Journal of Clinical Anesthesia (2006) 18, 221 – 223

Case report

Severe polycythemia in an infant with uncorrected

tetralogy of Fallot presenting for noncardiac surgery
Agnes I. Hunyady MD (Resident)*, Melissa A. Ehlers MD (Director)

Department of Anesthesiology, Albany Medical Center, Albany, NY, 12208, USA

Received 8 September 2004; accepted 11 August 2005

Keywords: Abstract Pediatric patients with uncorrected cyanotic congenital heart diseases may present for
Polycythemia; noncardiac surgery. Associated congenital defects and severe uncompensated secondary erythrocytosis
Secondary erythrocytosis; may complicate their anesthetic management. We describe the uncomplicated anesthetic for open G-
Uncorrected cyanotic tube placement of an ex-premature 8-month-old infant with uncorrected tetralogy of Fallot, multiple
congenital heart disease; associated congenital anomalies, and a preoperative hematocrit of 78% and review the anesthetic
Hematocrit implications of severe polycythemia.
D 2006 Elsevier Inc. All rights reserved.

1. Introduction was diagnosed with TOF and multiple other congenital

Anesthetic management of children with uncorrected
cyanotic heart disease can be very challenging, especially
when accompanied by severe uncompensated secondary
erythrocytosis. We describe the uncomplicated anesthetic
for open G-tube placement of an ex-premature 8-month-old
infant with uncorrected tetralogy of Fallot (TOF), multiple
associated congenital anomalies, and a preoperative hemat-
ocrit of 78%.
2. Case report
Our patient was an 8-month-old girl who was born at
34 weeks’ gestation with a birth weight of 1190 g. She
* Corresponding author. Department of Anesthesiology and Pain
Management, Children’s Hospital and Regional Medical Center, 4800
Sand Point Way NE, Seattle, WA 98105, USA. Tel.: +1 206 987 2000.
E-mail address: hunyadya@u.washington.edu (A.I. Hunyady).
defects, including cleft palate, severe microcephaly, and
oligodactyly. In addition, during her neonatal intensive
care unit stay, she had numerous protracted seizures and
apnea spells. Because of her multiple anomalies and bleak
neurological condition, she was discharged home to
hospice care with no plans for intervention in the future.
At the time of discharge, she was being fed solely by
nasogastric tube (secondary to her cleft palate), but by the
time she presented for surgery, she was pulling out her
feeding tube on a daily basis so gastric tube placement
was scheduled.
At the preoperative examination, she weighed 4.1 kg
and was obviously cyanotic, but in no distress with oxygen
saturations of 64% to 65% on room air. Microcephaly,
micrognatia, low set ears, short neck, and cleft palate were
all observed. On cardiac examination, she had no audible
murmur, breath sounds were clear bilaterally, and there
was no evidence of heart failure. An echocardiogram
obtained 5 weeks earlier showed TOF with a single ventri-
cular septal defect (VSD) and a very small infundibulum.

0952-8180/$ – see front matter D 2006 Elsevier Inc. All rights reserved.
doi:10.1016/j.jclinane.2005.08.012
222
The pulmonary arteries were of normal size and a small
ductus arteriosus was present with a gradient of approx-
imately 68 mm Hg; this was felt to be the primary source
of pulmonary blood flow. A patent foramen ovale was
also present with right to left flow. There was right ven-
tricular hypertrophy, but biventricular systolic function
was preserved. A complete blood count obtained during
her prescreening visit showed severe polycythemia with a
hemoglobin of 21.3 mg/dL, hematocrit of 78%, and
platelet count of 83 000/mL.
3. Anesthetic management
Because of the severe polycythemia, hospital admission
the night before surgery for intravenous hydration was
offered, but the parents declined after consultation with the
anesthesiologist. Subsequently, they were instructed to
continue hydration with pedialyte solution via the naso-
gastric tube up until 3 hours before the surgery. After the
patient was taken to the operating room, standard ASA
monitors were applied and a 24-g intravenous catheter was
placed. Because of the questionable airway status (micro-
gnatia plus cleft palate), an attempt was made at an awake
sedated endotracheal intubation, but not even the epiglottis
could be visualized (ie, grade IV view). At this point, a
cautious inhalation induction with sevoflurane in 100%
O2 was carried out using small intermittent boluses of
phenylephrine IV to maintain the Spo2 in the 60% to 70%
range. On direct laryngoscopy, the laryngeal structures still
could not be visualized, but blind orotracheal intubation
was successful. Anesthesia was maintained with sevoflur-
ane in 100% O2 and fentanyl, while controlled ventilation
and surgical access were facilitated with muscle relaxation
using rocuronium. According to the request of the parents
(after a lengthy discussion with the attending anesthesiol-
ogist), no invasive monitoring was initiated. Administra-
tion of intermittent boluses of phenylephrine were
continued to maintain the oxygen saturation in the 60%
to 70% range.
Surgery was uneventful with minimal blood loss, 85 mL
of Ringer’s lactate solution was given, and the patient was
transported intubated and ventilated to the pediatric inten-
sive care unit, where she was extubated approximately
6 hours later. Discharge to home occurred after 5 days
(receiving IV hydration until patency of the g-tube could be
proven), and she was seen 2 weeks later for a postoperative
check at which the wound was noted to be well healed. The
patient eventually died at home a few weeks later.
4. Discussion
Tetralogy of Fallot (VSD, overriding aorta, subpulmonic
stenosis, and right ventricular hypertrophy) is caused by one
single developmental abnormality: anterior displacement of
A.I. Hunyady, M.A. Ehlers
the infundibular septum. The severity of the disease
represents a spectrum ranging from minimal malalignment
of the VSD (pink tet) to severe cyanosis as a result of fixed
or dynamic right ventricular outflow tract obstruction
leading to decreased pulmonary blood flow. The dynamic
right ventricular outflow tract obstruction is most severe if
the right ventricular dimensions are small (hypovolemia),
the patient is tachycardic, and contractility is high. (For this
reason, these children are often treated with beta receptor
antagonists, typically propranolol.)
Anesthetic management of the child with TOF for
noncardiac surgery should focus on maintenance of the
pulmonary to systemic blood flow ratio by maintaining
euvolemia and normal pulmonary and systemic vascular
resistance. The latter can be achieved by using ketamine for
induction of anesthesia or administering vasopressors
(phenylephrine) in small boluses or via infusion. Continu-
ous intra-arterial blood pressure and central venous pressure
monitoring is recommended. Transesophageal echocardio-
graphy can be useful in severe cases.
On the extreme of the spectrum of children with TOF,
there is minimal or no flow through the infundibulum, and
pulmonary blood flow depends on a systemic to pulmonary
shunt flow through the persistent ductus arteriosus and/or
via major aortopulmonary collateral arteries. Unpalliated,
these children (like in our case) develop progressive
cyanosis with consequent polycythemia.
Polycythemia is defined by The Merck Manual as bA
chronic myeloproliferative disorder. . .characterized by an
increase in Hb concentration and RBC mass (eryth-
rocytosis).Q Two main types of polycythemia exist, the
brubra vera Q form, which has no known etiology, and
secondary polycythemia, which is a physiological response
to tissue hypoxia. As hypoxia stimulates erythropoietin
release from the kidneys and thus erythrocyte production in
the bone marrow, red blood cell (RBC) mass rises in an
effort to increase oxygen delivery to the tissues. Two forms
of erythrocytosis are believed to exist: a b compensatedQ
form where erythropoietin levels fall to normal after an
appropriate rise in RBC mass (and therefore oxygen
carrying capacity) and a bdecompensatedQ form where
erythropoietin levels remain elevated despite a continuously
increasing erythrocyte mass. Eventually, viscosity (of which
hematocrit is the major determinant) increases to the point
that it begins to interfere with tissue oxygenation; it has been
suggested that this form is most likely to occur in patients
with aortic O2 saturations less than 75% [1]. As hematocrit
levels rise, patients begin to complain of symptoms of
hyperviscosity such as headache, faintness/dizziness,
blurred vision, fatigue, myalgia, decreased mentation, and
chest or abdominal pain [2]. It has been noted by several
authors [1,3,4] that when iron deficiency is present,
symptoms of hyperviscosity appear at a much lower
hematocrit, presumably because iron-deficient erythrocytes
are relatively rigid. When these symptoms are present along
with a hematocrit of 60% to 65% or greater (in the absence
Severe polycythemia
of dehydration), it is recommended that isovolumic vene-
section (plus iron repletion when appropriate) be performed
in an effort to eliminate these symptoms [2]. A feared
complication of severe polycythemia is that of tissue infarct
in areas where critical sludging occurs, and many studies
have noted a correlation between hematocrit and increased
risk of cerebral and pulmonary infarcts. Although this
phenomenon may hold true for the population with the
bveraQ form of the disease, the overwhelming majority of
papers have found no evidence that increasing hematocrit
levels are associated with an increased risk of stroke in
patients with cyanotic congenital heart disease (CCHD) [2].
Besides hyperviscosity, another common disorder seen in
patients with polycythemia is a deficiency of the coagula-
tion factors II, V, VII, and IX as well as thrombocytopenia
[5]. It is felt that bsludgingQ of the RBCs (possibly most
prominently in the pulmonary microvasculature) [1] leads to
a state of low-grade DIC during which platelets and
coagulation factors are consumed, as well as on-going
fibrinolysis, and for this reason, a PT, PTT, and platelet
count should be checked preoperatively for all polycythe-
mic patients. One should bear in mind that in patients who
are severely polycythemic, although their intravascular
volume is expanded, their serum volume is relatively
diminished and thus the measured platelet count may be
artificially increased [6]. In cases in which hemostasis is
critical and polycythemia is severe, it has been reported that
venesection with 1:1 replacement using fresh frozen plasma
will improve hemostasis and platelet counts will usually
return to normal after 3 days [6].
There are no guidelines for the perioperative manage-
ment of polycythemic infants awaiting noncardiac surgery.
Avoiding dehydration is crucial. The beneficial effect of
partial exchange transfusion or venesection on the hemat-
ocrit to correct hyperviscosity and resultant coagulopathy
223
is to be weighed against the detrimental effect of possible
resultant iron deficiency. For adults with CCHD, vene-
section is not recommended for patients without symp-
toms of hyperviscosity. The presence of these symptoms
in infants can be difficult to determine. One study found a
relationship between thrombocytopenia and the severity of
clinical findings and partial exchange transfusion perfor-
mance rate in polycythemic newborns, suggesting that
thrombocytopenia might be a clinically useful marker of
hyperviscosity [7]. Its implication in infants with CCHD is
unknown. Partial exchange transfusion should be consid-
ered in infants with CCHD for noncardiac surgery with
hematocrit values greater than 0.65, depending on the type
of the planned surgery, expected blood loss, and presence
of possible coexisting congenital anomalies affecting
blood loss and hemostasis.
References
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congenital heart disease. Heart 1998;79:315 - 6.
[3] Gidding SS, Bessel M, Liao YL. Determinants of hemoglobin
concentration in cyanotic heart disease. Pediatr Cardiol 1990;11:121 - 5.
[4] Territo MC, Rosove MH. Cyanotic congenital heart disease: hemato-
logic management. J Am Coll Cardiol 1991;18:320 - 2.
[5] Henriksson P, Varendh G, Lundstrom NR. Hemostatic defects in
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