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Heart, Lung and Vessels. In press
Aortic to right atrial fistula
secondary to chronic ruptured sinus
of Valsalva aneurysm
Nisal K. Perera1, Sean D. Galvin1, Omar Farouque2, George Matalanis1
Departments of Cardiac Surgery1 and Cardiology2, Austin Health, Heidelberg, Victoria, Australia
Keywords: adult cardiac surgery, aortic surgery,
heart failure. A sinus of Valsalva aneurysm (SOVA) is an
uncommon cardiac anomaly occurring in
less than 1% of patients undergoing open
cardiac surgical procedures (1). Approximately 65-85% of SOVAs originate from
the right sinus of Valsalva, while SOVAs
originating from noncoronary (10-30%)
and left sinuses (<5%) are less common
(2). It may be congenital or acquired in origin and may rupture into any of the cardiac
chambers to form an aorto-cardiac fistula.
When intra-cardiac rupture occurs, early
surgical intervention is indicated as rupture may lead to serious hemodynamic instability, acute heart failure and, if left untreated, has a mean survival period of 1–2
years (3). Although there have been suc-
Figure 1 - Short Axis view of TEE confirmed a ruptured non-coronary SOVA with torrential aortic to
right atrial shunt. AV = Aortic Valve; FT = Fistula Tract; RA = Right Atrium.
Corresponding author:
Dr Nisal Kalhara Perera
Department of Cardiac Surgery
Austin Hospital
PO Box 5555,
Heidelberg, Victoria, 3084 Australia
E-mail: [email protected]
Heart, Lung and Vessels. 2014, Vol. 6
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Perera NK, Galvin SD, Farouque O, Matalanis G
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cessful reports of transcatheter closure (4,
5), no dedicated closure devices exist and
open surgical repair remains the mainstay
of treatment. We present a case of a chronic
ruptured non-coronary SOVA illustrating
the classical “windsock” appearance of the
aorta to right atrial fistula tract and correlate the non-invasive imaging appearances
with those seen at the time of open surgical
repair.
A 50-year-old female presented with a
2-week history of sudden onset shortness
of breath, impaired exercise tolerance and
orthopnoea. Past medical history included
rheumatoid arthritis. Her vital signs were:
heart rate 102 beats per minute, respiratory
rate 28 breaths per minute, blood pressure
112/52mmHg, oxygen saturation 98% on
room air and NYHA class III. On cardiovascular examination she was noted to have
clinical signs of biventricular heart failure
with a sinus tachycardia, elevated jugular
venous pulse, a continuous murmur, bilateral pulmonary crepitations and pitting
Figure 2 - Left heart catheterization study showing a large aortic to right atrial shunt. Asc = Ascending Aorta; FT = Fistula Tract; RA = Right
Atrium.
Figure 3 - Intra operative image of the fistula
tract protruding from the base of the non-coronary
sinus with a “windsock” appearance. Fistula tract
is identified by the forceps.
edema in the lower limbs. Transesophageal
echocardiogram (TEE) confirmed a ruptured non-coronary SOVA with a torrential
aortic to right atrial shunt and right heart
volume overload. The fistula tract was seen
as a “windsock” originating in the non-coronary sinus and projecting into the right
atrium (Figure 1). There were no other
cardiac abnormalities identified on TEE.
Right and left heart catheterization studies
showed normal coronary anatomy, elevated
right heart pressures [mean RA: 31 mmHg,
RV: 62/24 mmHg, PA: 55/31 (mean =
43 mmHg] and a large left to right shunt
(Qp:Qs 3.2:1) (Figure 2).
The patient was operated on with urgent
status (during current hospital stay) after
a short intensive period of diuretic therapy.
At operation there was a 2cm defect in the
non coronary sinus with a fistula tract of
thinned aortic wall protruding from the
base of the non coronary sinus into the
right atrium with the appearances of a
“windsock” (Figure 3). The “windsock”
was delivered back into the aorta from its
right atrial position and its apex was seen
to have multiple perforations resulting in
the fistula. The tissue that made up the
Heart, Lung and Vessels. 2014, Vol. 6
Chronic Ruptured SOVA
“windsock” was excised and a bovine pericardial patch was used to repair the defect
in the non-coronary sinus. After weaning
from cardiopulmonary bypass, intra-operative TEE revealed a satisfactory repair with
no residual fistula connection and normal
aortic and tricuspid valve function. The
patient had an unremarkable recovery and
was discharged home on the 6th post operative day. At 6-week follow up she was
symptom free and transthoracic echocardiogram revealed normal cardiac function
with no detectable shunt.
This case serves to highlight the importance of early identification and treatment
of a ruptured SOVA as it can bring rapid
and permanent symptomatic relief. Surgery
can be performed with low morbidity and
mortality with 10-year survival rates of 6393% (3, 6). In the presence of an otherwise
normal aortic valve and root, the SOVA can
be treated locally with either pericardial
patch closure of the defect or replacement
of the sinus with a Dacron patch with pres-
ervation of the native aortic valve. When
a diagnosis of ruptured SOVA is made, we
advocate early surgical repair as if left untreated progressive heart failure and premature death may occur.
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Cite this article as: Perera NK, Galvin SD, Farouque O, Matalanis G. Aortic to right atrial fistula secondary to chronic ruptured sinus of Valsalva aneurysm. Heart, Lung and Vessels. 2015. In press.
Source of Support: Nil. Disclosures: None declared.
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