Student Paper Communication tudiante

A case of valvular pulmonic stenosis and an aberrant coronary artery

in a Brittany spaniel
Chelsie Estey

Abstract Valvular pulmonic stenosis and aberrancy of the right coronary artery with subsequent subvalvular
stenosis was found on echocardiographic evaluation of a 9-month-old Brittany spaniel. Previous echocardiography
at 4 mo of age revealed the pulmonic stenosis; however, the aberrant coronary artery only became apparent during
the second evaluation.

Rsum Cas de stnose valvulaire pulmonaire et dartre coronarienne aberrante chez un pagneul breton.
Une stnose valvulaire pulmonaire et une aberration de lartre coronarienne droite avec une stnose subvalvulaire
subsquente ont t constates lvaluation chocardiographique dun pagneul breton g de 9 mois. Une
chocardiographie antrieure lge de 4 mois avait rvl la stnose pulmonaire; cependant, lartre coronarienne
aberrante est seulement devenue manifeste la deuxime valuation.
(Traduit par Isabelle Vallires)
Can Vet J 2011;52:541543

A 9-month-old castrated male, Brittany spaniel was presented

to the cardiology service at the Atlantic Veterinary College
for a recheck evaluation of moderate valvular pulmonic stenosis.
The dog initially had been referred for a cardiac evaluation at
4 mo of age following auscultation of a heart murmur during
a routine examination. Pulmonic stenosis was diagnosed via
echocardiography during the initial cardiac work-up.
On presentation, the physical examination revealed a bright,
alert, and responsive puppy with a normal body condition
(BCS 3/5). Temperature, pulse, and respiratory rate were within
normal limits. Mucous membranes were pink and moist and
capillary refill time was , 2 s. Cardiac auscultation revealed
a grade IV/VI systolic heart murmur with a point of maximal
intensity heard at the left parasternum. Heart rate and rhythm
were normal and femoral pulses were strong and regular. Normal
bronchovesicular lung sounds were heard in all lung fields. The
owner reported no change in the dogs behavior, demeanor, or
energy level. The puppy was doing very well and was free of
clinical signs.
Atlantic Veterinary College, 550 University Avenue,
Charlottetown, Prince Edward Island C1A 4P3.
Address all correspondence to Chelsie Estey; e-mail:
cmestey@upei.ca
Ms. Estey will receive 50 copies of her article free of charge,
courtesy of The Canadian Veterinary Journal.
Use of this article is limited to a single copy for personal study.
Anyone interested in obtaining reprints should contact the
CVMA office (hbroughton@cvma-acmv.org) for additional
copies or permission to use this material elsewhere.
A complete echocardiographic examination of the heart was
performed to determine possible progression of the pulmonic
stenosis. Right and left parasternal long axis and transverse
images were obtained. Pulmonic valves were examined on mul-
tiple views to determine their complete anatomic structure. The
right ventricular outflow tract, pulmonic valve, and pulmonary
trunk were thoroughly evaluated for the presence of an aberrant
coronary artery, which would manifest as a hypoechoic linear
structure crossing in this area. Continuous-wave Doppler echo-
cardiography was used to determine the pressure gradient across
the pulmonic valve from the right parasternal short axis view.
Echocardiographic examination revealed mild concentric
right ventricular hypertrophy. The pulmonic valve leaflets were
fused, creating a domed valve morphology. Moderate post-
valvular dilation of the main pulmonary artery was present.
An aberrant right coronary artery was seen across the right
ventricular outflow tract, creating mild subvalvular stenosis
(Figure 1). No masses or effusion were seen. Color doppler
imaging displayed marked turbulence across the main pulmo-
nary artery and mild pulmonic insufficiency (Figure 2). The
right ventricular to pulmonary artery pressure gradient was
38 mmHg (42 mmHg 5 mo previously). These findings
were consistent with moderate compensated valvular pulmonic
stenosis and the novel finding of subvalvular pulmonic stenosis
secondary to an aberrant right coronary artery.
Based on the fact that the patient had well-compensated
disease and the pulmonic stenosis had not progressed since the
initial examination, it was concluded that the disease process was
presently stable. Reassessment in 4 to 8 mo was recommended to
further evaluate the progression of his cardiac disease. The owner
was advised to observe for signs of lethargy, weakness, difficulty

CVJ / VOL 52 / MAY 2011 541

C O M M U N I CAT I O N T U D I A N T E
Figure 1. Right parasternal transverse image illustrating the
aberrant right coronary artery (arrow) via echocardiography.
breathing, exercise intolerance, or abdominal distention, all of
which could be indicators of a worsening in the disease process.
Discussion
Pulmonic stenosis is among the most common congenital car-
diac defects in dogs (1). This defect is particularly common in
the English bulldog, bull mastiff, fox terrier, samoyed, cocker
spaniel, and west highland white terrier (14). A hereditary
form has been detected in certain breeds, such as the Boykin
spaniel (5).
Pulmonic stenosis is a condition in which the right ven-
tricular outflow tract is narrowed, impeding flow from the right
ventricle. This narrowing elicits concentric right ventricular
hypertrophy, which increases pressure in the right ventricle
during ejection of blood. The degree of hypertrophy is related
to the severity of the stenosis. The resulting ventricular lumen
is decreased in size and the right ventricle has reduced diastolic
compliance and filling capacity.
Animals affected with pulmonic stenosis may be completely
free of clinical signs in cases of well-compensated disease. These
patients may present simply with a systolic murmur heard at the
level of the left heart base, in the area of the third intercostal
space. Moderately affected animals may present as exercise
intolerant or with a history of syncope or collapse. Conversely,
severely affected patients may show signs of right-sided heart
failure, such as hepatomegaly and ascites.
Diagnosis of pulmonic stenosis can be achieved using a
combination of diagnostic tools. Thoracic radiographs may
show right-sided heart enlargement or a bulge at the level of the
main pulmonary artery. Conversely, abdominal radiographs may
show evidence of hepatomegaly or ascites in severely affected
animals. Echocardiography is the best modality for diagnosing
pulmonic stenosis as it is noninvasive, sensitive, and accurate.
Right-sided heart enlargement, post-stenotic dilation of the
main pulmonary artery and right ventricular hypertrophy may
be present (6). An echocardiogram will allow direct visualiza-
tion of the defect as well as determination of pressure gradients
across the pulmonic valve (6). Echocardiography will also permit
542
Figure 2. Right parasternal transverse image illustrating
regurgitant flow across the pulmonic valve with color Doppler
echocardiography.
determination of the type of pulmonic stenosis and the presence
of any concurrent anomalies (7). Given that the diagnosis was
initially made at a young age in this patient, it is possible that
there would be worsening of the condition, which would be
more obvious using echocardiography as the dog approached
1y of age. Angiography can be used during corrective surgery
to obtain better visualization of the defect; however, this is a
not a method routinely used for initial diagnosis of pulmonic
stenosis or other cardiac anomalies.
Dogs with mild to moderate stenosis can have well-
compensated disease and show no clinical signs, allowing them
to live a normal life span. Doppler-derived right ventricular to
pulmonary artery pressure gradients of , 50 mmHg are consid-
ered mild and are associated with a favorable prognosis. Pressure
gradients between 50 to 80 mmHg are indicative of moderate
disease and those with pressure gradients greater than 80 mmHg
are generally associated with severe disease and a greater risk for
manifesting clinical signs. Signs may include exercise intoler-
ance, syncope, or sudden death (8).
Various treatment options are available to patients with
pulmonic stenosis. In cases of severe stenosis, severe right ven-
tricular hypertrophy, or in uncompensated patients, balloon
valvuloplasty is the treatment of choice (8). Valvular anatomy
can be used as a predictor of good post-valvuloplasty outcome in
dogs with pulmonic stenosis (6). Other predictors of successful
outcome following balloon valvuloplasty are age and balloon-
to-valve hinge-point diameter ratio (9). There are numerous
surgical treatment options which include patch-graft valvotomy,
transventricular valve dilation, pulmonary arteriotomy, and
valvuloplasty (1013). Given the cost and relative risk of these
treatment options, a few large-scale studies have been initiated
to examine their overall success.
The concurrent presentation of valvular pulmonic stenosis
and an aberrant coronary artery has been reported to occur
more commonly in brachycephalic breeds, such as English bull-
dogs and boxers (6,14). This concurrent anomaly has not been
documented as frequently in other breeds, such as the Brittany
spaniel. It is hypothesized that a single coronary artery is the
CVJ / VOL 52 / MAY 2011
product of failed development of the opposite coronary anlagen
(15). This single right coronary artery originates from the right
Valsalva sinus and then branches. The left branch encircles the
pulmonary trunk constricting the main pulmonary artery at
the level of the right ventricular outflow tract causing regional
stenosis (2,15). Different morphologies of aberrant coronary
5. Jacobs G, Mahaffey M, Rawlings CA. Valvular pulmonic stenosis in
four Boykin Spaniels. J Am Anim Hosp Assoc 1990;26:247252.
6. Buchanan JW. Pulmonic stenosis caused by a single coronary artery in
dogs: Four cases (19651984). J Am Vet Med Assoc 1990;196:115120.
7. Thomas WP. Therapy of congenital pulmonic stenosis. In: KirkRW,
Bonagura JD, eds. Kirks Current Veterinary Therapy. 12th ed.
Philadelphia: WB Saunders, 1995:817821.

S T U D E N T PA P E R
8. Johnson MS, Martin M, Edwards D, French A, Henley W. Pulmonic
arteries have been described and this can have an impact on stenosis in dogs: Balloon dilation improves clinical outcome. J Vet Intern
Med 2004;18:656662.
which treatment options are viable possibilities in patients with
9. Kan JS, White RI, Mitchell SE, et al. Percutaneous transluminal bal-
this anomaly (15). loon valvuloplasty for pulmonary valve stenosis. Circulation 1984;69:
Historically, an aberrant coronary artery would be a contrain- 554560.
10. Breznock EM, Wood GL. A patch-graft technique for correction of
dication for balloon valvuloplasty, patch-grafting, or instrument
pulmonic stenosis in dogs. J Am Vet Med Assoc 1976;169:10901094.
dilation as methods of repairing pulmonic stenosis as there is a 11. Orton EC, Bruecker KA, McCracken TO. An open patch-graft tech-
risk of avulsing the coronary artery during the procedure, which nique for correction of pulmonic stenosis in the dog. Vet Surg 1990;19:
148154.
would result in death (8,16). Alternative techniques, such as
12. Orton EC, Monnet E. Pulmonic stenosis and subvalvular aortic stenosis:
partial instrument dilation, could be attempted as this would Surgical options. Semin Vet Med Surg (Small Anim) 1994;9:221226.
allow better control over dilation of the stenotic region and 13. Hunt GB, Pearson MR, Bellenger CR, Malik R. Use of a modified
open patch-graft technique and valvulectomy for correction of severe
would reduce the risk of coronary artery avulsion.
pulmonic stenosis in dogs: Eight consecutive cases. Aust Vet J 1993;
In the present case, the valvular stenosis created by the fused 70:244248.
pulmonic valve leaflets was compounded by subvalvular stenosis 14. Minami T, Wakao Y, Buchanan J, et al. A case of pulmonic stenosis with
single coronary artery in a dog. Jpn J Vet Sci 1989;51:453456.
resulting from the aberrant coronary artery. The single right
15. Buchanan JW. Pathogenesis of single right coronary artery and pulmonic
coronary artery branched to give rise to a left coronary artery. stenosis in English bulldogs. J Vet Intern Med 2001;15:101104.
The anomalous left branch coursed across the right ventricular 16. Buchanan JW, Anderson JH, White RI. The 1st balloon valvuloplasty:
An historical note. J Vet Intern Med 2002;16:116117.
outflow tract creating a constriction at the subvalvular region
of the pulmonic valve (Figure 1). This puppy was showing no
clinical signs and presented with what was diagnosed as moder-
ate, compensated, pulmonic stenosis. This case illustrates the
importance of routine monitoring, particularly when diagnosing
cardiac anomalies in young animals. During the initial exami-
nation at 4 mo of age the right coronary artery was not well-
visualized, so it was only during the recheck evaluation at 9 mo
of age that the aberrant right coronary artery was observed. This
was particularly important in this case, as the second anomaly
will have a great effect on potential treatment options for this
dog should he ever require surgical repair of the stenosis. This
case also highlights the point that conditions with known breed
predilections may still appear, although less commonly, in other
breeds and it is important to keep these possibilities on the list
of differential diagnoses.

Acknowledgments
The author thanks the cardiology service at the Atlantic
Veterinary College Veterinary Teaching Hospital and in par-
ticular, Dr. Etienne Ct, Dr. Erin Trageser, and Elaine Reveler.
 CVJ

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