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Classical windsock deformity of ruptured sinus of Valsalva: an unusual appearance on transthoracic echocardiography Iranna Hirapur, 1 Rajeshwari Mantgol Veeranna, 2 Navin Agrawal 3 1 Department of Cardiology, R L Jalappa Narayana Hrudyalaya, Kolar, Karnataka, India 2 Department of Opthalmology, R L Jalappa Narayana Hrudyalaya, Kolar, Karnataka, India 3 Department of Cardiology, Care Hospital, Surat, Gujarat, India Correspondence to Dr Navin Agrawal, [email protected] Accepted 3 May 2014 To cite: Hirapur I, Veeranna RM, Agrawal N. BMJ Case Rep Published online: [ please include Day Month Year] doi:10.1136/ bcr-2014-204493 DESCRIPTION Aneurysm of sinus of Valsalva is a rare anomaly which arises from a congenital defect of the aortic media or due to damage caused by bacterial endo- carditis. It is more prevalent in men and people of Asian descent. 1 Ruptured sinus of Valsalva (RSOV) is a relatively uncommon cause of acute haemodynamic worsen- ing which is usually seen in young-aged or middle-aged individuals. We present a case of a 23-year-old man who presented with a 20-day history of symptoms of worsening breathlessness; examination showed elevated jugular venous pres- sure, high volume pulse, S3 and a continuous murmur. The patients echocardiogram showed RSOV from the right coronary sinus draining into the right ventricle with classical windsock deformity which is usually very uncommon to be seen on transthoracic echocardiogram (TTE; gures 13, videos 13). He was referred for surgical correction of the defect at another centre where he underwent successful treatment and has been asymptomatic on follow-up. Denitive diagnosis of RSOV is usually per- formed with sufcient accuracy using a TTE but sometimes requires a transoesophageal echocardio- gram (TEE) or cardiac catheterisation if the echo images are suboptimal or additional lesions need to be dened especially in cases caused by endocardi- tis. Details of involvement of other coronary sinuses and the extent of involvement and damage to the surrounding structures especially the aortic valve cusps are best obtained by a TEE. Intraprocedural TEE can also facilitate the performance of percutaneous or surgical closure of defect by assessment of any residual defects after the correction. The anomaly usually occurs in isolation but may coexist with ventricular septal defect or aortic valve regurgitation in about 3040% of patients. 12 The most common site of origin of aneurysms is from the right coronary sinus and the most common site of drainage is the right ventricle (70%). Uncorrected, the rupture almost invariably causes deterioration in heart function and has a rapid downhill course. Early surgical intervention is the treatment of choice as was performed in this case which led to a successful outcome that was maintained at 3 months of follow-up. Figure 1 Parasternal short-axis view at the level of the aortic valve showing the classical windsock appearance. Figure 2 Parasternal long-axis view showing the classical windsock appearance of the ruptured sinus of Valsalva. Figure 3 Modied parasternal short-axis view showing the classical windsock deformity. Hirapur I, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204493 1 Images in

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  • Classical windsock deformity of ruptured sinus ofValsalva: an unusual appearance on transthoracicechocardiographyIranna Hirapur,1 Rajeshwari Mantgol Veeranna,2 Navin Agrawal3

    1Department of Cardiology,R L Jalappa NarayanaHrudyalaya, Kolar, Karnataka,India2Department of Opthalmology,R L Jalappa NarayanaHrudyalaya, Kolar, Karnataka,India3Department of Cardiology,Care Hospital, Surat, Gujarat,India

    Correspondence toDr Navin Agrawal,[email protected]

    Accepted 3 May 2014

    To cite: Hirapur I,Veeranna RM, Agrawal N.BMJ Case Rep Publishedonline: [please include DayMonth Year] doi:10.1136/bcr-2014-204493

    DESCRIPTIONAneurysm of sinus of Valsalva is a rare anomalywhich arises from a congenital defect of the aorticmedia or due to damage caused by bacterial endo-carditis. It is more prevalent in men and people ofAsian descent.1

    Ruptured sinus of Valsalva (RSOV) is a relativelyuncommon cause of acute haemodynamic worsen-ing which is usually seen in young-aged ormiddle-aged individuals. We present a case of a23-year-old man who presented with a 20-dayhistory of symptoms of worsening breathlessness;examination showed elevated jugular venous pres-sure, high volume pulse, S3 and a continuousmurmur.The patients echocardiogram showed RSOV

    from the right coronary sinus draining into theright ventricle with classical windsock deformitywhich is usually very uncommon to be seen ontransthoracic echocardiogram (TTE; figures 13,videos 13). He was referred for surgical correctionof the defect at another centre where he underwentsuccessful treatment and has been asymptomatic onfollow-up.Definitive diagnosis of RSOV is usually per-

    formed with sufficient accuracy using a TTE butsometimes requires a transoesophageal echocardio-gram (TEE) or cardiac catheterisation if the echoimages are suboptimal or additional lesions need tobe defined especially in cases caused by endocardi-tis. Details of involvement of other coronarysinuses and the extent of involvement and damageto the surrounding structures especially the aorticvalve cusps are best obtained by a TEE.Intraprocedural TEE can also facilitate the

    performance of percutaneous or surgical closure ofdefect by assessment of any residual defects afterthe correction.The anomaly usually occurs in isolation but may

    coexist with ventricular septal defect or aortic valveregurgitation in about 3040% of patients.1 2 Themost common site of origin of aneurysms is fromthe right coronary sinus and the most common siteof drainage is the right ventricle (70%).Uncorrected, the rupture almost invariably

    causes deterioration in heart function and has arapid downhill course. Early surgical intervention isthe treatment of choice as was performed in thiscase which led to a successful outcome that wasmaintained at 3 months of follow-up.

    Figure 1 Parasternal short-axis view at the level of theaortic valve showing the classical windsock appearance.

    Figure 2 Parasternal long-axis view showing theclassical windsock appearance of the ruptured sinus ofValsalva.

    Figure 3 Modified parasternal short-axis view showingthe classical windsock deformity.

    Hirapur I, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204493 1

    Images in

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  • Learning points

    Ruptured sinus of Valsalva (RSOV) is a relatively uncommoncause of acute haemodynamic worsening which is usuallyseen in young-aged or middle-aged individuals but one ofthe most common causes of continuous murmur in them.

    Classical windsock appearance is most commonly seen ontransesophageal echocardiogram but it can also sometimesbe seen on a transthoracic echocardiogram.

    RSOV if managed appropriately with surgical correction ordevice closure can be successfully cured and can have anasymptomatic long-term outcome.

    Competing interests None.

    Patient consent Obtained.

    Provenance and peer review Not commissioned; externally peer reviewed.

    REFERENCES1 Moustafa S, Mookadam F, Cooper L, et al. Sinus of Valsalva aneurysms47 years of

    a single center experience and systematic overview of published reports. Am J Cardiol2007;99:115964.

    2 Wang ZJ, Zou CW, Li DC, et al. Surgical repair of sinus of Valsalva aneurysm in Asianpatients. Ann Thorac Surg 2007;84:15660.

    Video 1 Parasternal short axis view showing the classical windsockappearance.

    Video 2 Parasternal long axis view showing the classical wind sockappearance of the rupture sinus of Valsalva.

    Video 3 parasternal short axis view with colour doppler showing thecommunication of the ruptured sinus of Valsalva to the right ventricle.

    Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visithttp://group.bmj.com/group/rights-licensing/permissions.BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission.

    Become a Fellow of BMJ Case Reports today and you can: Submit as many cases as you like Enjoy fast sympathetic peer review and rapid publication of accepted articles Access all the published articles Re-use any of the published material for personal use and teaching without further permission

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    2 Hirapur I, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-204493

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    Classical windsock deformity of ruptured sinus of Valsalva: an unusual appearance on transthoracic echocardiographyDescriptionReferences