World Journal of Cardiovascular Surgery, 2013, 3, 119-121
http://dx.doi.org/10.4236/wjcs.2013.33023 Published Online July 2013 (http://www.scirp.org/journal/wjcs)
A Rare Case of Aortico-Right Ventricular Tunnel Along
with Ventricular Septal Defect Presented as Ruptured
Sinus of Valsalva in an Adult Female
Binay Krishna Sarkar*, Krishnendu Chakraborty, Dilip Saha, Paresh Banerjee
Department of Cardiovascular & Thoracic Surgery, Nil Ratan Sircar Medical College & Hospital, Kolkata, India
Received April 8, 2013; revised May 13, 2013; accepted May 23, 2013
Copyright © 2013 Binay Krishna Sarkar et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background: Aortico-right ventricular tunnel is an extremely rare congenital defect rarely described in an infant. This
diagnosis is likely to be missed due to its rare entity and similar clinical presentations with other aortico-right ven-
tricular communications like ruptured sinus of valsalva. Methods: We report a case of previously undiagnosed aor-
tico-right ventricular tunnel along with a perimembraneous ventricular septal defect in an 18-year-old female. She had
history of exertional dyspnoea, palpitation, history of recurrent lower respiratory tract infection. She was diagnosed as a
case of ruptured sinus of valsalva (RSOV) elsewhere. She had “to and fro” murmur, features of congestive cardiac fail-
ure. Her echo diagnosis was RSOV. On surgical exploration, after opening the aorta, we found a tunnel like opening in
the aorta leading to the roof of RV cavity in between right and non coronary sinuses at the commissural level. Cusps
were splayed wide part. Ventricular septal defect was conspicuous from right atrial approach. Post operative CT angio
was done. Results: Venricular septal defect was closed from the right atrial approach and aortico-right ventricular tun-
nel was repaired through aortic and right venricular approach. Postoperative CT angio also confirmed the location and
closure of the defects. Postoperative recovery was uneventful. Conclusions: Aortico-right ventricular tunnel in an adult
female has not been reported in the literature previously. This rare entity should be considered in the differential diag-
nosis of a critically ill patient with a “to and fro” murmur, and signs of right heart failure.
Keywords: Aortico-RV Tunnel; Ventricular Septal Defect
Aneurysm of the sinus of valsalva is a rare aortic lesion
[1,2]. Congenital aneurysms are caused by weakness at
the junction of the aortic media and the annulus fibrosus.
Congenital aneurysms are more common than acquired
aneurysms. Acquired lesions occur secondary to disease
processes that involve the aortic root like syphilis and in-
fective endocarditis [1,2]. It can arise from the right
coronary (77%), noncoronary (23%) and, sometimes, the
left coronary sinus of valsalva . It can remain asymp-
tomatic for many years. About half are associated with
aortic regurgitation. Symptoms can be caused by mecha-
nical obstruction, with compression of the conducting
system, or intracardiac rupture. Rupture of sinus of val-
salva aneurysms may be spontaneous, or triggered by
chest trauma or exertion, or iatrogenic (cardiac catheteri-
zation). Rupture can occur into the right ventricle, right
atrium or left atrium and rarely into the left ventricle or
interventricular septum [1,2]. A ruptured aneurysm of the
sinus of valsalva can present with acute congestive heart
failure and can be life threatening. Once an aneurysm
rupture, the median survival is reduced to one to two
years (if left untreated) and patients usually succumb to
cardiac failure or infective endocarditis . Prompt sur-
gical repair is the optimal therapy after the diagnosis is
made. The concomitant surgical repair of associated ven-
tricular septal defect, atrial septal defect, and the aortic
valve is often required.
Although ruptured sinus of valsalva is a rare but
well-known congenital anomaly, aortico-right ventricular
tunnel is an exceptionally rare entity [3-7]. Aortico-right
ventricular tunnel is an extremely rare congenital defect
rarely described in the literature especially in infants
[3-8]. This diagnosis is likely to be missed due to its rare
entity and similar clinical presentations with other aor-
tico-right ventricular communications like ruptured sinus
opyright © 2013 SciRes. WJCS
B. K. SARKAR ET AL.
of valsalva. We report a female patient with aortico-right
ventricular tunnel who was seen in adulthood and was
successfully treated by surgical closure.
Case Summary: An 18-year-old female was admitted
in the department of Cardiothoracic and vascular surgery
with the history of exertional dyspnoea, palpitation, his-
tory of recurrent lower respiratory tract infection. She
was diagnosed as a case of ruptured sinus of valsalva
(RSOV) elsewhere. She had also history of hospitaliza-
tion for heart failure. She had NYHA (New York Heart
Association) class 3 dyspnoea on admission and was on
multiple cardiac medications including antifailure treat-
ment. She had no history of syncopal attacks, chest pain
or hemoptysis. She had no history of diabetes. On exami-
nation, she was found to have features of cardiomegaly,
hepatomegaly, bilateral crepitations in chest, with con-
tinuous murmur in the left parasternal area best heard
over the 3rd intercostal space. Her electocardiogram re-
vealed left ventricular hypertrophy. Echo doppler study
revealed left to right shunt in the region of the right aortic
sinus and another shunt in the ventricular septum in the
perimembraneous area along with aortic regurgitation.
Surgery: On surgical exploration, after opening the
aorta, we found a tunnel like opening in the aorta (Figure
1) leading to the roof of right ventricular (RV) cavity in
between right and non coronary sinuses at the commis-
sural level. Cusps were splayed wide part. Right atrium
was opened via an incision parallal to the atrio-ventri-
cular groove and ventricular septal defect was delineated
through tricuspid valve. Right ventricle was opened and
Figure 1. Peroperative picture showing aortic end of the
aortic RV tunnel.
the aortico-right ventricular tunnel was confirmed through
aorta and right ventricle and closed with a similar auto-
logous treated pericardial patch.
Ventricular septal defect was conspicuous from right
atrial approach which was confirmed as a separate defect
and it was repaired with autologous treated pericardial
patch. Aortic valve cusps, aortic annulus and coronaries
were normal. Aortic valve repair was done without dis-
torting valve cusps. Post-operative computed tomogram
(CT) angiography was done.
This rare diagnosis could be documented postopera-
tively by Computed Tomography angio study and three
dimensional reconstructions which clearly showed the
location of the tunnel (Figure 2) with two separate de-
fects which were repaired with patch (Figures 3(a) and
(b)). Sinus of valsalva was found normal and sinus of
valsalva aneurysm was excluded. Location of the Aor-
ticoright ventricular tunnel defect was clearly shown be-
tween the right coronary and non coronary sinuses adja-
cent to the roof of the right ventricle.
Postoperative recovery had been uneventful. There
was mild elevation of serum bilirubin in the immediate
postoperative period which became normal within few
days. She was discharged after ten days without any in-
fective complication. She was doing well during her fol-
Aortico-right ventricular tunnel is a rare cardiac entity
described in infants and more rarely in adults [3-8]. The
etiology of aorto-ventricular tunnel is uncertain. It pro-
bly results from a combination of maldevelopment of the
cushions which give rise to the pulmonary and aortic
roots, and abnormal separation of these structures which
also explains its formation and that of the associated val-
var and coronary arterial lesions . Typical presentation
Figure 2. 3D CT angio picture showing aotico-RV tunnel.
Copyright © 2013 SciRes. WJCS
B. K. SARKAR ET AL.
Copyright © 2013 SciRes. WJCS
Patch closing aortico to
Figure 3. (a) showing patch closing VSD; (b) showing patch
closing aortic-RV tunnel.
has been a critically sick newborn with cyanosis, a “to
and fro” murmur, and signs of right heart failure [3,8].
The defect is more common in males and usually is seen
in infancy with a to and fro murmur, bounding peripheral
pulses, cardiomegaly, and dilatation of the aortic root.
The majority of patients have congestive cardiac failure,
and a precordial diastolic thrill may be palpable. These
findings may also occur with ruptured sinus of valsalva
and large coronary artery fistula. Rarity of the condition
makes its diagnosis difficult. The correct diagnosis can
be made echo-cardiographically and confirmed by car-
diac catheterization  but the condition may be missed
or misdiagnosed as in our case. Our initial diagnosis was
ruptured sinus of valsalva with a ventricular septal defect
with aortic regurgitation. The diagnosis was made per-
operatively and further documented post-operatively with
a CT angiography and three dimensional reconstructions
. Lin and associates have described this entity in an
adult male which was successfully corrected surgically.
The diagnosis was preoperatively suspected on the basis
of echocardiography with doppler study and it was con-
firmed by a three-dimensional computed tomographic
scan. There may be associated anomaly of coronary ar-
tery origin, right ventricular outflow tract, aortic valve
abnormality and ventricular septal defect [5-8]. Talwar 
in their study described an anomalous right coronary
artery originating from the distal end of the tunnel. Var-
gas et al.  reported this anomaly in an infant and they
found the origin of the aortico-right ventricular tunnel
independently from the left coronary ostium and above
the sinus of valsalva.
Aortico-right ventricular tunnel should be considered
in the differential diagnosis in a critical patient with a “to
and fro” murmur, and signs of right heart failure. Before
surgical intervention, every effort should be made to di-
agnose the coronary artery anatomy and surgery should
be undertaken soon after the diagnosis is made. The sur-
gical intervention should be individualized based on
unique cardiac anomaly of each patient.
 T. Generali, A. Garatti, A. Biondi, A. Varrica and L. Me-
nicanti, “Aorta to Right Atrial Shunt Due to the Rupture
of a Degen-Erative Aneurysm of the Noncoronary Sinus
of Valsalva,” Journal of Cardiovascular Medicine, Vol.
14, No. 1, 2013, pp. 71-73.
 B. Topi, J. John, A. Agarwal, N. Nerella, V. Shetty, A.
Sadiq and J. Shani, “An Uncommon Cause of a Conti-
nuous Murmur,” Experimental & Clinical Cardiology, Vol.
17, No. 3, 2012, pp. 148-149.
 R. McKay, “Aorto-Ventricular Tunnel,” Orphanet Journal
of Rare Diseases, Vol. 2, 2007, p. 41.
 B. S. Lin, X. H. Zhang, Y. Z. Jiang, B. M. Zhang, D. Gao
and J. Song, “Diagnosis and Surgical Treatment of Adult
Aortico-Right Ventricular Tunnel,” The Annals of Thora-
cic Surgery, Vol. 89, No. 6, 2010, pp. 2024-2026.
 S. Talwar, U. K. Choudhary, S. S. Kothari and B. Airan,
“Aortico-Right Ventricular Tunnel,” International Jour-
nal of Cardiology, Vol. 70, No. 2, 1999, pp. 201-205.
 F. J. Vargas, A. Molina, J. C. Martinez, M. E. Ranzini
and J. C. Vazquez, “Aortico-Right Ventricular Tunnel,”
The Annals of Thoracic Surgery, Vol. 66, No. 5, 1998, pp.
 J. A. Van Son, J. Hambsch, P. Schneider and F. W. Mohr,
“Repair of Aortico-Right Ventricular Tunnel,” European
Journal Cardio-Thoracic Surgery, Vol. 14, No. 2, 1998,
pp. 214-217. doi:10.1016/S1010-7940(98)00168-7
 S. Westaby and N. Archer, “Aortico-Right Ventricular
Tunnel,” The Annals of Thoracic Surgery, Vol. 53, No. 6,
1992, pp. 1107-1109.