Anesth Analg 2008; 107:1652-1654
© 2008 International Anesthesia Research Society
doi: 10.1213/ane.0b013e3181864d6e
OBSTETRIC ANESTHESIOLOGY
The Anesthetic Management for Cesarean Delivery in a Patient with Shone's Syndrome
Kathleen Sachse, MD, and
Medhat Hannallah, MD, FFARCS
From the Department of Anesthesiology, Georgetown University School of Medicine, Washington, DC.
Address correspondence to Medhat Hannallah, MD, FFARCS, Department of Anesthesia, Georgetown University Hospital, 3800 Reservoir Rd., NW, Washington DC 20007. Address e-mail to hannallm{at}georgetown.edu.
Abstract
Shone's syndrome is a rare congenital cardiac condition that consists of up to four obstructive left-sided cardiac lesions. We report a 17-yr-old nullipara with Shone's syndrome who presented for cesarean delivery. She had mild mitral stenosis and mild left ventricular outflow tract obstruction. Epidural anesthesia was attempted, but the patient developed severe hypotension associated with fetal bradycardia necessitating immediate cesarean delivery under general anesthesia. The peripartum anesthetic and management considerations for these patients are discussed.
Shone's syndrome is a congenital cardiac condition that consists of up to four obstructive left-sided cardiac lesions: supravalvular mitral ring, parachute deformity of the mitral valve, subaortic stenosis, and coarctation of the aorta.1 Both a complete form (all four lesions), and an incomplete form (fewer than four lesions) have been described.2 It is an extremely rare syndrome, especially in pregnant women.3 We describe an adolescent patient with Shone's syndrome who presented for elective cesarean delivery. This report describes attempted epidural anesthesia complicated by severe maternal hypotension and fetal bradycardia necessitating immediate cesarean delivery in a patient with Shone's syndrome.
CASE REPORT
A 17-yr-old primigravida presented for an elective cesarean delivery at 39 wk gestation. The patient was known to have Shone's syndrome consisting of a parachute mitral valve with mild mitral stenosis, a bicommissural aortic valve, and a subaortic spur associated with mild left ventricular outflow tract obstruction. She had been followed in a congenital heart clinic since age 2 yr. She was asymptomatic during childhood and no interventions were necessary.
At age 14 yr, the patient presented with fatigue primarily with exercise. Echocardiograms with and without a treadmill stress test showed mild mitral and aortic stenosis with excellent biventricular function. The findings were reviewed by other pediatric centers. No further interventions were recommended based on the hemodynamic data and the conclusion was that the patient's symptoms were not related to her cardiac pathology.
At age 16 yr, the patient was seen at the pediatric cardiology clinic at 17 wk gestation. Since the start of her pregnancy the patient complained of palpitations and fatigue. She had two pillow orthopnea. She was 162-cm tall and weighed 62 kg. Her heart rate was 72 bpm and her arterial blood pressure (BP) measured 100/61 mm Hg. Apart from a grade 3/6 aortic stenotic murmur heard along the upper left sternal border, her physical examination was essentially normal, including clear lungs, normal jugular venous pulse, and absence of peripheral edema. Because of concern that her new symptoms of fatigue and orthopnea could have been caused by fluid retention, daily digoxin 0.125 mg and furosemide 20 mg were prescribed. Her symptoms improved.
An echocardiography performed at 26 wk gestation showed a mean mitral valve gradient of 6 mm Hg, no evidence of pulmonary hypertension, 22 mm Hg mean and 36 mm Hg peak systolic gradients across the subaortic region.
Consultation between the obstetrician and congenital heart cardiologist at 36-wk gestation resulted in a decision for cesarean delivery. The decision was based upon the multiple level heart obstruction and symptoms associated with exercise during pregnancy.
On admission the patient's BP measured 103/51 mm Hg, her heart rate was 93 bpm, and room air oxygen saturation was 98%. Her physical examination was unchanged. The electrolytes, red blood cell, and platelets counts were normal. The electrocardiogram showed normal sinus rhythm. After detailed discussion with the patient and her mother, epidural anesthesia with invasive arterial and central venous pressure monitoring was planned; however, noninvasive BP monitoring (1-min intervals) was used during placement of the epidural catheter and administration of the initial epidural dose. Fetal (FHR) and maternal heart rate and maternal pulse oximetry were continuously monitored. Lactated Ringer's solution, 500 mL was infused and oxygen by nasal cannula was administered during the epidural procedure. The epidural catheter was placed in the L3–4 interspace with the patient in the sitting position using the loss-of-resistance to air technique. Lidocaine 20 mg/mL with epinephrine 5 µg/mL 1.5 mL, was injected through the Tuohy needle after a negative aspiration test. A multiorifice epidural catheter was inserted and secured 5 cm into the epidural space. After a negative aspiration test, an additional 1.5 mL of the lidocaine/epinephrine solution was injected through the epidural catheter. The patient was positioned supine with left uterine displacement. In the absence of any change in vital signs or of any sensory or motor changes, an additional 3 mL of lidocaine/epinephrine solution combined with fentanyl 100 µg was injected through the epidural catheter. Three minutes after the second epidural injection, while preparations for the arterial line placement were underway, the patient's BP decreased to 82/30 mm Hg and her heart rate increased to 117 bpm. IV phenylephrine 100 µg was administered and an IV bolus infusion of lactated Ringer's solution was initiated. One minute later, the patient's BP continued to decrease to 54/30 mm Hg and the FHR decreased rapidly to approximately 60 bpm. The patient's systolic BP remained between 56 and 69 mm Hg for approximately 3 min before it returned to baseline after the administration of additional phenylephrine 300 µg and ephedrine 10 mg, and turning the patient completely on her left side. During that time she remained in sinus rhythm and her heart rate ranged between 90 and 110 bpm. As the FHR remained slow despite recovery of the maternal BP, the decision was made to immediately deliver the baby. Since sensory testing revealed inadequate anesthesia, rapid-sequence induction of general anesthesia with cricoid pressure was immediately performed using sodium thiopental 100 mg and succinylcholine 120 mg. The cesarean delivery was rapidly performed and a healthy infant (1- and 5-min Apgar scores of 9 and 9, respectively; umbilical cord arterial blood pH: 7.13) was delivered 7 min after the initial decrease in FHR. The patient remained hemodynamically stable during the remainder of the 90-min procedure, and throughout the postpartum period. Neuroblockade could not be demonstrated upon emergence from general anesthesia.
DISCUSSION
Multilevel obstruction of left heart structures was initially described by Shone et al.1 Their original description included four defects: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Only two of the eight patients they described had all four lesions, the rest presented with an incomplete form of the syndrome. Literature about patients with Shone's syndrome is scarce, consisting mainly of pediatric case reports.4 The majority of patients present initially in the neonatal period with coarctation of the aorta as the predominant outflow obstruction lesion. Patients with Shone's syndrome are seen with a wide spectrum of major anatomical and hemodynamic abnormalities and a wide variety of clinical presentations and possible outcomes which makes formulation of appropriate management strategies difficult.5–9 Mild forms are managed conservatively, and the syndrome has been discovered incidentally in adults.6,7 More severe cases require surgical repair, possibly consisting of multiple procedures.8–10 The severity of mitral valve disease seems to be the predominant factor affecting outcome.
We did not identify previous reports of epidural anesthesia for cesarean delivery in a patient with Shone's syndrome. We identified two reports of pregnant patients with Shone's syndrome. The first case was that of a 29-yr-old woman diagnosed with Shone's syndrome (which included coarctation of the aorta complicated by a 65 mm ascending aortic aneurysm) at 31 wk gestation.7 At 35-wk gestation, the patient underwent combined cesarean delivery, surgical repair of the coarctation, replacement of the aortic valve, and ascending aorta under general anesthesia.
The second case was that of a 15-yr-old female who presented at 25 wk gestation with dyspnea, orthopnea, and paroxysmal nocturnal dyspnea.3 She was hypertensive and her chest radiograph showed pulmonary edema. A transthoracic echocardiogram showed mitral stenosis with a mean gradient of 22 mm Hg and coarctation of the aorta with a gradient of 70 mm Hg. The patient responded well to aggressive medical management. Labor was induced at 37-wk gestation with invasive BP monitoring. The course of labor and delivery were described as being uncomplicated; however, anesthetic/analgesic management was not discussed.
The physiologic changes during pregnancy may have profound implications for patients with heart disease. These changes include an increase in plasma volume, decrease in systemic vascular resistance, as well as an increase in heart rate, stroke volume and cardiac output. In general, regurgitant valvular lesions are well tolerated during pregnancy because of the favorable hemodynamic effects of tachycardia and afterload reduction.11,12 In contrast, stenotic or obstructive lesions, such as those present in patients with Shone's syndrome, are less well tolerated because of the fixed stroke volume and the detrimental effect of tachycardia.11,12 Assessment of the pregnant patient with heart disease may be complicated by the normal anatomical and functional changes in the cardiovascular system during pregnancy.13 These changes may result in signs and symptoms that may mimic heart disease, such as fatigue, shortness of breath, palpitations, dizziness, and edema. It is possible that these normal changes were at least partly responsible for the symptoms experienced by our patient whose echocardiographic examinations consistently revealed mild stenotic lesions with good ventricular function. However, concern about the possibility of cardiac decompensation during prolonged labor prompted the recommendation to proceed with cesarean delivery. An alternative obstetrical management plan consisting of vaginal delivery with appropriate analgesia and shortened second stage has been recommended by others, reserving cesarean delivery for obstetrical indications and for patients with cardiac instability.12,13
The optimal-anesthetic technique for cesarean delivery in patients with stenotic valvular lesions is controversial.12–16 Neuraxial anesthesia and analgesia may lead to afterload reduction, hypotension, and tachycardia. Tachycardia decreases the time available for left ventricular filling in patients with mitral stenosis and decreases stroke volume and coronary filling time in patients with aortic stenosis. Acute reduction in afterload can severely compromise coronary perfusion. Alternatives to neuraxial techniques may also have side effects. Inadequate labor analgesia is associated with hypertension and tachycardia. General anesthesia may be associated with sympathetic stimulation in response to endotracheal intubation. Reports describing the successful use of neuraxial anesthesia and analgesia for vaginal and cesarean delivery in patients with aortic stenosis and other valvular heart lesions emphasized the importance of using an incremental technique and avoidance of aorto-caval compression to maintain preload and avoid acute decrease in afterload.12–17 Continuous monitoring of arterial and central venous or pulmonary artery pressures may permit immediate diagnosis and rapid correction of hypotension and guide IV fluid and vasopressors administration.
Despite these reports of the successful use of neuraxial anesthesia and analgesia in patients with stenotic valvular lesions during both vaginal and cesarean deliveries, it is important to appreciate that the risks and benefits of neuraxial analgesia/anesthesia differ between the two modes of delivery. For vaginal delivery, there are no good alternatives to neuraxial analgesia because other forms of analgesia are ineffective and do not prevent tachycardia. In contrast, cesarean delivery can be performed with general anesthesia and thus might be the anesthetic of choice for elective delivery.
The etiology of the rapid and profound hypotension after the epidural injection of a small dose (6 mL) of lidocaine/epinephrine and fentanyl in our patient is not completely clear. It may have been precipitated by hypovolemia in a patient with a mild degree of fixed cardiac output. The hypovolemia may have been exaggerated by the use of diuretics, inadequate intravascular volume loading, and aorto-caval compression. Other possible causes of the hypotension include unrecognized subarachnoid or subdural injection of the local anesthetics. The lack of surgical anesthesia at the time of fetal bradycardia and emergence of general anesthesia suggests that subarachnoid injection was unlikely. Subdural injection of local anesthetics may lead to extensive and patchy neuroblockade and cannot be excluded. Its onset, however, is typically delayed.18 Tachycardia associated with accidental intravascular injection of the epinephrine-containing anesthetic solution could have been responsible for a reduction in cardiac output leading to hypotension.
In summary, we describe the attempted use of epidural anesthesia for cesarean delivery in a patient with Shone's syndrome. Although it is not clear that invasive monitoring would have prevented the observed complication in our patient, if invasive monitoring is planned, it makes sense to initiate monitoring before the initiation of anesthesia in order to more rationally titrate fluid and anesthetic administration and treat complications. Additionally, our management can be criticized for the administration of sodium thiopental for induction of general anesthesia in a hypotensive patient with stenotic heart disease, and for the use of an epinephrine-containing local anesthetic solution. The 32nd Bethesda Conference recommendations for the care of obstetrical patients with congenital heart disease state that these patients should be managed by a multidisciplinary team including an obstetrician, cardiologist, anesthesiologist, and pediatrician. The team should meet early in the patient's pregnancy to review the cardiac lesions, anticipated effects of pregnancy, and potential problems, and develop a management plan regarding the timing and mode of delivery, the type of anesthesia/analgesia, and the need for hemodynamic monitoring before and after delivery.19 Such a comprehensive approach was not pursued in this case and may have contributed to the adverse events associated with our patient's delivery.
Footnotes
Accepted for publication June 25, 2008.
No external funding was provided.
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