcohol and substance (cocaine) abuse from which she claimed abstinence during pregnancy, two elective abortions for nonmedical reasons, and full-time employment as a coal miner. She had no difficulty with the physical demands of her work until she became pregnant. At 15-wks’ gestation, she went on temporary disability for increasing fatigue and dyspnea. Her medications included prenatal vitamins. An echocardiogram performed at the time of hospital admission was compared with a first trimester examination and revealed multiple changes, including a decrease in ejection fraction from 50% to 45%, an increase in systemic AV (morphologic tricuspid) valvular regurgitation, significant worsening of subpulmonary and pulmonic valve stenosis (peak gradient 52 mm Hg), and new onset nonsystemic (morphologic mitral) AV valve regurgitation (mild). Although worsening of the subpulmonary and pulmonic gradient may be expected with progression of the pregnancy, the patient’s symptoms and poor functional status indicated that she was unable to meet the increased cardiac demands of pregnancy. The patient was initially managed conservatively with bedrest and fetal monitoring. After 5 days, with only minimal improvement in her symptoms, it was decided to proceed with cesarean delivery for breech presentation.
Endocarditis prophylaxis and supplemental oxygen were administered after obtaining peripheral IV access. In addition to standard noninvasive monitors, a right radial arterial line was placed before initiation of epidural anesthesia. A lumbar epidural catheter was placed and a standard lidocaine/epinephrine test dose was administered. Bupivacaine 5 mg/mL (20 mL) with fentanyl 100 μg was slowly administered until a T4 sensory level was obtained. A low transverse cesarean delivery was performed with breech extraction of a vigorous 2625-g infant. Oxytocin 20 U diluted in 1 L of LR solution was administered. Her estimated blood loss was 500 mL, and 700 mL of LR solution was administered during the procedure. Her systolic blood pressure remained between 110 and 120 mm Hg. No vasoactive medications were required.
Postpartum, the patient was observed in the intensive care unit for 24 h. Epidural analgesia was provided with bupivacaine 1.25 mg/mL and fentanyl 10 mcg/mL at 6 mL/h. Captopril, 6.25 mg twice daily, was initiated within hours of delivery. However, this was discontinued after 24 h because of hypotension and lightheadedness. Chest radiographs demonstrated no signs of congestive heart failure. The patient was discharged on postoperative Day 4 with no postpartum cardiovascular complications.
A 31-yr-old woman, gravida 1 para 0, presented at 36 wks’ gestation for elective induction of labor. Upon admission, her respiratory rate was 22 breaths/min with a room air oxygen saturation of 92%. Her arterial blood pressure was 111/71, and heart rate was 75 bpm in normal sinus rhythm. The patient was diagnosed with CCTGA at birth. Her medications included prenatal vitamins. The current pregnancy was uncomplicated, with no clinical signs or symptoms of congestive heart failure. Echocardiography demonstrated dextrocardia, severe systemic AV (morphologic tricuspid) valvular regurgitation, systemic (morphologic right) ventricular dilation with an ejection fraction of 40%, a small atrial septal defect (ASD), a small membranous ventricular septal defect (VSD) with bidirectional shunt, and severe subpulmonary and pulmonic valvular stenosis (peak gradient 70 mm Hg) with moderate pulmonary valve regurgitation.
On the basis of the presence of several significant cardiac lesions, the cardiology and obstetric teams elected to induce labor at 36 wks’ gestation to avoid progression of maternal systemic AV (morphologic tricuspid) valvular regurgitation and congestive heart failure, as well as to ease vaginal delivery with a smaller newborn. Labor was induced with misoprostol, and endocarditis prophylaxis was administered. A central venous line was placed in the right internal jugular vein followed by placement of a lumbar epidural catheter. The patient refused an invasive arterial catheter. Monitoring in the labor room included five-lead electrocardiogram (ECG) (placed in reverse because of dextrocardia), continuous pulse oximetry, noninvasive arterial blood pressure monitoring, central venous pressure monitoring, fetal heart rate monitoring, and tocodynamometry. When contractions became regular, an epidural infusion of bupivacaine 1.25 mg/mL with fentanyl 2 μg/mL was initiated at 6 mL/h. The epidural infusion was titrated between 6 and 14 mL/h based on patient comfort. Forty minutes before delivery, 10 mL of the epidural solution was administered as a bolus. During the second stage of labor, the fetus was allowed to descend without maternal pushing until low outlet forceps could be applied. With gentle traction on the forceps, the patient pushed with every other contraction until the delivery of a vigorous 2670-g infant after the third push. Her systolic blood pressures remained between 100 and 120 mm Hg and central venous pressure between 6 and 8 mm Hg throughout labor and delivery, except at birth, when the arterial blood pressure increased to 138/88 mm Hg and the central venous pressure increased to 14 mm Hg. Her estimated blood loss was 750 mL. During the final 12 h of labor, 2800 mL of crystalloid solution was administered. The patient was monitored in the intensive care unit for approximately 24 h and discharged from the hospital on postpartum Day 3.
Three weeks postpartum, the patient reported to her Mayo Clinic cardiologist that she had been admitted to her local hospital for persistent vaginal bleeding, symptomatic hypotension, and a hemoglobin of 7.0 g/dL. She was transfused two units of packed red blood cells and discharged 3 days later. Six weeks postpartum, she returned to her local physician with marked lower extremity edema, progressive dyspnea on exertion, and paroxysmal nocturnal dyspnea. The patient was treated with oral furosemide as an outpatient, with complete resolution of her symptoms. One year later, the patient remained mildly symptomatic. Her echocardiogram demonstrated persistent moderate to severe right ventricular enlargement, a reduction in systolic function, and severe systemic AV (morphologic tricuspid) valvular regurgitation. It was recommended that she undergo surgical repair of her ASD and VSD, and replacement of both the pulmonic and systemic valves, or alternatively, a double-switch procedure with appropriate valve replacement or repair.
This case series describes the successful obstetric and anesthetic management of three patients with CCTGA and associated comorbidities. The report is consistent with previous outcome studies that have shown that CCTGA patients generally tolerate pregnancy, labor, and delivery quite well. A case series examining pregnancy outcomes among 22 women with CCTGA described 60 pregnancies resulting in 50 (83%) live births.4 Six (12%) patients delivered by cesarean and 44 (88%) patients by vaginal birth. Three patients (5% of the pregnancies) had pregnancy-related complications. One patient experienced congestive heart failure and systemic (morphologic tricuspid) AV valve regurgitation, one patient was diagnosed with gestational diabetes and hypertension, and one patient had life-threatening congestive heart failure, toxemia, endocarditis, and myocardial infarction. Another case series examined 45 pregnancies among 19 CCTGA patients resulting in 6 (13%) elective terminations, 12 (27%) spontaneous abortions, and 27 (60%) live births.5 Five patients (26%) had pregnancy-related complications including congestive heart failure (three patients), worsening cyanosis (one patient), and a cerebral vascular event (one patient). Neither of these series described the anesthetic management of patients during labor and delivery. Preexisting cardiac lesions, the presence of intracardiac shunting, systemic ventricular function, and the propensity for cardiac dysrhythmias are all important considerations when developing an anesthetic care plan for patients with CCTGA.
Associated Intracardiac Lesions
CCTGA is often associated with a variety of congenital intracardiac lesions that may complicate anesthetic management. Abnormalities include VSD, anomalies of the AV valves, pulmonary outflow obstruction, myocardial perfusion defects, conduction abnormalities, and dextrocardia.9 The life expectancy of patients with CCTGA is highly dependent on the presence and severity of these coexisting conditions. In fact, patients with no additional intracardiac lesions often achieve a normal lifespan.10,11 Similarly, the ability of patients to tolerate the peripartum period is dependent on coexisting heart defects.
The decrease in systemic vascular resistance (SVR) associated with pregnancy reduces the workload of the systemic (right) ventricle and decreases systemic (tricuspid) AV valve regurgitation often present in CCTGA. This complementary physiology may, in part, explain why many of these women do relatively well during pregnancy, labor, and delivery. Similarly, patients with CCTGA are likely to tolerate decreases in SVR associated with neuraxial anesthesia without difficulty. The exceptions to this are CCTGA patients in whom decreases in SVR lead to increases in right-to-left intracardiac shunting, such as those with significant septal defects. If invasive arterial and central venous monitoring is planned in these patients, the anesthesiologist may wish to consider placing these monitors before initiation of neuraxial technique. Furthermore, identification of the epidural space using a loss-of-resistance with saline instead of air technique should be considered, and air filters should be placed on the IV lines to prevent paradoxical air embolism.
Systemic Ventricular Function
Approximately half of patients with CCTGA have systemic (morphologic right) ventricular dysfunction.1 Systemic ventricular function will likely dictate the anesthetic management of parturients with CCTGA. Normal ventricular function is critically important for a successful pregnancy, labor, and delivery. In fact, CCTGA patients with an ejection fraction <40% are often counseled to avoid pregnancy.12 The progressive decrease in systemic ventricular function in CCTGA is attributed to the fact that the thinner-walled right ventricle is not designed to adequately pump against systemic pressures and to an imbalance between myocardial supply and demand. Coronary anatomy is concordant, and therefore, the morphological right (systemic) ventricle is perfused by a single right coronary artery.13 In addition, the morphologic right (systemic) ventricle has a complex relationship with systemic AV (tricuspid) valve regurgitation, such that when systemic ventricular failure occurs, AV valve regurgitation is often the primary cause. Therefore, CCTGA parturients should be evaluated by a cardiologist knowledgeable in adult congenital heart disease on a regular basis beginning in the first trimester. If possible, an echocardiogram should be performed before pregnancy and reevaluated during the second trimester to monitor for any changes in systemic ventricular function or AV valve regurgitation.
Because cardiac output increases significantly during pregnancy, the systemic (morphologic right) ventricle in CCTGA parturients may dilate and decrease in function. In patients with dextro- (not congenitally corrected) transposition of the great arteries in which a Mustard (atrial switch) procedure has been performed, the right (systemic) ventricle will often dilate with progressive tricuspid regurgitation during pregnancy which does not resolve after pregnancy.14 Whether this is true for patients with CCTGA is not known. However, congenital heart specialists consider it critical to avoid congestive heart failure during the peripartum period in patients whose right ventricle is their systemic ventricle to preserve long-term systemic ventricular function. In fact, experts recommend that CCTGA parturients with early evidence of congestive heart failure be delivered prematurely before further heart failure develops. Even with these recommendations, anesthesia providers must be prepared to recognize and manage both chronic and acute congestive heart failure in CCTGA patients during labor and delivery. Considering the significant autotransfusion that occurs immediately after delivery, anesthesia providers should consider being present at the time of delivery in patients with evidence of heart failure to treat acute heart failure, and patients should be monitored in an acute care setting in the immediate (24 h) postpartum period.
Propensity for Dysrhythmias
The anesthesiologist must also recognize the CCTGA parturient’s propensity for dysrhythmias. Complete heart block, most likely due to a malpositioned AV node and an inherently abnormal conduction system, is common in CCTGA patients.15 In fact, 5%–13% of patients with CCTGA are born with complete heart block,16 with the rate of development of heart block estimated at 2% per year.17 Therefore, β-blockade must be used with extreme caution. Systemic AV valve insufficiency may also result in atrial dilation and subsequent atrial dysrhythmias. Spontaneous ventricular tachycardia deteriorating to ventricular fibrillation has been reported as well.18,19 Therefore, a five-lead ECG tracing should be monitored continuously by a qualified health care provider to rapidly intervene when arhythmias occur during labor and delivery. Importantly, CCTGA parturients with dextrocardia (Case 3) should have the position of their ECG leads reversed for appropriate monitoring. CCTGA patients with a pacemaker (Case 1) should be managed in accordance with the American Society of Anesthesiologists Practice Advisory.20
According to present guidelines, most CCTGA parturients do not require antibiotic prophylaxis for infective endocarditis (IE) for surgical or vaginal delivery. The most recent American Heart Association guidelines for IE prophylaxis do not recommend prophylaxis for patients who undergo genitourinary procedures,21 and the American College of Obstetrics and Gynecology and the American Heart Association guidelines state that IE prophylaxis is not necessary for uncomplicated vaginal or cesarean delivery.22 Even for “high-risk cardiac lesions,” prophylaxis is optional. High-risk lesions include prosthetic cardiac valves, a history of IE, complex cyanotic congenital malformations, and surgically repaired systemic to pulmonary shunts. Most CCTGA parturients are, therefore, not considered high risk, but in those patients who have “high-risk cardiac lesions” (e.g., systemic AV valve replacement), it may be reasonable to consider antibiotic prophylaxis at the time of delivery, recognizing that proof of efficacy is not available.
Monitoring the CCTGA patient depends on baseline cardiopulmonary status, the mode of delivery (vaginal versus cesarean), and the anesthetic technique. Beside standard labor or anesthetic monitors, invasive arterial blood pressure and central venous pressure monitoring can be considered. For example, maternal dyspnea or nonreassuring fetal status during the second stage of labor may warrant placement of an invasive central or arterial line to monitor the effects of uterine contractions and maternal expulsive efforts on hemodynamic stability. Invasive arterial blood pressure and central venous pressure monitoring may be helpful during cesarean delivery to guide fluid management, particularly if the parturient is at risk for uterine atony or hemorrhage. If the parturient requires a general anesthetic, cardiac function may also be assessed using transesophageal echocardiography.
PA catheterization should be carefully considered in CCTGA patients. Catheter placement may be complicated by preexisting conduction abnormalities (e.g., complete heart block) and anatomic defects (e.g., ASD, VSD, pulmonary valve stenosis). Because the AV node is positioned abnormally within the anterior wall of the right atrium,23 PA catheter placement may result in an increased risk for cardiac dysrhythmias. In addition, severe subpulmonary and pulmonary valve stenosis (Cases 2 and 3) may further complicate PA catheter placement. However, in CCTGA parturients with acute or chronic congestive heart failure, PA catheterization may be valuable. Regardless, the increased risk of dysrhythmias and the potential for difficult line placement needs to be carefully considered.
Vaginal delivery is acceptable in most parturients with cardiovascular disease, with the possible exceptions of women who have a markedly dilated aortic root, severe left heart obstruction, or are anticoagulated with warfarin at the time of delivery (due to the risk of fetal intracranial hemorrhage during vaginal delivery).15 For cesarean delivery, most anesthesiologists prefer neuraxial anesthesia for congenital heart disease parturients,24 probably because the maintenance of spontaneous respiration may result in fewer cardiovascular and respiratory complications. A continuous epidural or spinal anesthetic will allow a gradual titration of neuroblockade without sudden hemodynamic changes. The treatment of choice for intraoperative hypotension will depend on several variables; for example, phenylephrine may be the drug of choice in the presence of tachycardia, intracardiac shunt, or a history of tachyarrhythmias. General anesthesia may be prudent in the setting of extensive bleeding or anticipated hemodynamic instability, acute congestive heart failure, or when transesophageal echocardiography is desirable.
Pain and anxiety during labor and vaginal delivery may cause catecholamine surges that result in tachycardia, hypertension, and increased cardiac output and systemic ventricular afterload. In patients at risk of developing congestive heart failure, this may lead to cardiac dysrhythmias, myocardial ischemia, or fulminate congestive heart failure. Early initiation of neuraxial analgesia (e.g., epidural catheter placement with gradual local anesthetic titration or intrathecal opioid-only combined spinal-epidural analgesia) may be prudent to limit hemodynamic shifts.
In summary, the long-term cardiac sequelae of pregnancy, labor, and delivery in parturients with CCTGA are unknown. However, parturients with CCTGA require a thorough multidisciplinary peripartum management plan to promote the safest possible delivery for both the mother and baby. Preexisting intracardiac lesions, the presence of intracardiac shunting, systemic ventricular dysfunction, and a propensity for cardiac dysrhythmias are important considerations in CCTGA.
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