Cardiovascular Journal of Africa: Vol 28 No 4 (July/August 2017)

CARDIOVASCULAR JOURNAL OF AFRICA • Volume 28, No 4, July/August 2017

AFRICA

a formal classification for sinus of Valsalva aneurysm, according

to the coronary sinus affected and the area towards which they

protrude or rupture (Table 1).

A true aortico-cameral connection may be difficult to

differentiate from a coronary cameral fistula. Levy stresses the

origin of the abnormal tunnel above and separate from the

coronary orifice.

Bove and Schwartz, however, emphasise the

separation of the orifice of the tunnel from the adjacent sinus of

Valsalva by a small well-defined fibrous ridge.

In our case, the

tunnel gave rise to the right main coronary artery.

To date, spontaneous closure of the aorta–right atrial tunnel

has not been reported. The pressure that is created during a

left-to-right shunt is too high to close spontaneously. Clinical

presentation varies according to the degree of the left-to-right

shunt, which can range from completely asymptomatic to

symptoms related to the volume overload on the ventricles.

Surgical and percutaneous interventions are the main

therapeutic options. Transcatheter closure of a tunnel is

technically straightforward in the hands of an experienced

interventionist. It is cost intensive, but has low peri-procedural

risk.

Percutaneous intervention with a plug or coil is easiest

when the coronary arteries arise separately from the tunnel.

Complications of prolonged patency of the tunnel include

calcification of its wall, biventricular volume overload or

aneurysmal expansion, congestive cardiac failure, pulmonary

vascular disease, infective endocarditis, and higher mortality

rate during surgery if the lesion is left uncorrected until the

patient ages.

With regard to our patient, the CT angiogram

demonstrated calcifications within the wall of the tunnel already.

Multiple aortico-cameral tunnels have been reported

once previously in an elderly male patient. Transthoracic

echocardiogram, transoesophageal echocardiogram and cardiac

catheterisation revealed separate tunnels from the right coronary

sinus to the left ventricle, right ventricle and right atrium. Surgery

was performed successfully.

Mahesh

et al

. presented a case of

pre-natal diagnosis of aorta–right atrial tunnel, subsequently

treated with percutaneous coil embolisation.

Diagnosis

Thurnam diagnosed all ruptured sinus of Valsalva aneurysms at

autopsy.

In 1951, Venning was possibly the first to diagnose an

acute rupture of an aneurysm of the sinus of Valsalva in life.

The first successfully diagnosed and treated case of aorta–right

atrial tunnel was reported by Coto

et al

. in 1980.

Aneurysm of the sinus of Valsalva may be difficult to

diagnose clinically, and echocardiography is usually the first

step to diagnosis.

Two-dimensional echocardiography has been

improved with the use of contrast, spectral Doppler and colour-

flow imaging.

It is non-invasive and easily accessible. Chaing and

co-workers were able to accurately diagnose a ruptured sinus of

Valsalva aneurysm in 58% of patients using two-dimensional

echocardiography alone. The addition of intravenously injected

bubble contrast improved the sensitivity to 75%.

One limitation of echocardiography is the inability to visualise

the coronary anatomy.

Transoesophageal echocardiography is

more sensitive than transthoracic echocardiography due to the

probe’s proximity to the relevant structures and it optimises echo

graphic windows with distorted anatomy.

Ultrasound has improved markedly in the past 15 to 20

years. Three-dimensional echocardiography offers the ability

to improve and expand the diagnostic capabilities of cardiac

ultrasound. Serial two-dimensional images are obtained and

three-dimensional data are reconstructed from this data set,

thereby complimenting and supplementing two-dimensional

cardiac imaging. Thus far it has been successfully applied to

the detection and assessment of several anatomical cardiac

defects. This method has not yet been specifically applied to

the detection or imaging of aorta–right atrial tunnel. Further

technological improvements and additional clinical studies will

broaden the list of appropriate applications of this exciting new

ultrasound modality.

Cardiac catheterisation and retrograde angiography have

been considered necessary to differentiate these aneurysms

from other cardiac anomalies, and also prove useful to map

out the coronary arteries prior to surgery.

The high cost,

Table 2. Descriptive summary of cases from 2003, diagnosed using electron-beam tomography, CT angiography and CMR

Author

Age Gender Type Origin Termination Coronary artery Imaging

Treatment

Turkay (2003)

29 M anterior RCS lateral aspect

of right atrium

proximal tunnel electron beam tomography; echocardiography;

angiography, intra-op images

surgery

Akar (2006)

57 M anterior RCS right atrium separate

transoesophageal echocardiogram, cardiac cath-

eterisation, CT angiogram, intra-op images

surgery

Krishna (2010)

11 F posterior LCS SVC/RA junc-

tion

separate

echocardiography, cardiac catheterisation, CT

angiogram, intra-op images

surgery

Walker (2010)

25 F

NCS right atrium

transoesophageal echocardiogram, CT angiogram surgery

Chandra (2011)

12 F anterior RCS posterior wall

right atrium

separate

transoesophageal echocardiogram, CT angiogram,

cardiac catheterisation

percutanous transcatheter

occlusion of tunnel

Myers (2011)

33 M posterior LCS right atrium tunnel

cardiac catheterisation, CT angiogram, intra-op

images

surgery

Sung (2011)

73 F anterior RCS right atrium separate

CT angiogram

surgery

Salehi (2013)

71 M anterior RCS right atrium abutting origin

of tunnel

cardiac catheterisation, transoesophageal echocar-

diogram, CMR

surgery

Tossios (2013)

47 F posterior LCS right atrium separate

transthoracic echocardiogram, transoesophageal

echocardiogram, cardiac catheterisation, CT

angiogram, CMR

surgery

Iyisoy (2014)

18 F posterior LCS roof of right

atrium

proximal tunnel transoesophageal echocardiogram, cardiac cath-

eterisation, CT angiogram

surgery

Kim (2014)

36 F

anterior RCS right atrium separate

transoesophageal echocardiogram, cardiac cath-

eterisation, CT angiogram

surgery

CT, computed tomography; CMR, cardiac magnetic resonance; SVC, superior vena cava; RA, right atrial; RCS, right coronary sinus; LCS, left coronary sinus; NCS,

non-coronary sinus