Secondary Logo

Journal Logo

A Case Series of the Anesthetic Management of Parturients with Surgically Repaired Tetralogy of Fallot

Arendt, Katherine W. MD*; Fernandes, Susan M. MHP, PA-C§; Khairy, Paul MD, PhD§; Warnes, Carole A. MD; Rose, Carl H. MD; Landzberg, Michael J. MD§; Craigo, Paula A. MD*; Hebl, James R. MD*

doi: 10.1213/ANE.0b013e31821ad83e
Obstetric Anesthesiology: Research Reports
Free
SDC

BACKGROUND: Most case reports of pregnancies after surgical repair of tetralogy of Fallot have focused on cardiovascular and obstetric concerns, with relatively few authors focusing on specific intrapartum and postpartum anesthetic management strategies.

METHODS: The Mayo Clinic Congenital Heart Disease Clinic and the Boston Adult Congenital Heart Disease Service databases were cross-referenced with the Mayo Clinic and the Brigham and Women's Hospital Department of Anesthesiology databases to identify patients with tetralogy of Fallot who delivered at their respective hospital from January 1, 1994, to January 1, 2008. We reviewed each medical record to evaluate parturient care during pregnancy, labor, and delivery with a focus on anesthetic management.

RESULTS: During the 14-year study period, a total of 27 deliveries in 20 patients with repaired tetralogy of Fallot were identified. Twenty-one deliveries (78%) among 15 parturients (75%) involved a trial of labor; all parturients received neuraxial analgesia for labor and delivery, including 18 (86%) epidural, 2 (10%) combined spinal–epidural, and 1 (5%) continuous spinal anesthetic after an unintended dural puncture. Of the 21 patients undergoing labor, 3 (14%) received invasive arterial blood pressure monitoring; 5 (24%) received continuous telemetry; 3 (14%) experienced congestive heart failure that required diuresis; 4 (19%) had obstetric or neonatal complications; and 3 (14%) had anesthesia complications. Cesarean delivery was required in 4 patients (19%) because of labor complications. Concurrent cardiovascular, obstetric, and anesthetic complications in 1 patient resulted in neonatal death. Six (22%) parturients underwent elective cesarean delivery; 4 received epidural and 2 received spinal anesthesia; no anesthetic or immediate obstetric complications occurred. Among all parturients, 5 deliveries in 5 separate parturients (19% of deliveries) reported symptoms of congestive heart failure at the time of delivery.

CONCLUSIONS: Pregnancy outcomes for patients with repaired tetralogy of Fallot were found to be generally favorable. All patients undergoing a trial of labor or cesarean delivery had neuraxial analgesia or anesthesia. Recognition and management of congestive heart failure was necessary in 19% of deliveries.

Published ahead of print May 19, 2011 Supplemental Digital Content is available in the text.

From the *Department of Anesthesiology, Division of Cardiovascular Diseases, and Department of Obstetrics and Gynecology, Mayo Clinic, Rochester, Minnesota; and §Boston Adult Congenital Heart Service, Brigham and Women's Hospital and Children's Hospital, Harvard Medical School, Boston, Massachusetts.

Funding: Intramural funding has been provided by the Department of Anesthesiology, Mayo Clinic, Rochester, Minnesota.

The authors declare no conflicts of interest.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (www.anesthesia-analgesia.org).

Reprints will not be available from the authors.

Address correspondence to Katherine W. Arendt, MD, Department of Anesthesiology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905. Address e-mail to arendt.katherine@mayo.edu.

Accepted March 2, 2011

Published ahead of print May 19, 2011

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease encountered in patients surviving into adulthood in the United States.1 The fundamental anatomic defect in TOF consists of a displaced infundibular septum, resulting in the classic tetrad of a ventricular septal defect, an overriding aorta, infundibular obstruction of the right-sided outflow tract, and associated right ventricular (RV) hypertrophy. Surgical repair of TOF includes RV outflow tract reconstruction and closure of the ventricular septal defect. Several case reports28 and limited case series916 have been published that describe the obstetric and cardiac concerns of pregnancy, labor, and delivery; these reports include women with the full spectrum of unoperated, palliated, incompletely repaired, and completely repaired TOF. However, a limited number of these reports describe the anesthetic management; a single report focused on anesthetic techniques but does not specifically describe the management of TOF versus other congenital heart diseases.17 In an effort to identify how anesthesiologists are managing parturients with TOF during labor and delivery, we performed a retrospective chart review of all parturients with TOF who delivered at the Mayo Clinic Rochester and Brigham and Women's Hospital (BWH) over 14 years.

Back to Top | Article Outline

METHODS

After approval by the IRBs of both the Mayo Clinic and BWH, the Mayo Clinic Congenital Heart Disease Clinic database was queried to retrospectively identify pregnant patients with TOF from January 1, 1994, to January 1, 2008. Identified patients were then cross-referenced with the Department of Anesthesiology database to limit the search to those patients delivering at Mayo Clinic Rochester. A similar search process was conducted with the Boston Adult Congenital Heart Disease database to identify women who delivered at BWH in Boston. The medical records of all patients with TOF who delivered at the Mayo Clinic or BWH were reviewed to evaluate care during pregnancy, labor, and delivery. Demographic data including maternal age, parity, and gestational age at delivery were obtained along with the patient's obstetric, medical, and cardiac surgical history. Specific notation was made of previous episodes of cardiac arrhythmia or heart failure. Medications at the time of delivery as well as the most recent recorded electrocardiogram (ECG) and transesophageal echocardiography (TEE) report before delivery were reviewed. Delivery data were extracted, including the type of delivery, if maternal expulsive efforts were permitted, type of labor analgesia, method of hemodynamic monitoring, and postpartum care. Immediate neonatal outcome and any maternal cardiac, obstetric, or anesthetic complications were also recorded as well as the overall status of the mother and baby as documented at the 6-week postpartum obstetric follow-up appointment.

Back to Top | Article Outline

RESULTS

Six patients with TOF undergoing 7 deliveries at the Mayo Clinic and 14 patients with TOF undergoing 20 deliveries at BWH were identified during the study interval. All patients had undergone surgical repair before conception. Patient demographics, parity, and gestational age at delivery are listed in Table 1. Each patient's cardiac history, surgical history, and cardiac comorbidities are provided in Table 2. The obstetric and anesthetic management and outcomes of patients, including the need for a postpartum intensive care unit (ICU) admission, are presented in Table 3. Web Supplement Tables 4 and 5 summarize the patients' most recent TEE and ECG before delivery, respectively (see Supplemental Digital Content 1, http://links.lww.com/AA/A273, and 2, http://links.lww.com/AA/A274). Patient characteristics and their associated outcomes are described below on the basis of their method of delivery.

Table 1

Table 1

Table 2

Table 2

Table 3

Table 3

Back to Top | Article Outline

Trial of Labor

Twenty-one deliveries (78%) among 15 patients (75%) involved a trial of labor. All patients undergoing a trial of labor received neuraxial analgesia, including 18 (86%) epidural and 2 (9%) combined spinal–epidural (CSE) techniques, as well as 1 (5%) continuous spinal technique after an unintended dural puncture. All patients underwent continuous pulse oximetry monitoring and intermittent noninvasive arterial blood pressure monitoring during labor. Invasive arterial blood pressure monitoring (arterial line placement) was performed in 3 patients (14%) and continuous telemetry (5-lead ECG) in 5 patients (24%) during labor and delivery. No patient underwent central line placement for central venous pressure (CVP) monitoring. The fluid management of patients is summarized in Web Supplement Table 6 (see Supplemental Digital Content 3, http://links.lww.com/AA/A275). Three patients (14%) who underwent a trial of labor had congestive heart failure (CHF) requiring diuresis during their pregnancy (2 patients during the second trimester and 1 patient during the third trimester). Three patients (14%) experienced significant obstetric or neonatal complications, including 2 episodes of postpartum hemorrhage and 1 neonatal death from anoxia. There were 3 (14%) anesthetic complications, including 2 patients with inadequate analgesia after epidural catheter placement and replacement, and 1 patient with an unintentional intrathecal catheter placement. Of the 15 patients who had (assisted or unassisted) vaginal delivery, 11 (73%) had a passive second stage involving minimal to no pushing. In 4 patients (19%), a trial of labor was unsuccessful, necessitating cesarean delivery. Three patients underwent cesarean delivery under epidural anesthesia using an epidural catheter placed for labor analgesia, and 1 patient received a single-shot spinal anesthetic because of inadequate labor epidural analgesia.

Back to Top | Article Outline

Elective Cesarean Delivery

Six (22%) patients underwent elective cesarean delivery for obstetric or fetal indications (2 breech presentations; 1 repeat cesarean delivery; 1 fetal cardiac anomaly; 1 maternal congenital pelvic defect; 1 maternal request). Although 1 patient developed CHF during the third trimester requiring diuresis, this was not the indication for her cesarean delivery. No cesarean delivery was scheduled exclusively for maternal cardiac or anesthetic indications. Four patients (67%) received epidural anesthesia and 2 (33%) patients received spinal anesthesia. Two patients underwent both arterial and CVP monitoring, 1 patient received arterial monitoring alone, and the remaining 3 patients received standard noninvasive monitoring. Intraoperative fluid management is summarized in Web Supplement Table 6 (see Supplemental Digital Content 3, http://links.lww.com/AA/A275). There were no anesthetic or immediate obstetric complications. One patient with compensated CHF underwent cesarean delivery and experienced postpartum ventricular ectopy and pulmonary edema, requiring prolonged telemetry monitoring and increased beta-blocker therapy. One patient was treated 3 weeks postpartum for pneumonia, and another patient was treated 3 weeks postpartum for retained products of conception.

Back to Top | Article Outline

Cardiac Complications

Symptoms of CHF during pregnancy occurred in 5 patients, 2 during the second trimester and 3 during the third trimester. None of the patients developed new-onset CHF during or immediately after labor and delivery. Three of the 5 patients with CHF underwent a trial of labor with epidural analgesia. Of these, 1 patient labored with an arterial line and continuous telemetry monitoring. Four of the 5 patients who experienced CHF during pregnancy had severe pulmonary regurgitation and either elevated RV systolic pressures or TEE signs of RV systolic dysfunction during prenatal assessment. Of the 15 patients who had severe pulmonary regurgitation, 4 (27%) developed CHF during pregnancy. Of the 7 patients who had RV systolic dysfunction, 3 (43%) developed CHF during pregnancy.

The only patient who developed CHF without a history of either severe pulmonary regurgitation or RV dysfunction suffered an acute obstruction of her RV-to-pulmonary artery (Rastelli) conduit during the third trimester of pregnancy. Labor was induced at 36 weeks' gestation for right heart failure. Successful epidural analgesia was achieved after multiple failed epidural and spinal attempts. Four hours later, an emergent cesarean delivery was performed for nonreassuring fetal status, with delivery of a preterm infant with 1- and 5-minute Apgar scores of 2 and 2, respectively. The infant subsequently died in the neonatal ICU.

Postpartum ICU admission was required in 4 patients. The patient with an acute obstruction of her Rastelli conduit also suffered from obstructive sleep apnea and was admitted to the ICU. Two patients were admitted for hemodynamic monitoring and observation and 1 for a history of nonsustained ventricular tachycardia during this pregnancy that recurred during cesarean delivery. She was treated in the ICU with an increased dose of beta-blocker therapy, resulting in restoration of sinus rhythm. No other patients required postpartum monitoring beyond standard postpartum care.

Back to Top | Article Outline

Fluid Management

Fluid management was variable for both patients in labor (fluid volume range: 52 mL/h to 1750 mL/h) and for patients during cesarean delivery (fluid volume range: 1300 mL to 2200 mL) (Web Supplement Table 6, see Supplemental Digital Content 3, http://links.lww.com/AA/A275). Crystalloid was used for all patients; no colloid solutions were administered to any patient during labor and delivery. One patient experienced postpartum hemorrhage requiring 2 U of packed red blood cells.

Back to Top | Article Outline

Epidural Test Dose

In our series of 25 deliveries managed with planned epidural (or CSE) analgesia/anesthesia, 9 patients received an epidural test dose (Table 3). Of the patients who delivered at Mayo Clinic, 6 of 7 received a test dose. Four of the 6 test doses contained both local anesthetic and epinephrine, and 1 contained local anesthetic only. One record indicated that a test dose was given but did not document the type and amount of drug. Of the patients who delivered at the BWH, only 3 of 18 planned epidural procedures included an epidural test dose; 1 of these was the unintentional spinal catheter in which 0.25% bupivacaine 1.5 mL was administered to confirm intrathecal placement; 1 contained local anesthetic and epinephrine; and 1 record indicated that a test dose was given but did not document the type and amount of drug. It was not possible to determine from the chart whether a test dose was administered in 3 patients. No patient in our series experienced untoward side effects from the test dose, local anesthetic toxicity from unintended intravascular placement, or a high spinal from an unrecognized intrathecal catheter.

Back to Top | Article Outline

DISCUSSION

Surgical repair of TOF was first described in 1955.18,19 Subsequent refinement of the original surgical technique has resulted in a 32-year actuarial survival rate of >85%,20 allowing most women to reach childbearing age. Consequently, the majority of women delivering today, including all patients in this case series, have undergone surgical repair of TOF during childhood or adolescence. However, rare cases of unrecognized TOF may still present to labor and delivery units.7,21 The current series is one of the few reports in the literature that describes the anesthetic techniques and intrapartum monitoring in patients with surgically corrected TOF. Neuraxial analgesia or anesthesia was used in all patients; however, the use of invasive monitoring varied.

Back to Top | Article Outline

Risk Stratification

The outcomes reported in this series support previously published studies that suggest patients with surgically corrected TOF generally tolerate pregnancy, labor, and delivery quite well. One series reporting retrospective surveys of 43 women with repaired and unrepaired TOF in 82 successful pregnancies found that only 6 patients (14%) experienced cardiovascular complications, including supraventricular tachycardia, CHF, RV dilation, and pulmonary embolism.12 In another series involving women with repaired TOF, 25 women with 54 pregnancies experienced no cardiac complications or functional status deterioration during pregnancy and delivery.15 Finally, there were no adverse cardiovascular events reported in another series of 26 pregnancies in 16 women.16 Prior outcome studies have shown that patients with TOF who have severe pulmonary regurgitation or depressed RV systolic function or left ventricular (LV) systolic dysfunction are at increased risk for complications during pregnancy, labor, and delivery.12,14 The only neonatal death in the current report followed an emergent cesarean delivery for nonreassuring fetal status in a patient with acute RV-to-pulmonary artery conduit obstruction, resulting in severe pulmonary regurgitation and RV failure.

Determining which patients with TOF are at increased risk for pregnancy-induced complications can be instructional for anesthesia providers in developing an appropriate anesthetic care plan. Prognostic indicators for adverse outcomes during pregnancy for patients with heart disease include impaired RV or LV systolic function,12,13 severe pulmonary regurgitation and RV dysfunction,1214 and severe pulmonary hypertension.12 Other large series have found that patients with acquired or congenital heart disease, including TOF, who have New York Heart Association Functional Class ≥2,11,13 a history of CHF or systemic ventricular dysfunction,11,13 a history of arrhythmias,11 or a history of smoking13 are at increased risk of cardiac events during pregnancy.

Studies of nonpregnant patients with TOF may further assist in risk stratification. For example, factors that increase the risk for sudden cardiac death after TOF repair include LV dysfunction,22 the development of complete heart block,23 and a history of ventricular tachycardia or inducible polymorphic or monomorphic ventricular tachycardia.24 Severe LV dysfunction in the presence of a QRS duration ≥180 milliseconds has a high positive predictive value for future sudden cardiac death.22 Chronic pulmonary regurgitation is a common finding after TOF repair; severe pulmonary regurgitation can cause RV dilation, QRS prolongation, and QT dispersion, which are associated with sustained ventricular tachycardia and sudden cardiac death.2527 As a result, many TOF patients will undergo pulmonary valve replacement late after primary TOF repair to reduce RV dilation.28 Decreased long-term survival has been associated with the performance of a palliative Waterston or Potts shunt procedure before repair, age older than 12 years at the time of initial repair, and high RV-to-LV systolic pressure ratios immediately after repair.20

Although absolute maternal and fetal mortality risk during pregnancy may be low, significant morbidity can still occur. Patients with repaired TOF may exhibit residual cardiac disease such as valvular dysfunction (commonly pulmonary regurgitation), pulmonary vascular anomalies, RV or LV failure, residual RV outflow tract obstruction, residual shunting, or dysrhythmias. Occasionally, secondary pulmonary hypertension may result from increased pulmonary bloodflow from a previous shunt.

Because of the variability of anatomy, physiology, and cardiac history of each patient with repaired TOF, there is no single formula for pregnancy and delivery management that applies to all patients with repaired TOF. The American College of Cardiology and the American Heart Association (AHA) recommend that patients with TOF have a care plan formulated by a cardiologist who specializes in adults with congenital heart disease (class IC recommendation).29 Likewise, the anesthesia care team should consider reviewing the patient's cardiovascular testing, including TEE findings, before admission for delivery to develop an intrapartum care plan.

Back to Top | Article Outline

Anesthetic Management: Endocarditis Prophylaxis

For routine vaginal or cesarean deliveries, the AHA and the American Congress of Obstetrics and Gynecology do not recommend routine antibiotic prophylaxis against infective endocarditis.30 However, according to the 2007 AHA recommendations, routine infective endocarditis prophylaxis is considered a class IIa recommendation for high-risk patients with prosthetic cardiac valves, patients with a history of infective endocarditis, patients with unrepaired or palliated cyanotic congenital heart disease, those with completely repaired congenital heart disease with prosthetic material placed within the past 6 months or placed at or adjacent to a residual defect that prevents endothelialization, and cardiac transplantation recipients with valvulopathy.31 As reported in the American College of Cardiology and the American Heart Association 2008 Guidelines for the Management of Adults with Congenital Heart Disease, there is conflicting evidence and a divergence of opinion with regard to routine infective endocarditis prophylaxis for repaired TOF.29 Of note, all patients in this series received infective endocarditis prophylaxis for delivery, and no patient developed endocarditis within the first 6 weeks after delivery.

Back to Top | Article Outline

Anesthetic Management: Monitoring

As is true for most patients with cardiac disease, decisions regarding appropriate hemodynamic monitoring are dependent upon the patient's current cardiopulmonary status, the anticipated method of delivery (vaginal versus cesarean), and the selected anesthetic technique. In addition to routine noninvasive monitoring, if hemodynamic variables warrant closer surveillance, consideration should be given to invasive arterial blood pressure or CVP monitoring. If a patient with TOF was palliated with a Blalock– Taussig (subclavian artery-to-pulmonary artery) shunt before their initial repair, bloodflow to the ipsilateral arm may be compromised and arterial blood pressures should be monitored in the arm contralateral to the shunt.

Pulmonary artery catheter placement requires careful consideration of potential anatomic defects, such as residual RV outflow tract obstruction or pulmonary valve dysfunction, that can lead to difficult pulmonary artery catheter placement. Typically, pulmonary artery catheterization is reserved for patients with significant ventricular dysfunction or pulmonary hypertension. However, the risks associated with pulmonary artery catheter placement— including dysrhythmias, infection, and pulmonary artery rupture—may outweigh the potential benefit of monitoring pulmonary artery pressures. General anesthesia with intraoperative TEE can also be considered a method of evaluating intraoperative cardiac function. None of the patients in the current series underwent pulmonary artery catheterization nor intraoperative TEE.

In patients with a history of a dysrhythmia that resulted in hemodynamic compromise, consideration should be given to continuous ECG monitoring during labor and delivery. In a series of 73 women and 87 pregnancies with a history of tachyarrhythmia, the recurrence rate of paroxysmal supraventricular tachycardia was 50%, paroxysmal atrial fibrillation or atrial flutter 52%, and ventricular tachycardia 27% during pregnancy.32 Recurrence during the antepartum period increased the risk of adverse fetal complications independent of other maternal or fetal risk factors. Therefore, patients with TOF should be questioned about a history of dysrhythmias to determine monitoring requirements. Although spontaneous ventricular tachycardia has been described in patients with surgically repaired TOF,13 continuous ECG monitoring is unlikely to be helpful in patients without an arrhythmia history.

Back to Top | Article Outline

Anesthetic Management: Anesthetic and Analgesic Considerations

Experts believe that vaginal delivery is preferable for most patients with surgically repaired TOF, with cesarean delivery being reserved for standard obstetric indications.29,33 For labor analgesia, early dense epidural analgesia should be considered to minimize catecholamine surges and hemodynamic fluctuations. All of our laboring patients received neuraxial analgesia. In patients with a residual shunt, loss of resistance to saline rather than air may be prudent because venous air embolism during epidural placement has been reported.34 The risks and benefits of using an epinephrine-containing epidural test dose should be considered, because intravascular epinephrine injection may lead to dysrhythmias or rapid hemodynamic instability. The practice of using an epidural test dose was variable in our series; no untoward side effects were observed.

Because maternal pushing efforts during the second stage of labor may induce significant changes in cardiac output, some cardiologists and obstetricians recommend a “cardiac delivery,” that is, a passive second stage with operative vaginal delivery. During a passive second stage, fetal descent is accomplished by uterine contractions (without maternal expulsive efforts), with delivery effected by low/outlet forceps or vacuum assistance. Under these conditions, a dense epidural with sacral dermatome coverage is required. In deciding whether to have a patient with TOF push during labor, the risks of a longer second stage and operative vaginal delivery must be weighed against the negative physiologic effects of repetitive Valsalva maneuvers. The practice of avoiding second stage pushing remains a topic of debate among many cardiologists and obstetricians.35 There is no evidence that supports better outcomes with this practice. A recent retrospective study involving parturients with heart disease demonstrated that in comparison with parturients who actively pushed during second stage, those women who avoided pushing had a significantly longer second stage of labor, a significantly higher risk of postpartum hemorrhage, and a greater likelihood of third or fourth degree lacerations.35

There is no evidence that either neuraxial or general anesthesia is superior for cesarean delivery in parturients with TOF. However, this study supports other observational data that neuraxial anesthesia can be delivered safely in these women. Furthermore, there is no evidence to defend the superiority of either spinal or epidural anesthesia for these women. Traditionally, however, anesthesiologists have preferred epidural anesthesia in parturients with cardiac disease because of the potential for rapid vasodilation with single-shot spinal anesthesia. Of the 6 elective cesarean deliveries within our series, 2 patients received a single-shot spinal anesthetic technique with 1 patient's hypotension treated successfully with ephedrine and the other with both ephedrine and phenylephrine. Four of the 6 patients undergoing elective cesarean delivery received an epidural anesthetic; only 1 did not require the use of either ephedrine or phenylephrine.

A final technique that may be considered for patients with significant cardiac disease is a sequential low-dose CSE.36 During this technique, low-dose (3 to 5 mg) intrathecal bupivacaine is administered, followed by slow, incremental epidural local anesthetic administration to attain appropriate surgical anesthesia. Although this technique has been reported to provide the density of a spinal anesthetic with a lesser degree of hypotension,37 one study in healthy parturients found no difference in cardiac output or the incidence of hypotension, regardless of whether 5 mg or 10 mg of intrathecal bupivacaine was administered during a CSE technique for cesarean delivery.38

Our data suggest that women with corrected TOF who have an uneventful labor and delivery may successfully receive standard postpartum care. Patients with significant cardiac events may require more intensive monitoring in an ICU.

This case series demonstrates specifically that women with repaired TOF who carry an otherwise uncomplicated singleton pregnancy past the age of viability can successfully deliver in a tertiary medical center with coordinated multispecialty input with minimal alterations in anesthetic and obstetric management. The limitations of this study arise from the patient selection process and the retrospective methodology. All studied patients were functional enough to conceive and carry a pregnancy well past the limit of fetal viability. Women with repaired TOF who avoided or terminated pregnancy for medical indications were not included. Therefore, caution should be used in extrapolating from this select group to the general population of women with repaired TOF. Though the group of described patients was large in relation to the relevant medical literature, the number was still small enough that none of the complications of anesthesia and delivery that can be life threatening in even the healthiest parturient were observed. There were no infectious or thromboembolic complications; no patient had preeclampsia, diabetes mellitus, or multiple gestations. There were no unrecognized intrathecal or intravascular epidural catheters, no positive epidural test doses, difficult tracheal intubations, high or total neuraxial anesthetics, or drug administration errors. No patient received uterotonic drugs other than oxytocin, and no patient received tocolysis with terbutaline. The only episodes of hemorrhage were mild and occurred postpartum. A further limitation is that this study encompassed a 14-year study period during which time obstetric anesthetic management in general may have changed, and familiarity with the adult with congenital heart disease may have evolved. Thus, the long study period may have contributed to management strategy differences among those who received care early in the study period in comparison with late. It should be emphasized that although obstetric and anesthetic management was relatively standard, care was conducted in large tertiary specialty centers by experienced physicians with resources available around the clock to intensify monitoring and management should complications ensue.

Back to Top | Article Outline

Summary

In summary, patients with surgically repaired TOF generally tolerate pregnancy, labor, and delivery quite well, but can still experience significant peripartum complications. Complications in this case series included the development of CHF during the course of pregnancy, intrapartum arrhythmias, and neonatal death. Residual cardiac disease such as anatomic defects, residual shunt, or a history of dysrhythmias may be present after TOF repair and need to be considered when developing an anesthetic plan for labor and delivery. Neuraxial analgesia and anesthesia can be safely administered to patients with repaired TOF, and was the technique of choice for labor analgesia and surgical anesthesia for all patients within the current series. Continuous telemetry monitoring should be considered during labor in patients with a history of an arrhythmia that has led to hemodynamic compromise. Patients with a history of CHF require meticulous fluid management. Finally, enhanced postpartum monitoring may not be necessary for this subgroup of patients unless indicated secondary to intrapartum events. Increased vigilance, excellent labor analgesia, and attentive surgical anesthesia can lead to successful outcomes for the majority of patients with surgically repaired TOF.

Back to Top | Article Outline

DISCLOSURES

Name: Katherine W. Arendt, MD.

Contribution: This author helped design the study, conduct the study, analyze the data, and write the manuscript.

Attestation: This author has seen the original study data, reviewed the analysis of the data, approved the final manuscript, and is the author responsible for archiving the study files.

Name: Susan M. Fernandes, MHP, PA-C.

Contribution: This author helped conduct the study, analyze the data, and write the manuscript.

Attestation: This author has seen the original study data, reviewed the analysis of the data, and approved the final manuscript.

Name: Paul Khairy, MD.

Contribution: This author helped analyze the data and write the manuscript.

Attestation: This author has seen the original study data, reviewed the analysis of the data, and approved the final manuscript.

Name: Carole A. Warnes, MD.

Contribution: This author helped design the study, analyze the data, and write the manuscript.

Attestation: This author has seen the original study data, reviewed the analysis of the data, and approved the final manuscript.

Name: Carl H. Rose, MD.

Contribution: This author helped design the study, analyze the data, and write the manuscript.

Attestation: This author has seen the original study data, reviewed the analysis of the data, and approved the final manuscript.

Name: Michael J. Landzberg, MD.

Contribution: This author helped design the study, analyze the data, and write the manuscript.

Attestation: This author has seen the original study data, reviewed the analysis of the data, and approved the final manuscript.

Name: Paula A. Craigo, MD.

Contribution: This author helped design the study and write the manuscript.

Attestation: This author has seen the original study data, reviewed the analysis of the data, and approved the final manuscript.

Name: James R. Hebl, MD.

Contribution: This author helped design the study, analyze the data, and write the manuscript.

Attestation: This author has seen the original study data, reviewed the analysis of the data, and approved the final manuscript.

Back to Top | Article Outline

REFERENCES

1. Hoffman JIE, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890–900
2. Busky A, Grusetz M. Pregnancy and delivery following operation for tetralogy of Fallot. Am J Obstet Gynecol 1955;70:1143
3. Meyer E, Tulsky A, Sigmann P, Silber E. Pregnancy in the presence of tetralogy of Fallot. Observations on two patients. Am J Cardiol 1964;14:874
4. Piccaro M, Rastelli D, Zecchi P, Moneta E. Tetralogy of Fallot and pregnancy. Minerva Ginecol 1982;34:547–57
5. Ralstin JH, Dunn M. Pregnancies after surgical correction of tetralogy of Fallot. JAMA 1976;235:2627–8
6. Malbranche-Aupecle MH, Mavel A, Jahier J, Kamp A, Feldman JP. Pregnancy in a patient with an isotopic pacemaker placed for surgical atrioventricular block following treatment of tetralogy of Fallot. Apropos of a case. Review of the literature. Rev Fr Gynecol Obstet 1985;80:105–7
7. Larsen-Disney P, Price D, Meredith I. Undiagnosed maternal Fallot tetralogy presenting in pregnancy. ANZJOG 1992;32:169–71
8. Vaclavinkova V, Machado L. Delivery in a multipara with unoperated Fallot's tetralogy. Int J Gynaecol Obstet 1994;44:165–6
9. Singh H, Bolton PJ, Oakley CM. Pregnancy after surgical correction of tetralogy of Fallot. BMJ 1982;285:168
10. Nissenkorn A, Friedman S, Schonfeld A, Ovadia J. Fetomaternal outcome in pregnancies after total correction of the tetralogy of Fallot. Int Surg 1984;69:125–8
11. Siu SC, Sermer M, Colman JM, Alvarez AN, Mercier LA, Morton BC, Kells CM, Bergin ML, Kiess MC, Marcotte F, Taylor DA, Gordon EP, Spears JC, Tam JW, Amankwah KS, Smallhorn JF, Farine D, Sorensen S. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation 2001;104:515–21
12. Veldtman GR, Connolly HM, Grogan M, Ammash NM, Warnes CA. Outcomes of pregnancy in women with tetralogy of Fallot. J Am Coll Cardiol 2004;44:174–80
13. Khairy P, Ouyang DW, Fernandes SM, Lee-Parritz A, Economy KE, Landzberg MJ. Pregnancy outcomes in women with congenital heart disease. Circulation 2006;113:517–24
14. Meijer JM, Pieper PG, Drenthen W, Voors AA, Roos-Hesselink JW, van Dijk APJ, Mulder BJM, Ebels T, van Veldhuisen DJ. Pregnancy, fertility, and recurrence risk in corrected tetralogy of Fallot. Heart 2005;91:801–5
15. Pedersen LM, Pedersen TAL, Ravn HB, Hjortdal VE. Outcomes of pregnancy in women with tetralogy of Fallot. Cardiol Young 2008;18:423–9
16. Gelson E, Gatzoulis M, Steer PJ, Lupton M, Johnson M. Tetralogy of Fallot: maternal and neonatal outcomes. BJOG 2008;115:398–402
17. Dob DP, Yentis SM. UK registry of high-risk obstetric anaesthesia: report on cardiorespiratory disease. Int J Obstet Anesth 2001;10:267–72
18. Kirklin J, Dushane J, Patrick R, Donald D, Hetzel P, Harshbarger H, Wood E. Intracardiac surgery with the aid of a mechanical pump–oxygenator system (gibbon type): report of eight cases. Proc Staff Meet Mayo Clin 1955;30:201–6
19. Lillehei C, Cohen M, Warden H, Read RC, Aust JB, De Wall RA, Varco RL. Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects: report of the first 10 cases. Ann Surg 1955;142:418–42
20. Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, McGoon DC, Kirklin JW, Danielson GK. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993;329:593–9
21. Vaclavinkova V, Machado L. Delivery in a multipara with unoperated Fallot's tetralogy. Int J Gynaecol Obstet 1994;44:165–6
22. Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J. Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot. J Am Coll Cardiol 2002;40:1675–80
23. Hokanson JS, Moller JH. Significance of early transient complete heart block as a predictor of sudden death late after operative correction of tetralogy of Fallot. Am J Cardiol 2001;87:1271–7
24. Khairy P, Landzberg MJ, Gatzoulis MA, Lucron H, Lambert J, Marçon F, Alexander ME, Walsh EP. Value of programmed ventricular stimulation after tetralogy of Fallot repair: a multicenter study. Circulation 2004;109:1994–2000
25. Gatzoulis MA, Till JA, Somerville J, Redington AN. Mechanoelectrical interaction in tetralogy of Fallot: QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation 1995;92:231–7
26. Balaji S, Lau YR, Case CL, Gillette PC. QRS prolongation is associated with inducible ventricular tachycardia after repair of tetralogy of Fallot. Am J Cardiol 1997;80:160–3
27. Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD, Redington AN. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000;356:975–81
28. Therrien J, Siu SC, Harris L, Dore A, Niwa K, Janousek J, Williams WG, Webb G, Gatzoulis MA. Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot. Circulation 2001;103:2489–94
29. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham TP Jr, Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (Writing Committee to Develop Guidelines on the Management of Adults with Congenital Heart Disease): developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008;118:e714–833
30. ACOG practice bulletin: prophylactic antibiotics in labor and delivery. Int J Gynecol Obstet 2004;84:300–7
31. Wilson W, Taubert KA, Gewitz M, Lockhart PB, Baddour LM, Levison M, Bolger A, Cabell CH, Takahashi M, Baltimore RS, Newburger JW, Strom BL, Tani LY, Gerber M, Bonow RO, Pallasch T, Shulman ST, Rowley AH, Burns JC, Ferrieri P, Gardner T, Goff D, Durack DT. Prevention of infective endocarditis: guidelines from the American Heart Association. Circulation 2007;116:1736–54
32. Silversides CK, Harris L, Haberer K, Sermer M, Colman JM, Siu SC. Recurrence rates of arrhythmias during pregnancy in women with previous tachyarrhythmia and impact on fetal and neonatal outcomes. Am J Cardiol 2006;97:1206–12
33. Siu SC, Colman JM. Heart disease and pregnancy. Heart 2001;85:710
34. Saberski LR, Kondamuri S, Osinubi OYO. Identification of the epidural space: is loss of resistance to air a safe technique? A review of the complications related to the use of air. Reg Anesth 1997;22:3–15
35. Ouyang DW, Khairy P, Fernandes SM, Landzberg MJ, Economy KE. Obstetric outcomes in pregnant women with congenital heart disease. Int J Cardiol 2010;144:195–9
36. Hamlyn EL, Douglass CA, Plaat F, Crowhurst JA, Stocks GM. Low-dose sequential combined spinal–epidural: an anaesthetic technique for caesarean section in patients with significant cardiac disease. Int J Obstet Anesth 2005;14:355–61
37. Choi DH, Ahn HJ, Kim JA. Combined low-dose spinal–epidural anesthesia versus single-shot spinal anesthesia for elective cesarean delivery. Int J Obstet Anesth 2006;15:13–7
38. Bray JK, Fernando R, Patel NP, Columb MO. Suprasternal doppler estimation of cardiac output: standard versus sequential combined spinal epidural anesthesia for cesarean delivery. Anesth Analg 2006;103:959–64

Supplemental Digital Content

Back to Top | Article Outline
© 2011 International Anesthesia Research Society