CASE REPORT

Device sizing for transcatheter closure of ruptured sinusof Valsalva as per echocardiography color Doppler turbulentflow jet diameter

Khurshid Ahmed • Muhammad Munawar •

Rabin Chakraborty • Beny Hartono •

Achmad Yusri

Received: 2 December 2013 / Accepted: 14 February 2014

� Japanese Association of Cardiovascular Intervention and Therapeutics 2014

Abstract Rupture of sinus of Valsalva (SV) is a rare

occurrence with a wide spectrum of presentation, ranging

from an asymptomatic murmur to cardiogenic shock or

even sudden cardiac death. We hereby report a case which

was successfully closed by transcatheter technique. In this

case, ruptured SV was entered from the aorta, an arterio-

venous loop was created and device was implanted using a

venous approach. The procedure was safe, effective and

uncomplicated, obviating the need for surgery. In this case,

the authors report for the first time the use of echo color

Doppler turbulent flow jet diameter as a reference value for

sizing the device.

Keywords Sinus of Valsalva � Echocardiography �Transthoracic � Right atrium � Right ventricle �Color Doppler echocardiography

Abbreviations

ASO Amplatzer septal occluder

LCS Left coronary sinus

NCS Non-coronary sinus

NCSV Non-coronary sinus of Valsalva

PDA Patent ductus arteriosus

RCS Right coronary sinus

SV Sinus of Valsalva

TTE Transthoracic echocardiogram

Introduction

Sinus of Valsalva (SV) is a dilatation of the aortic wall

located between the aortic valve and the sinotubular

junction. Its location is related to the coronary arteries

designated as the right coronary sinus (RCS), left coronary

sinus (LCS) and non-coronary sinus (NCS) [1, 2]. It is a

rare disease that has been reported in 0.09 % in one

autopsy series, but in Western surgical studies incidence of

0.14–0.23 % has been reported and 0.46–3.5 % in an Asian

series with prevalence in males [1, 3]. Traditional treatment

is surgical excision and patch closure under cardiopulmo-

nary bypass [4].

Percutaneous closure of ruptured SV aneurysm was first

attempted by Cullen et al. [5] using a Rashkind umbrella.

Since then a few reports have been published with the use

of different available closure devices [6–9]. We hereby

report a case of successful closure of SV rupture by

transcatheter technique. To the best of our knowledge, the

sizing of the lesion using color Doppler flow is being

reported for the first time in this case. The technique uti-

lized is very basic, simple and cost effective.

Case report

An 18-year-old Indonesian female presented to us in March

2013 with the history of shortness of breath on moderate

exertion and on-and-off chest discomfort for the last 2 years.

Few months ago she was seen at other hospital, where she

was diagnosed to have ruptured non-coronary sinus of

Valsalva (NCSV) and advised for surgery. Patient did not

want surgery and came to our hospital for second opinion.

On examination, she had good volume regular pulse at

76/min and blood pressure 106/56 mmHg. Her jugulo-

K. Ahmed � M. Munawar (&) � B. Hartono � A. Yusri

Binawaluya Cardiac Center/Department of Cardiology

and Vascular Medicine, Faculty of Medicine,

University of Indonesia, Jakarta, Indonesia

e-mail: muna@cbn.net.id

K. Ahmed � R. Chakraborty

Apollo Gleneagles Hospital, Kolkata, India

123

Cardiovasc Interv and Ther

DOI 10.1007/s12928-014-0257-5

venous pressure was not raised and she had no pedal

edema. On auscultation, she had continuous ‘‘machinery

murmur’’ grade V/VI over lower left sternal border. Lab-

oratory results were normal. Electrocardiogram showed

normal sinus rhythm. Transthoracic echocardiogram (TTE)

revealed ruptured NCSV of diameter 5.99 mm and a sig-

nificant aorta-to-RV shunt (Fig. 1), tricuspid aortic valve

with left ventricular ejection fraction 60 % and tricuspid

annular planar systolic excursion (TAPSE) 2.6 cm, trivial

tricuspid regurgitation with pulmonary artery systolic

pressure (PASP) *35 mmHg and trivial pulmonary

regurgitation. All 4 chambers were of normal size. Trans-

catheter occlusion of ruptured SV was planned.

Cardiac catheterization revealed mean PASP 28 mmHg

with normal left and right NCSV. An aortic root angiogram

confirmed the presence of NCSV, which had ruptured into

the RV (Fig. 2). Balloon sizing of the ruptured sinus

showed 6.94 mm diameter (Fig. 3). TTE and fluoroscopy

Fig. 1 Transthoracic echocardiogram. a B-mode image demonstrated the sinus of Valsalva and its rupture into the right ventricle, b color

Doppler showed the turbulent flow jet through the rupture

Fig. 2 Aortic root angiogram showing rupture (indicated by arrow)

of non-coronary sinus of Valsalva into right ventricleFig. 3 Balloon sizing of the rupture

K. Ahmed et al.

123

aided the positioning of the device over a guide-wire using

an arteriovenous loop from the inferior vena cava to the

aorta. We initially chose 8/10 mm Lifetech Scientific Heart

PDA occluder, but it appeared smaller than the defect and

could not be deployed. The device was still connected with

the delivery system so it was successfully withdrawn.

TTE was repeated using different views that showed

maximum turbulent color flow jet diameter across ruptured

SV 9.82 mm in parasternal short axis at the aortic valve

level and we took this diameter as a reference value for

selection of the device size. Subsequently, the defect was

successfully closed with 10/12 mm Lifetech Scientific

Heart PDA occluder. A post-procedural aortic root angio-

gram (Fig. 4) and TTE (Fig. 5) showed successful device

closure with no residual shunt. The patient was discharged

home 4 days later and the hospital stay was uneventful.

Discussion

A ruptured aortic sinus is a major cardiovascular event that

demands prompt diagnosis and treatment. Nevertheless,

given the relative infrequency of the condition, achieving a

definite diagnosis can be challenging. In our case, patient

presented at 18 years of age and lack of predisposing

factors suggests a possible causal association with con-

genital rupture.

SV aneurysm is a dilatation caused by incomplete fusion

of the distal bulbar septum that divides the pulmonary

artery and the aortic valve. There is lack of continuity

between the aortic media and the aortic valve. Thinning of

the aortic media is also observed in affected sinus which

can progressively dilate over time, especially in cases of

arterial hypertension [1–3, 10].

The most common cause is congenital, although its

origin may be acquired (trauma, infection, or degenerative

diseases) [2, 3]. It commonly coexists with other malfor-

mations, such as ventricular septal defect, anomalies of the

aortic valve and coarctation of the aorta [1]. RCS is the one

most frequently affected followed by the NCS and, rarely,

the LCS [1, 2, 11]. The left sinus is not derived embryo-

logically from bulbar septum and, therefore, is rarely

affected by congenital lesions. This anomaly can be

unrecognized for many years. Only rarely, in cases of

major aneurysm, do they cause A-V block, aortic

Fig. 4 Post-procedural aortic root angiogram showed successful device closure with no residual shunt in RAO and LAO projections

Fig. 5 Post-procedural transthoracic echocardiogram showed well-

seated patent ductus arteriosus occluder and no residual shunt

Device sizing for ruptured sinus of Valsalva

123

incompetence or subvalvular pulmonary stenosis [12, 13].

Symptoms usually appear when the aneurysm ruptures into

a cardiac chamber causing continuous murmur, exercise

intolerance, symptomatic heart failure, or sudden death,

depending on the magnitude of left to right shunt. A

diagnosis of aortic sinus rupture and fistula can be con-

firmed by echocardiography, either transthoracic or trans-

esophageal, or by cardiac catheterization (right and left).

TTE with color Doppler shows the affected sinus and the

chamber the shunt is directed to. It can lead to an accurate

diagnosis in virtually most of these patients [14], and

transesophageal echocardiogram may be useful when TTE

is inconclusive. Extravasation of contrast medium after

injection of the aortic root demonstrates the location of the

fistulous tract. Traditionally, the gold standard for the

diagnosis of ruptured SV is cardiac catheterization.

The natural history of asymptomatic aneurysm of an aortic

sinus is unclear, and variant cases with rapid clinical

deterioration or many years of stabilization have been

described. However, once symptoms develop or rupture

occurs, urgent intervention is recommended [15]. The

unique thing which we learnt from our case is regarding

sizing of the device. Two-dimensional TTE demonstrated

the diameter of ruptured NCS 5.99 mm, and angiographi-

cally stretched balloon diameter across the rupture was

6.94 mm. But color Doppler revealed maximum turbulent

flow jet diameter across ruptured SV to be 9.82 mm after

multiplane evaluation, which was significantly larger than

5.99 and 6.94 mm. We took turbulent flow jet diameter as a

reference value for device sizing and deployed 10/12

Lifetech Scientific Heart PDA occluder. A post-procedural

aortic root angiogram and TTE showed successful and

appropriate device closure with no residual leak. The

procedure was safe, effective and uncomplicated, obviating

the need for surgery. We suggest that the maximum

diameter of the RSV should be carefully measured with

color Doppler turbulent flow jet diameter and this diameter

should be used for choosing the size of the occluding

device.

Currently, there is no literature available evaluating the

relationship between sizing of the device and echo color

Doppler turbulent flow jet diameter. Color Doppler echo-

cardiography is extremely sensitive in the detection of

intracardiac shunts. It has been reported that a leak jet

through a 1 mm orifice can be detected by Doppler color

flow mapping [16]. As per our speculation, the margin

across the orifice of the rupture is assumed to be uneven

and non-uniform. When a rupture occurs, there are many

tiny slits across its walls. These slits are not detected by

two-dimensional echocardiography. Whereas Doppler

color flow can potentially pick up, in addition to ruptured

orifice, these tiny slits of high velocity blood flow. Due to

this combined effect, it results in larger diameter of the

ruptured orifice.

Aortic wall has relatively greater stiffness due to its high

connective fiber content (elastin and collagen) which

imparts the elastic properties and strength of the aorta,

respectively [17]. As a result, it can resist fairly well, up to

certain extent, the mechanical stretch pressure on its wall

transmitted by the balloon. Due to this, sometimes the

orifice is not fully stretched up to the desired far ends of

these tiny slits. Unlike atrial and ventricular septa, a con-

siderably larger stress is required in the aorta. Considering

the histological structure of the aortic wall, a marked dif-

ference was observed in diameter measured by stretched

balloon across the rupture. We attribute this as the reason

for discrepancy between color Doppler diameter and

angiographic balloon sizing. In our case, we found that

Doppler color flow diameter correlates fairly well with the

size of the device. A better result with the use of turbulent

color flow diameter as a reference value for sizing the

device is being reported for the first time in this case. Our

finding is based on only one case, so our procedure and

observations need verification in more number of patients.

It is a novel approach of sizing the device that is very basic,

simple, easily available and cost effective. We do also

believe that 3D echo will be a better option for exact sizing

of the device.

Acknowledgments The authors sincerely thank Dr. Sumera Ahmed

for her help in the preparation of this case report.

Conflict of interest There are no potential conflicts to declare.

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