Cardiology in the Young (2013), 23, 746748 r Cambridge University Press, 2012

doi:10.1017/S1047951112001837

Brief Report

Systemic hypertension in an infant with unrepaired tetralogy

of Fallot: case report

Michael Khoury,1 Michael Kallile,2 Joseph May,2 Rajesh Punn2

1
Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada; 2Division of Pediatric
Cardiology, Lucile Packard Childrens Hospital, Palo Alto, CA, United States of America

Abstract Patients with severe right ventricular outflow tract obstruction in tetralogy of Fallot typically have
right-to-left shunting, resulting in low pulmonary blood flow and cyanosis. Here we present the case of an
infant with tetralogy of Fallot and severe pulmonary valve stenosis, complicated by systemic hypertension, the
presence of which altered flow dynamics and possibly prevented cyanosis.

Keywords: Tetralogy of fallot; hypertension; systemic vascular resistance

Received: 20 June 2012; Accepted: 22 September 2012; First published online: 13 November 2012

no umbilical artery catheter placement. His weight

T
HE VENTRICULAR SEPTAL DEFECT IN TETRALOGY OF
Fallot is almost always large and non-
restrictive, resulting in equal pressure in the
two ventricles. The direction and magnitude of flow
across the defect are primarily determined by the
relative vascular resistance and by the degree of
infundibular obstruction. Typically, if the degree of
right ventricular outflow tract obstruction is severe,
a right-to-left shunt will yield low pulmonary blood
flow and cyanosis, requiring early intervention.1,2
Here we present a unique case of an infant with
tetralogy of Fallot and severe pulmonary valve
stenosis, complicated by systemic hypertension, the
presence of which altered flow dynamics and possibly
prevented cyanosis.
Case
A term male infant antenatally diagnosed with
tetralogy of Fallot was assessed at 5 weeks of age in
our paediatric cardiology clinic in preparation for
elective surgical repair. He had been well with no
parental concerns and no noted cyanosis. No medical
interventions had been required. Specifically, there was
Correspondence to: Dr R. Punn, MD, Division of Pediatric Cardiology, Lucile
Packard Childrens Hospital, 750 Welch Road, Suite no. 305, Palo Alto, CA
94304, United States of America. Tel: 11 650 498 4563; Fax: 11 650 725
8343; E-mail: rpunn@stanford.edu
and height were at the 75th and 40th percentile,
respectively.
Four-limb blood pressure measurement in the
clinic yielded a right arm pressure of 140/95, left
arm of 137/80, right leg of 140/80, and left leg
of 137/89. On the basis of age, gender, and size,
the definition of hypertension in this patient
was ,104 mmHg systolic.3 His heart rate was
162 beats/min, pulse oxygenation was 100% on
room air, and the child was afebrile. Auscultation
revealed a harsh systolic ejection murmur (III/VI) at
the left upper sternal border radiating to both lungs.
Electrocardiogram showed normal sinus rhythm and
right ventricular hypertrophy with an axis at 2188.
Echocardiogram showed an anterior-malaligned
ventricular septal defect that was unrestrictive in
nature, an overriding aorta, severe deviated conal
septum-type infundibular stenosis, severe pulmon-
ary valve stenosis, mild right ventricular hypertro-
phy, and normal left ventricular function with no
coarctation of the aorta. The pulmonary valve was
bicuspid, the annulus was 5 mm (Z score 24.5)4 in
diameter, and the right ventricular outflow tract
gradient was 147 mmHg. There was a patent
foramen ovale with left-to-right shunting. The
right pulmonary artery measured 4 mm (Z score 23),4
and the left pulmonary artery measured 3 mm
Vol. 23, No. 5 Khoury et al: Tetralogy of Fallot with systemic hypertension 747

Figure 1.
(a) Parasternal long-axis view in diastole demonstrates the ascending aorta (AAo) overriding the crest of the interventricular septum (*),
a classic depiction of tetralogy of Fallot. (b and c) Aliasing colour Doppler flow is seen across the right ventricular outflow tract (RVOT)
with a peak systolic velocity over 5 m/s (peak systolic gradient of over 130 mmHg). (d) On this parasternal short-axis view, the conal septum
is displaced anterior causing constriction across the RVOT (*) with a large ventricular septal defect (VSD) noted more posteriorly. The
pulmonary valve (PV) appears significantly smaller than the aortic valve (AoV). (e) This apical five-chamber view shows unobstructed left
to right flow across the VSD and the left ventricular outflow tract into the AAo. The low velocity red colour flow across the large VSD along
with the very elevated RVOT gradient depict elevated left ventricular (LV) pressure, consistent with systemic hypertension. RV 5 right
ventricle; LA 5 left atrium; RA 5 right atrium; TV 5 tricuspid valve; PV 5 pulmonary valve; AoV 5aortic valve.

(Z score 24.6)4 (Fig 1). The apical five-chamber
view showed left to right flow across the ventricular
septal defect. The patient was hypertensive during
the echocardiogram, with four-limb blood pressures
similar to those noted above. Of note, the right
ventricular outflow tract gradient at a community
cardiologists clinic about a week prior was
,70 mmHg; however, no blood pressure measure-
ment was obtained at the time.
The infant was admitted for pre-operative
evaluation of his systemic hypertension. Anti-
hypertensives were not initiated to prevent a sudden
decrease in pulmonary blood flow secondary to a
decrease in systemic vascular resistance. Pre-operatively,
the patient had a number of episodes of desaturation,
typically occurring when the systolic blood pressure
would drop to 80100 mmHg (3050 mmHg lower
than his typical mean systolic blood pressure levels).
As part of the evaluation for the patients
hypertension, the following tests measured in the
normal range: creatinine, blood urea nitrogen,
sodium, potassium, aspartate and amino transami-
nase, thyroid-stimulating hormone, free T4 levels,
serum aldosterone, and renin. Urine studies for
pheochromocytoma were also normal. Renal Doppler
ultrasound revealed no evidence of renal artery
stenosis, and both kidneys were normal in cortical
echogenicity and corticomedullary differentiation. The
right adrenal gland was visualised and was grossly
normal. The left adrenal was not visualised. Head
ultrasound was normal and did not reveal any
intracranial masses. Fluorescence in situ hybridisation
was negative for 22q11 microdeletion. Although
advanced imaging techniques may have revealed more
subtle renovascular abnormalities, these imaging
modalities were not considered to be part of the
standard of care in the immediate evaluation of
neonatal hypertension. In addition, owing to the
fragile nature of the patient pre-operatively, such
advanced imaging modalities were deferred in
favour of maintaining the patients clinical stability
and arranging urgent surgical repair.
The patient was taken to the operating room for a
complete tetralogy of Fallot repair, involving patch
closure of the ventricular septal defect, trans-annular
right ventricular outflow tract patch augmentation,
748 Cardiology in the Young
creation of a mono-cusp pulmonary valve, infundibu-
lum muscle resection, and ligation and division of the
ductus arteriosus. The patient tolerated the procedure
well without residual defects. Post-operatively, the
patient continued to have hypertension that was
gradually controlled with captopril with resultant
systolic blood pressures of 8090 mmHg and diasto-
lics of 4060 mmHg. The systolic blood pressure
while asleep decreased to 70 mmHg consistently
such that captopril could not be titrated to above
0.5 mg/kg per day, and it was eventually discontinued.
Therefore, further evaluation of the pre-operative
hypertension was not pursued. The patients desatura-
tion episodes ceased post-operatively.
Discussion
To the best of the authors knowledge, this is the
first reported case of systemic hypertension in a
young infant with unrepaired tetralogy of Fallot.
Systemic hypertension could not be adequately
managed in this setting given the possibility of a
hypercyanotic spell.
Much like infants and older children, the causes
of hypertension in neonates are vast,5 with the two
most common aetiologies being renovascular and
renal parenchymal diseases. Other possible causes of
neonatal hypertension include aortic coarctation,
idiopathic arterial calcification, subdural haematomas,
hyperaldosteronism, and adverse events related to
umbilical artery catheter insertion.5 We were unable
to find an aetiology for our patients pre-operative
hypertension despite extensive investigations.
An increase in systemic blood pressure has previously
been reported to improve arterial oxygenation and
recovery from anoxic spells in tetralogy of Fallot.68
Intravenous phynylephrine is a well-documented
therapeutic intervention to raise systemic vascular
resistance and consequently increase flow through
the right ventricular outflow tract and improve
arterial oxygenation.9
This case carries with it a number of clinical
considerations for the physician to be aware of.
Primarily, the absence of cyanosis does not exclude
the presence of severe right ventricular outflow
October 2013
tract obstruction. These patients should be con-
sidered for early surgical repair. Secondarily, the
clinician should be cautious with respect to the
management of systemic hypertension in a patient
with tetralogy of Fallot. Decreasing the systemic
vascular resistance may decrease pulmonary blood
flow, inducing hypoxia.
Conclusion
We report the first case of an infant with unrepaired
tetralogy of Fallot and systemic hypertension of
undetermined aetiology. The elevated systemic
vascular resistance presumably allowed adequate
pulmonary blood flow despite a severe right ventri-
cular outflow tract obstruction. This case shows that
the clinician should carefully consider the role that
systemic blood pressure plays in tetralogy of Fallot
physiology.
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