166

Editorial Comment
Is There a Choice of Palliation for
Tetralogy of Fallot?*
ROBERT J. SOMMER, MD,
RICHARD J. GOLINKO, MD, FACC
New York, New York
The surgical management of symptomatic infants with tetral-
ogy of Fallot remains controversial. Several centers (1-4)
have achieved acceptably low mortality and morbidity rates
using primary correction at the time of diagnosis. However,
the unknown long-term consequences of early right ventric-
ulotomy and trans annular patch placement leave the issue of
optimal therapy largely unresolved. Most centers continue
to provide palliative treatment for these infants with some
type of aortopulmonary shunt and perform definitive correc-
tive surgery in older children. In this issue of the Journal,
Sreeram et al. (5) present their results with balloon pulmo-
nary valvuloplasty performed as an alternative palliative
procedure to aortopulmonary shunting in 67 infants with
tetralogy of Fallot.
Indications for surgery. Infants with tetralogy of Fallot
may manifest symptoms in two ways. First, they may have
cyanosis at rest because of right to left shunting at the
ventricular septal defect. This is of concern because chronic
hypoxemia appears to be related to lower intelligence (6) and
results in polycythemia with its attendant risks (7). The most
urgent danger to these patients, however, is the classic
cyanotic "spell" of tetralogy of Fallot. Infundibular spasm
and sudden increase in right ventricular outflow obstruction
contribute to decreased pulmonary blood flow (8). A marked
fall in systemic oxygen content follows, with a risk of
syncope, seizures and sudden death. This is a life-
threatening emergency and in most centers is considered an
indication for immediate surgical intervention.
The classic palliative procedure for these patients has
been the Blalock-Taussig shunt (subclavian artery to pulmo-
nary artery anastomosis). Systemic to pulmonary artery
flow, across the shunt, results in a higher level of oxygen
saturation at rest through the augmentation of anterograde
pulmonary blood flow. More important, in the presence of an
alternate source of pulmonary blood flow, acute infundibular
obstruction is no longer a life-threatening event.
"Editorials published in Journal of American College of Cardiology reflect
the views of the authors and do not necessarily represent the views of JACC
or the American College of Cardiology.
From the Division of Pediatric Cardiology, Mount Sinai Medical Center,
New York, New York.
Address for reprints: Richard J. Golinko, MD, Director, Pediatric Cardi-
ology, Mount Sinai Medical Center, Box 1201, One Gustave L. Levy Place,
New York, New York 10029.
1991 by the American College of Cardiology
JACC Vol. 18, No.1
July 1991:166-7
Role of balloon valvuloplasty. Because valvular stenosis
contributes significantly to the degree of right ventricular
outflow tract obstruction in the patient with tetralogy of
Fallot, balloon valvuloplasty may increase pulmonary blood
flow and systemic oxygen saturation at rest. Balloon pulmo-
nary valvuloplasty has been proved a safe and efficacious
procedure for the treatment of isolated valvular pulmonary
stenosis in infants and children (9). However, the obstruc-
tion to pulmonary flow in tetralogy of Fallot is complex and
may include subvalvular (infundibular), valvular, supraval-
vular and often peripheral stenotic components. Although
balloon angioplasty may also provide palliative treatment for
supravalvular and peripheral lesions (10), it has no direct
impact on the degree of infundibular stenosis. The latter is
due anatomically to the anterior malalignment of the inter-
ventricular septum.
Therefore, the underlying premise of balloon valvulo-
plasty for tetralogy of Fallot must be questioned. The risk of
"spells" remains real, regardless of the systemic oxygen
saturation at rest, as a result of potential subvalvular com-
promise of pulmonary blood flow.
The present study. The 67 patients of Sreeram et al. (5)
were grouped retrospectively by outcome. In two patients
the anulus could not be crossed with the catheter and
aortopulmonary shunting was performed; 65 patients under-
went balloon dilation. The investigators (5) did not mention
the occurrence of cyanotic spells during the catheterization
procedure, a frequent complication of angiographic studies
of patients with tetralogy of Fallot. Of the 35 patients who
were restudied angiographically at a later date, 11 (group B)
had undergone aortopulmonary shunting secondary to "in-
adequate palliation." Sreeram et al. (5) did not discuss the
clinical indications for surgical intervention. Did these chil-
dren have life-threatening cyanotic spells? The other 24
patients (group A) were considered to have "adequate
palliation." However, in 10 of these patients medical ther-
apy with propranolol had been initiated because of recurrent
cyanotic spells that were presumably not severe enough to
warrant surgical intervention. Of the 30 patients who were
not yet restudied (group C), 3 were being treated medically
for cyanotic episodes, 7 had received a surgical shunt and 5
died during the follow-up period. The cause of death in three
of these five was not mentioned.
With beta-adrenergic blocking therapy, the frequency and
severity of cyanotic episodes are lessened but not elimi-
nated. Therefore, the patients on medical therapy must also
be considered to have had an unsuccessful palliative proce-
dure. When the patients who required beta-blocker therapy
are grouped with those who underwent surgical palliation,
the failure rate among the 67 patients is 57%. More signifi-
cant, the authors (5) were unable to distinguish prospectively
the patients who would require subsequent surgical pallia-
tion or beta-blocker therapy. Thus, there was no way to be
certain that a given child would be adequately protected
from the potential complications of a cyanotic spell.
0735-1097/911$3.50
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JACC Vol. 18, No.1
July 1991:166-7
Implications. The growth of the pulmonary anulus in this
series of patients (5) should be noted. Adequate annular
growth would be of great benefit because it might obviate the
need for a transannular patch at a later surgical correction.
From this perspective, perhaps balloon valvuloplasty could
be incorporated as part of an overall palliative schema after
an aortopulmonary shunt. Valvuloplasty itself would cer-
tainly be safer in the patient after a shunt and would avoid
the danger of "spells" in the catheterization laboratory.
Although some patients treated in this manner would de-
velop congestive heart failure secondary to excessive pul-
monary blood flow and some would develop significant
pulmonary insufficiency, the potential benefit of avoiding a
transannular patch might outweigh both of these risks.
Conclusions. The work by Sreeram et al. (5) is an inter-
esting new application of an established palliative technique.
However, balloon pulmonary valvuloplasty in patients with
tetralogy of Fallot provides inadequate control of pulmonary
blood flow. These patients remain at risk for cyanotic spells
with the possibility of neurologic injury or sudden death, or
both. Valvuloplasty should not be considered an alternative
to surgical palliation.
SOMMER AND GOLINKO
EDITORIAL COMMENT
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