CATECHOLAMINE EXCESS
Pheochromocytoma is a catecholamine-secreting
tumor that consists of cells originating from the embryonic neural crest. This
tumor accounts for 0.1% of all cases of hypertension. Although the tumor is
usually localized in a single adrenal gland, 10–15% are bilateral or
extraadrenal. Approximately 10% of tumors are malignant. The cardinal
mani-festations of pheochromocytoma are paroxysmal hypertension, headache,
sweating, and palpitations. Unexpected intraoperative hypertension and
tachy-cardia during manipulation of abdominal structures may occasionally be
the first indications of an undi-agnosed pheochromocytoma. The pathophysiology,
diagnosis, and treatment of these tumors require an understanding of
catecholamine metabolism and of the pharmacology of adrenergic agonists and
antag-onists.
Preoperative assessment should focus on the
adequacy of α-adrenergic blockade and volume replacement. Specifically,
resting arterial blood pressure, orthostatic blood pressure
and heart rate, ventricular ectopy, and
electrocardiographic evi-dence of ischemia should be evaluated.
A decrease in plasma volume and red cell mass
contributes to the severe chronic hypovolemia seen in these patients. The
hematocrit may be normal or elevated, depending on the relative contribution of
hypovolemia and anemia; thus neither hemato-crit nor hemoglobin concentration
reliably defines the adequacy of intravenous volume. Preoperative α-adrenergic blockade with phenoxybenzamine(a
noncompetitive inhibitor) helps correct the vol-ume deficit, in addition to
correcting hypertension.
Blockade should not be initiated
prior to initia-tion of α blockade
but may be added if there is a need to control heart rate and to reduce
arrhythmias provoked by excess catecholamine concentrations. A drop in
hematocrit should accompany the expan-sion of circulatory volume, sometimes
unmasking an underlying anemia.
Potentially life-threatening variations in blood pressure—particularly
during induction and manipulation of the tumor—indicate the useful-ness of
invasive arterial pressure monitoring and of adequate intravenous access. Young
patients with minimal or no heart disease may not need a cen-tral venous line.
Patients with evidence of cardiac disease (or in whom cardiac disease is
suspected) may benefit from having a central line (a convenient route of access
for administering catecholamines, should they be required) and from
intraoperative transesophageal echocardiography.
Intubation should not be attempted until a
deep level of general anesthesia (possibly also including local anesthesia of
the trachea) has been established. Intraoperative hypertension can be treated
with phentolamine, nitroprusside, nicardip-ine, or clevidipine. Phentolamine
specifically blocks α-adrenergic receptors
and blocks the effects ofexcessive circulating catecholamines. Nitroprusside
has a rapid onset of action, a short duration of action, and as a nitric oxide
donor can be effective in cases where calcium channel blockers are
ineffec-tive. Nicardipine and clevidipine are being used more frequently
preoperatively and intraoperatively.
Drugs or techniques that indirectly stimulate
or promote the release of catecholamines(eg,
ephedrine, hypoventilation, or large bolus doses of ketamine), potentiate the
arrhythmic effects of catecholamines (classically halothane), or consis-tently
release histamine (eg, large doses of atracu-rium or morphine sulfate) are best
avoided.
After ligation of the tumor’s venous supply,
the primary problem frequently becomes hypotension
from hypovolemia, persistent adrenergic blockade, and tolerance to the high
levels of endogenous cat-echolamines that have been abruptly withdrawn.
Appropriate fluid resuscitation should reflect surgical bleeding and other
sources of fluid loss. Assessment of intravascular volume can be guided by
echocardiographic assessment of left ventricular filling using transesophageal
echocardiography or other noninvasive measures of cardiac output and stroke
volume. Infusions of adrenergic agonists, such as phenylephrine or
norepinephrine, often prove necessary. Postoperative hypertension is rare and may indicate the presence of unresected
occult tumors.
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