Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in 44-year-old patient

Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in 44-year-old patient

CRVASA-456; No. of Pages 5 cor et vasa xxx (2017) e1–e5 Available online at www.sciencedirect.com ScienceDirect journal homepage: http://www.elsevie...

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CRVASA-456; No. of Pages 5 cor et vasa xxx (2017) e1–e5

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Case report

Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in 44-year-old patient: Case report Andrey Slautin a,b,*, Vladimír Mikulenka a, Jaroslav Hlubocký a,b, Róbert Novotný a,b, Miroslav Špaček a,b, Vladimír Vondráček a, Jaroslav Lindner a,b a b

Second Surgical Clinic of Cardiovascular Surgery, General University Hospital, Prague, Czech Republic First Faculty of Medicine, Charles University in Prague, Czech Republic

article info

abstract

Article history:

We hereby present a case report of ruptured sinus of Valsalva aneurysm into the right atrium

Received 6 December 2016

in 44-year-old patient with 8-month progressive exertional dyspnoea, occasional palpita-

Received in revised form

tions and oedema of lower extremities. A massive left-to-right shunt between the outflow

8 January 2017

tract of the left ventricle and right atrium was discovered from the echocardiogram

Accepted 10 January 2017

examination. The patient was indicated for surgery, during which it was discovered a defect

Available online xxx

in the area of the non-coronary sinus extending into the right atrium, calcifications in the

Keywords:

abundant tissue was excised from the right atrium which had originally been an aneurysm

annulus of the congenitally bicuspid aortic valve, without signs of endocarditis present, Atrial septal defect

of the non-coronary sinus of the aortic root. Sinus of Valsalva aneurysm is a very rare

Ruptured sinus of Valsalva

pathology which is generally asymptomatic. In this case it manifested through a rupture into

aneurysm

the right atrium and symptomatology of right-sided heart failure. Surgery was the only

Cardiac surgical procedures

possible treatment. © 2017 The Czech Society of Cardiology. Published by Elsevier Sp. z o.o. All rights reserved.

Introduction Sinus of Valsalva aneurysm is an aneurysm of the aortic root in the area between the annulus of the aortic valve and sinotubular junction. It is present in 0.14–4.9% of patients undergoing cardiac surgery, more frequently amongst the Asian population [1–3]. It appears most frequently in the third and fourth decade of life, and most often is manifested by a rupture

[4,5]. Men are afflicted by the illness 2–4 times more often than women [2–5]. Most studies state that most often there occurs an aneurism of the right coronary sinus, followed by aneurysm of non-coronary sinus and rarely of left coronary sinus [3,5,6]. After rupture of the aneurysm there occurs a shunt most often between the aortic root and the right ventricle. Less often there is a rupture into the right atrium [3,5,6]. Rarely into the left ventricle [7], pulmonary artery [8], interventricular septum [9,10] or pericardium [11]. We hereby present a case report of sinus of Valsalva aneurysm rupture into the right atrium.

* Corresponding author at: Second Surgical Clinic of Cardiovascular Surgery, General University Hospital, Prague, Czech Republic. E-mail address: [email protected] (A. Slautin). http://dx.doi.org/10.1016/j.crvasa.2017.01.007 0010-8650/© 2017 The Czech Society of Cardiology. Published by Elsevier Sp. z o.o. All rights reserved.

Please cite this article in press as: A. Slautin et al., Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in 44-year-old patient: Case report, Cor et Vasa (2017), http://dx.doi.org/10.1016/j.crvasa.2017.01.007

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Picture 1 – Chest X-Ray.

Case report The forty-four-year-old patient was admitted to our hospital after eight months of progressing exertional dyspnoea, occasional palpitations and swelling of the legs. The symptoms appeared after greater physical exertion, before this he had not been treated for anything. He had neither a raised temperature nor chills. On a chest X-ray he had a significantly expanded heart shadow (Picture 1). During transoesophageal echocardiography a significant left-to-right shunt was discovered via a defect approximately 15 mm wide between the aortic bulb (at the level of the base of the aortic cusps, area of transition of the non-coronary to the right sinus) and the right atrium (Picture 2); with calcification of border, many fine flail fibres approximately 25 mm long starting on the atrial side of

the defect (Pictures 3 and 4). Bicuspid aortic valve with billowing of slightly thickened cusps and calcification in right commissura with medium significant regurgitation was presented, dilation of right-side chambers with preserved systolic function of right ventricle, small tricuspid regurgitation during tenting of cusps and dilation of annulus (48–49 mm), insignificant mitral regurgitation. Ascending aorta without dilation, aortic root 36 mm. On a coronarography there was a normal finding on the coronary arteries, high flow in aorta and coronary bed, second degree aortic regurgitation. Surgery was indicated for the patient, during which a defect was discovered in the area of the non-coronary sinus going into the right atrium (Picture 5), with calcification in the annulus of the congenitally bicuspid aortic valve, without signs of endocarditis present. Abundant tissue was excised from the right atrium (Picture 6) which had originally been an aneurysm of the non-coronary sinus of the aortic root. The aortic valve was replaced with a biological prosthetic, the communication to the right atrium was closed using backing sutures by drawing both edges of the defect to the prosthetic and tricuspid annuloplasty with a ring. The operative period passed without complications, and the patient was discharged on the 6th day after the operation. During check-up transoesophageal echocardiography no intracardiac shunt was evident. Normal function of the left ventricle was recorded (EF 57%), slight increase in right cardiac chambers, reduction in the systolic function of the right ventricle trace regurgitation on the tricuspid valve, normal finding on aortic bioprosthesis (AVAi 1.2 cm2/m2), mitral regurgitation 1+. The patient was clinically compensated, and subjectively he did not state any difficulties or limitations on physical activity.

Discussion The morphology of sinus of Valsalva aneurysm was first described by Edwards, who pointed to a deficiency of normal elastic tissue in the media of the aortic sinus. On the basis of

Picture 2 – Transesophageal Echocardiography (TEE), defect between the aortic bulb and the right atrium. Please cite this article in press as: A. Slautin et al., Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in 44-year-old patient: Case report, Cor et Vasa (2017), http://dx.doi.org/10.1016/j.crvasa.2017.01.007

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Picture 3 – TEE, flail fibres in the right atrium.

Picture 4 – TEE, flail fibres in the right atrium.

this structural change, first of all a blind-ended diverticle is formed which gradually increases due to high pressure until it ruptures [12]. The anatomy of sinus of Valsalva aneurysm as a direct impact on the clinical course of the sinus of Valsalva rupture. A rupture of the right and non-coronary sinus usually causes a shunt between the aorta and outflow tract or aorta and right atrium. A rupture of left sinus of Valsalva aneurysm is less significant clinically, it causes communication to the left atrium and outflow tract of the left ventricle [3,7]. In the literature there is also a description of a rupture into the interventricular septum with significant obstruction of the outflow tract of the left ventricle [9,10]. The rupture may be expressed through substernal pain, abdominal pain, medium to marked dyspnoea. In many cases patients have the symptoms of acute heart failure, cardiac tamponades, haemodynamic dysfunctions and sudden cardiac arrest [4,5]. In

30–50% of cases sinus of Valsalva aneurysm is complicated by aortic regurgitation, in 31% of cases a defect of the ventricular septum is present as a combined heart defect, in 9% bicuspid aortic valve [4,5,13]. The survival period for untreated patients with sinus of Valsalva rupture is 1–2 years, and so early intervention is always indicated [14]. Treatment of sinus of Valsalva aneurysm rupture is traditionally surgical, although cases have been described where the defect was closed by catheterisation [4,15–18]. The defect is closed using one of three possible approaches: using aortotomia, via the cardiac chamber where the rupture occurred, or via a combination. There are two conventional techniques for closing the defect: direct suture of defect or using patch. Direct suturing is indicated for a small rupture [2,4,5,18,19]. Patch closure is used when there is a larger sinus of Valsalva rupture, where direct suturing may deform the aortic sinus and thus cause

Please cite this article in press as: A. Slautin et al., Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in 44-year-old patient: Case report, Cor et Vasa (2017), http://dx.doi.org/10.1016/j.crvasa.2017.01.007

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Picture 5 – Ruptured non-coronary sinus of Valsalva aneurysm, view from the aortic bulb.

Picture 6 – Abudant tissue in the right atrium.

insufficiency of the aortic valve or where due to a large excessive tension of tissue there may be a recurrence of the rupture. Operative mortality is between 1.9 and 3.6%, and 15year survival is around 90% [4]. Although surgical closure of a sinus of Valsalva rupture is the method of choice, it is also possible to perform a percutaneous closure of the defect in strictly indicated localisations. Cullen first described a percutaneous closure of a sinus of Valsalva rupture using the occlusive system (Rashkind umbrella) in 1994 [15]. Currently it is performed using new generation instruments: Amplatzer septal occluder or Amplatzer duct occluder [16–18,20]. The advantage of SOVA percutaneous closure is the lesser level of invasiveness, in particular in patients with serious heart failure or polymorbid complicated patients.

well as the overall state. A simple defect can often be closed percutaneously, but complicated defects in an adverse localisation and with other complications (medium to significant aortic regurgitation, extensive defect, fistula or long channel shape of aneurism, multiple ruptures) or associated with the coexistence of other pathologies (large defect of interventricular septum, active endocarditis and other congenital defects requiring surgical intervention) must be dealt with surgically.

Conclusion

Ethical statement

The decision on whether to close the defect percutaneously or surgically depends on its nature and associated pathologies, as

We declare that the work was performed in compliance with all ethical standards.

Conflict of interest None.

Please cite this article in press as: A. Slautin et al., Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in 44-year-old patient: Case report, Cor et Vasa (2017), http://dx.doi.org/10.1016/j.crvasa.2017.01.007

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Informed consent [10]

We hereby declare that the patient had signed the informed consent at the time of admission to the hospital. [11]

Funding body None.

[12]

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Please cite this article in press as: A. Slautin et al., Ruptured non-coronary sinus of Valsalva aneurysm into the right atrium in 44-year-old patient: Case report, Cor et Vasa (2017), http://dx.doi.org/10.1016/j.crvasa.2017.01.007