A Rare Case of Aortico-Right Ventricular Tunnel Along with Ventricular Septal Defect Presented as Ruptured Sinus of Valsalva in an Adult Female

doi:10.4236/wjcs.2013.33023

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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

Email: *[email protected]

Received April 8, 2013; revised May 13, 2013; accepted May 23, 2013

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

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

1. Introduction

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 [2]. 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 [2]. 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

*Corresponding author.

B. K. SARKAR ET AL.

120

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-

low-up visits.

2. Discussion

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 [3]. Typical presentation

Figure 2. 3D CT angio picture showing aotico-RV tunnel.

B. K. SARKAR ET AL.

121

(a)

Patch closing aortico to

RV tunnel

aorta

(b)

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 [3] 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

[4]. 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 [5]

in their study described an anomalous right coronary

artery originating from the distal end of the tunnel. Var-

gas et al. [6] 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.

REFERENCES

[1] 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.

doi:10.2459/JCM.0b013e3283528f8c

[2] 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.

[3] R. McKay, “Aorto-Ventricular Tunnel,” Orphanet Journal

of Rare Diseases, Vol. 2, 2007, p. 41.

doi:10.1186/1750-1172-2-41

[4] 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.

doi:10.1016/j.athoracsur.2009.10.073

[5] 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.

doi:10.1016/S0167-5273(99)00059-5

[6] 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.

1793-1795. doi:10.1016/S0003-4975(98)00927-8

[7] 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

[8] S. Westaby and N. Archer, “Aortico-Right Ventricular

Tunnel,” The Annals of Thoracic Surgery, Vol. 53, No. 6,

1992, pp. 1107-1109.

doi:10.1016/0003-4975(92)90401-O