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CORRESPONDENCE: CASES

To the editor: e sinuses of Valsalva are focal expansions forming

the aortic root walls. Rupture of the sinus of Valsalva usually occurs

secondary to aneurysms of the sinus.[1] A continuous murmur is

oen audible in patients with a rupture, and in approximately one-

sixth of patients with a continuous murmur, the murmur is caused

by a rupture.[2] We report two cases of rupture of the sinus of Valsalva

without any aneurysmal dilation of the aortic root, showing stulous

connections with the cardiac chambers.

In our rst case, a 46-year-old man presented with complaints of

episodic chest pain and breathlessness for 6 months. He had recently

been diagnosed with type 2 diabetes mellitus. On presentation, his

blood pressure was 130/70 mmHg with a pulse rate of 78 bpm. On

physical examination, a continuous murmur could be heard in the

parasternal region. Laboratory values were normal, and the chest

radiograph was unremarkable.

On transthoracic echocardiography, there was evidence of prolapse

of the right coronary cusp of the sinus of Valsalva, with a 5 mm defect

in the right sinus with turbulent continuous (systolic and diastolic)

ow through it seen on colour Doppler ultrasound. ese ndings

raised a strong suspicion of rupture of the right cusp of the sinus of

Valsalva with a communication with the right ventricular outow

tract and a le-to-right shunt. e ndings were accompanied by mild

mitral regurgitation and tricuspid regurgitation. Echocardiography

revealed that all the cardiac chambers were normal in size, and the

ventricles showed normal contractility.

e patient then underwent a cardiac computed tomography (CT)

scan with coronary angiography. e CT examination was performed

with an Ingenuity Core 128-slice CT scanner (Philips, India). Images

were obtained with a slice thickness of 0.6 mm in the axial planes using

electrocardiogram (ECG) gating, and reconstruction was done with

coronal and sagittal images on the console. e contrast-enhanced

images were obtained aer bolus administration of a contrast agent.

e CT scan showed a defect of 5.5 mm in size along the le lateral

margin of the right coronary cusp of the sinus of Valsalva (Fig. 1). It

also demonstrated a tubular stulous connection between the right

coronary cusp and the right ventricle at the level of the right ventricular

outow tract. e rupture was classied as type I according to the

modied Sakakibara classication.[3] Incidentally seen on the cardiac

CT scan were two small tubular accessory appendages arising from

the le atrial appendage, pointing towards the atrio-aortic groove.

Surgical closure of the ruptured sinus of Valsalva was performed.

In our second case, a 61-year-old man presented with complaints

of breathlessness for 4 months. is was not associated with any

chest pain. He did not report any existing comorbidities. On physical

examination, the blood pressure was 135/78 mmHg with a pulse

rate of 75 bpm. On auscultation a continuous murmur was heard

in the parasternal region, and transthoracic two-dimensional

echocardiography revealed a 7 mm defect in the non-coronary cusp

of the aortic valve, which was seen to be communicating with the

right atrium through a tunnel. CT aortography was then performed

to visualise the defect in greater detail and detect any coexisting

vascular abnormalities. Aer bolus administration of a contrast

agent, images with a slice thickness of 1 mm were obtained in the

axial planes with reconstruction to coronal and sagittal sequences

done on the console. A defect in the non-coronary cusp of the

aortic valve was seen to be communicating with the dilated right

atrium (Fig. 2). e rupture was classied as type IV according to the

modied Sakakibara classication.[3] Contrast reux was visualised

in the form of early enhancement of the inferior vena cava and

hepatic veins in the arterial phase. No other abnormalities were

seen in the thoracic or abdominal aorta or the other great vessels.

e cardiac chambers appeared normal without any evidence of

dilation or wall hypertrophy.Surgical closure of the ruptured sinus

of Valsalva was performed.

The aortic root is made up of the aortic valve leaflets, the

commissures, the sinus of Valsalva, the sinotubular junction, and the

annulus.[4] ere are three sinuses of the aortic root, lying between

the superior attachment formed by the sinotubular junction, which

is a relatively constricted segment between the aortic root and the

ascending aorta, and inferiorly by the aortic valve leaets, which

separate the aorta from the le ventricle. e right coronary sinus

is the origin of the right coronary artery and the le coronary sinus

is the origin of the le coronary artery. e non-coronary sinus does

not give rise to any coronary artery. e attachment of the aortic valve

leaets into the aortic root wall in a semilunar fashion gives rise to

a three-dimensional ring known as the aortic annulus.[4,5] Previous

studies have shown the normal mean (standard deviation) end-

diastole diameter of the sinus of Valsalva to be 3.2 (0.6) cm for men and

2.9 (0.5) cm for women.[4] ese sinuses play an important role in aortic

valve function by providing space to prevent blocking of the orices

of the coronary arteries from the aortic leaets.

It has been found that rupture of the sinus of Valsalva is more common

in men than in women, and in Asians than in other ethnic groups.[6]

Congenital causes of rupture are deciency of elastic tissue, as seen in

Marfan syndrome or Ehlers-Danlos syndrome, or focal weakness of the

elastic laminae between the aorta and the annulus brosus. Acquired

causes are atherosclerosis, cystic medial necrosis, tuberculosis, bacterial

endocarditis or cardiac complications of syphilis, and trauma. Iatrogenic

causes such as a pseudoaneurysm resulting from haematoma formation

aer aortic valve replacement have been reported.[6]

Ruptures of the sinus of Valsalva are associated with other cardiac

anomalies such as bicuspid aortic valve, ventricular septal defects,

and other coronary artery anomalies. e aneurysms most commonly

arise from the right coronary sinus and non-coronary sinuses and can

rupture into the adjacent right ventricle, right atrium, le atrium, or

rarely the interventricular septum.[7]

ese aneurysms can cause complications and rupture later in life.

Non-ruptured aneurysms may remain asymptomatic or manifest

acutely as a result of a mass eect on adjacent structures. Asymptomatic

aneurysms may present with a continuous murmur. Rupture of these

Rupture of the sinus of Valsalva with stulous connection with the

cardiac chambers: A report of 2 cases

26 AJTCCM VOL. 30 NO. 1 2024

CORRESPONDENCE: CASES

aneurysms can lead to chest pain, dyspnoea,

congestive heart failure, and cardiac arrest in

extreme cases.[6] Untreated ruptured aneurysms

may result in cardiac tamponade and eventually

death in a few cases. One of the common

complications of both ruptured and unruptured

aneurysms is aortic regurgitation.[6]A continuous

murmur is often audible in patients with a

ruptured sinus of Valsalva and must be seen as

a warning sign.[2]

e prevalence of the aortocardiac stulas

seen in our cases has not been studied

extensively.[8] They can be congenital or

acquired. If acquired, they can be due to

trauma, iatrogenic causes such as cardiac or

aortic surgery, or non-traumatic causes such

as rupture of a sinus of Valsalva aneurysm,

infective endocarditis or aortic dissection. A

study showed that many patients with aorto-

cardiac fistulas presented with symptoms of

heart failure, with few of them experiencing

chest pain or other symptoms.[8] Asymptomatic

patients accounted for only 9% of the studied

group, with a continuous murmur on

auscultation being the only demonstrable

nding.

Most ruptures of the sinus of Valsalva

are initially seen on colour Doppler

echocardiography, but the diagnosis is usually

confirmed on angiography. Both CT and

magnetic resonance imaging (MRI) have

an additional role in diagnosing rupture

of sinus of Valsalva aneurysms, as they are

used in emergency settings.[6] ECG-gated

contrast-enhanced CT provides better spatial

resolution than other imaging modalities, and

also gives detailed anatomical information on

the surrounding structures. It is also possible

to identify an aorto-cardiac shunt if present,

and to identify a ruptured sinus of Valsalva

and its connection to adjacent cardiac

chambers. e surgeon can also be provided

with three-dimensional representations,

which are useful in deciding on the surgical

approach. MRI has an advantage of accurate

assessment of the origin and size of aneurysms

and the surrounding anatomical structures. It

is also helpful in identifying accompanying

aortic regurgitation and in evaluating the

haemodynamic pattern of the le ventricle.[6]

In this case report we have classied the

sinus of Valsalva ruptures on the basis of the

modified Sakakibara classification.[3] The

first case was classified as type I, which is

rupture into the right ventricle just below the

A B C

DE F

A B C

D E F

Fig. 1. (A - D) CT coronary angiography images in axial and reconstructed coronal and oblique

planes, showing a 5.5 mm defect along the le lateral margin of the right coronary cusp of the

sinus of Valsalva with a tubular stulous connection (arrows) between the right coronary cusp

and the right ventricle at the level of the right ventricular outow tract. (E) CT angiography image

in the axial plane with an incidental nding of two small tubular accessory appendages arising

from the le atrial appendage, pointing towards the atrio-aortic groove (arrows). (F) Volume-

rendered 3D CT image showing a defect in the right coronary cusp of the sinus of Valsalva with

a tubular stulous connection (arrow) between the right coronary cusp and the right ventricle.

(CT = computed tomography; RV = right ventricle; PA = pulmonary artery; LA = le atrium;

LV = le ventricle.)

A B C

DE F

A B C

D E F

Fig. 2. (A - C) CT aortography images in the axial and reconstructed coronal and oblique planes

showing a defect in the non-coronary cusp of the aortic valve that communicates with the dilated

right atrium (arrows). (D) CT aortography image at the level of the abdominal aorta show

contrast reux in the form of early enhancement of the inferior vena cava and hepatic veins in the

arterial phase. (E and F) Reconstructed CT aortography images showing the defect visualised in

the non-coronary cusp of the aortic valve communicating with the dilated right atrium (arrows).

(CT = computed tomography; RA = right atrium; LA = le atrium; LV = le ventricle; RV =

right ventricle.)

AJTCCM VOL. 30 NO. 1 2024 27

CORRESPONDENCE: CASES

pulmonary valve, and the second case was classied as type IV, in which

there is protrusion and rupture into the right atrium.

An aggressive approach to a ruptured sinus of Valsalva is advisable, to

prevent clinical deterioration and progression to severe cardiac failure.

Operative procedures may include patch repair and aortic root or valve

replacement if the rupture is accompanied by signicant aortic or other

valvular regurgitation. Reports suggest perioperative mortality of up

to 3.9%, especially in the setting of pre-existing sepsis or endocarditis,

so there is a need for strict endocarditis prophylaxis. Aer the surgical

repair, patients’ life expectancy is similar to that of the healthy

population. Recent advances include percutaneous transcatheter

closure of ruptured sinus of Valsalva aneurysms using a duct occluder.

[9] Percutaneous closure may be a better choice than surgical repair

in critically ill patients with poor cardiac function and no associated

anomalies that may require surgical repair, because of its advantages of

less trauma and haemorrhage and rapid post-procedural recovery.[10]

In conclusion, our cases were unique as in both patients the rupture

of the sinus of Valsalva was not accompanied by any aneurysmal

dilation of the aortic root, which is commonly reported in the literature.

In our cases, CT was useful in accurately depicting rupture of the right

coronary cusps and non-coronary cusps of the aortic root at the sinus of

Valsalva and their stulous communications with the cardiac chambers,

which demonstrates that CT can provide superior anatomical details of

the sinus of Valsalva and the surrounding structures in comparison with

other available imaging modalities.

T Kalekar, MD Radiodiagnosis, Professor