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Congestive heart failure after cesarean section in a patient with congenital
myopathy
Running title: Postpartum CHF in congenital myopathy
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Abstract
Congenital myopathies constitute a family of rare congenital musculoskeletal disorders. Their
anesthetic management is challenging because of the elevated risk of malignant
hyperthermia, musculoskeletal deformity, respiratory dysfunction, and cardiac complications.
A 23-year-old woman was admitted because of premature rupture of membranes at full term
pregnancy. She had been diagnosed with the adult-onset form of congenital myopathy, but
she only presented slight motor weakness in the upper limb girdle musculature and had no
history of heart failure. Although cesarean section was performed uneventfully under spinal
anesthesia, transient congestive heart failure occurred 7 hours after the operation. The patient
responded well to restriction of fluid intake and to diuretics. Auto-transfusion due to
retraction of the uterus and sympathetic nerve stimulation induced by insufficient
postoperative pain relief might have caused this hemodynamic exacerbation by increasing
preload and afterload. Thus, we believe that appropriate anesthetic management is crucial for
these patients.
Keywords: Cesarean Section, Congenital Myopathy, Congestive Heart Failure, Spinal
Anesthesia
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Introduction
Congenital myopathies are clinical entities whose main manifestations are early-onset muscle
weakness, as well as characteristic histopathological findings on muscle biopsy and normal to
slightly elevated creatine kinase levels.1,2 According to muscular biopsy findings, they are
classified into nemaline rod myopathy, core myopathy, centronuclear myopathy, and others.
The relations between those phenotypes and genotypes have been well elucidated;2 however,
little is known about the anesthetic or obstetric management of patients with this syndrome.
In some of these patients, not only the skeletal muscle but also the cardiac muscle is
affected.3 Therefore, it is important to monitor these patients closely. Here, we present a case
of congestive heart failure after cesarean section in a patient with congenital myopathy.
Case Report
A 23-year-old woman was admitted to Osaka City University Hospital with a diagnosis of
premature rupture of membranes (PROM) at 40 weeks and 5 days of pregnancy. The
patient’s height was 156 cm and her weight was 63.1 kg (body mass index, 25.9 kg/m2; body
weight before pregnancy, 53 kg). Four years previously, she had been diagnosed by muscle
biopsy with the adult-onset form of congenital myopathy. Because of her refusal to undergo
genetic tests, the genetic subtype could not be determined. Although slight weakness of her
proximal upper limb muscle had been noted, she had not experienced any disability in daily
life or any cardiac symptoms. Her pregnancy course was uneventful until the development of
PROM. Laboratory tests on admission showed a markedly high serum creatine kinase level
(2261 IU/L) and slightly elevated serum liver enzyme levels (aspartate transaminase, 44
IU/L; alanine transaminase, 45 IU/L; lactate dehydrogenase, 318 IU/L). The preoperative
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electrocardiogram showed normal sinus rhythm, with a heart rate of 75 beats/min.
Transthoracic echocardiography performed 1 week before admission revealed left ventricular
dilatation (ratio of left ventricular diastolic diameter to left ventricular systolic diameter
[LVDd/LVDs], 54 mm / 44 mm), mild mitral regurgitation, and depressed left ventricular
systolic function (left ventricular ejection fraction [LVEF], 45%).
Initially, induced delivery with oxytocin drip infusion was planned, but the cardiotocogram
showed frequent variable deceleration, which suggested fetal distress. Therefore, a cesarean
section was scheduled to facilitate a rapid parturition.
Spinal anesthesia with 2.2 mL of 0.5% hyperbaric bupivacaine (11 mg) was administered.
Five milligrams of ephedrine was administered at 5 min and 10 min after subarachnoid
injection of bupivacaine to prevent hypotension. Once the analgesia level reached the T4
dermatome, the cesarean section procedure was initiated. The baby’s Apgar score was 8 at 1
min and 9 at 5 min after delivery. The mother’s systolic blood pressure was maintained
between 95 mmHg and 145 mmHg, and her pulse rate was between 80 beats/min and 120
beats/min throughout the operation. Fifteen units of oxytocin were administered by drip
infusion intraoperatively. The patient did not have any complaints, except for slight nausea,
which was treated with a bolus injection of 10 mg metoclopramide. The operative time was
59 min. The total amount of infused crystalloid and colloid was 1050 mL. The intraoperative
blood loss was 310 g and urine output was 500 mL.
The patient was admitted to the maternal-fetal intensive care unit. Two hours later, she
complained of wound pain, and 2 intramuscular injections of 30 mg pentazocine were
administered with a 4-hour interval. Seven hours after the operation, she complained of
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dyspnea and the oxygen saturation value dropped to 93%. Emergent chest radiography
showed remarkable bilateral hilar congestion (Figure 1). Oxygen (3 L/min) was administered
via a face mask and 10 mg of furosemide was administered intravenously. Transthoracic
echocardiography on postoperative day 1 revealed a dilated left ventricle (LVDd/LVDs,
65mm /52 mm) and decreased LVEF (40%), both of which showed a deterioration compared
to the preoperative values. Laboratory tests revealed further elevation of the serum creatine
kinase level (4074 IU/L). She was diagnosed as having congestive heart failure, and the
oxygen and furosemide administration was continued accordingly. Her oral intake of water
and food was restricted to 1.5 kg/day. By postoperative day 2, the dyspnea was resolved and
the hilar congestion observed on the chest radiograph had decreased. The serum creatine
kinase level had decreased to 2392 IU/L. On postoperative day 4, the oxygen saturation value
was maintained at over 97% on room air; therefore, the oxygen inhalation, furosemide
administration, and oral intake restriction were discontinued. The cardiothoracic ratios
obtained during postoperative chest radiography were as follows: 50.6% (postoperative day
1), 56.8% (postoperative day 2), 50.4% (postoperative day 3), 46.9% (postoperative day 4).
By postoperative day 6, no heart failure symptoms were present. The patient was discharged
from our hospital 7 days after the operation.
Discussion
The circulating blood volume of parturients can reportedly increase by up to 150% compared
to the value before pregnancy.4 Following delivery, the retraction of the uterus decreases the
vascular bed, which increases the puerperant’s cardiac preload and afterload. Although most
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puerperants can tolerate these hemodynamic changes, those who experience cardiovascular
diseases may be at risk of heart failure.
Some types of central core myopathies and nemaline rod myopathies, which involve
mutations of the ryanodine receptor type 1 gene, are thought to be associated with a high risk
of malignant hyperthermia.1 The relations between the other types of congenital myopathies
and malignant hyperthermia are not well known. Therefore, general anesthesia should be
avoided for patients with congenital myopathy. In the present report, we administered spinal
anesthesia to the patient, and noted that her cardiovascular changes during the operation
could be easily controlled. If her preoperative cardiovascular capacity had been poor, we
might have performed combined spinal epidural anesthesia, because cesarean section of a
parturient with severe cardiomyopathy can reportedly be managed successfully using this
anesthetic technique.5
Insufficient postoperative analgesia could have contributed to our patient’s congestive heart
failure by increasing preload and afterload, both of which were induced by sympathetic nerve
stimulation related to pain. Pentazocine, which was administered for postoperative pain relief,
is a mu antagonist and kappa agonist.6 Its analgesic effect is limited and it is known to have a
sympathomimetic effect.7 Therefore, it may be necessary to administer other mu agonists,
such as fentanyl or morphine, both of which have potent analgesic effect in a dose-dependent
manner,8 as well as a sympatholytic effect.9 Intrathecal morphine10 or continuous epidural
analgesia, both of which have excellent analgesic potency, might also have effectively
suppressed the postoperative pain and postpregnancy pains. In particular, epidural anesthesia
may be good option for these patients because of its sympatholytic characteristics.
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Congenital myopathies are rare, but are complicated by various kinds of musculoskeletal
disorders, such as scoliosis,11 clubfoot, and hip joint dislocation.12 The respiratory function
may also be impaired.13 Consequently, these patients may undergo multiple orthopedic
operations and may require intensive respiratory support. If a patient with this condition gets
pregnant,14 the clinician should be aware of the appropriate preoperative and perinatal
management required. Thus, we emphasize the importance of the appropriate anesthetic
management of patients with congenital myopathies in order to prevent perioperative
cardiovascular dysfunction.
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Figure legends
Figure 1. Chest radiograph 8 hours postoperatively showing bilateral hilar congestion.
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