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Year : 2022  |  Volume : 6  |  Issue : 1  |  Page : 69-71

A patient with type IV unroofed coronary sinus presenting with wide QRS Tachycardia

Department of Cardiology, Dayanand Medical College, Ludhiana, Punjab, India

Date of Submission09-Jun-2021
Date of Acceptance26-Dec-2021
Date of Web Publication14-Mar-2022

Correspondence Address:
Dr. Ashish Kumar
Department of Cardiology, Dayanand Medical College, Ludhiana, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_25_21

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Unroofed coronary sinus (UCS) is a congenital heart disease characterized by defects in the formation of the coronary sinus. UCS usually manifests as dyspnea on effort, fatigue, or palpitation. However, here, we present a case of a 53-year-old female whose presentation was wide QRS tachycardia. In this interesting case, we describe the role of various echocardiographic modalities used to reach its elusive diagnosis.

Keywords: Congenital heart disease, unroofed coronary sinus, ventricular tachycardia, wide QRS tachycardia

How to cite this article:
Kumar A, Tandon R, Goyal A, Wander GS. A patient with type IV unroofed coronary sinus presenting with wide QRS Tachycardia. J Indian Acad Echocardiogr Cardiovasc Imaging 2022;6:69-71

How to cite this URL:
Kumar A, Tandon R, Goyal A, Wander GS. A patient with type IV unroofed coronary sinus presenting with wide QRS Tachycardia. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2022 [cited 2023 Jun 10];6:69-71. Available from: https://jiaecho.org/text.asp?2022/6/1/69/339672

  Introduction Top

Unroofed coronary sinus (UCS) is a rare congenital heart disease in which there is partial or complete absence of the roof of the coronary sinus, which results in a communication between the coronary sinus and the left atrium. It is often associated with persistent left superior vena cava and other forms of complex congenital heart diseases (CHD). The diagnosis of this lesion is often difficult because of nonspecific clinical features. Echocardiographic modalities play an important role in its diagnostic evaluation and treatment planning.

  Case Report Top

A 53-year-old female presented to the emergency room with hemodynamically unstable wide QRS tachycardia [Figure 1]. She was cardioverted immediately. Post-cardioversion electricardiogram showed normal sinus rhythm with incomplete right bundle branch block [Figure 2]. Possibility of ventricular tachycardia originating from right ventricular (RV) apex versus supraventricular tachycardia with aberrancy having left bundle branch block morphology was kept. Before this episode, she had been admitted twice for emergency treatment of supraventricular tachyarrhythmias. She did not have any past history of diabetes mellitus, coronary artery disease, hypertension, or cerebrovascular accident. Her vitals were- peripheral oxygen saturation 90% on room air, blood pressure 140/80 mm of Hg, and pulse rate 76 bpm regular. Her transthoracic echocardiogram (echo) showed dilated coronary sinus (CS), right atrium (RA), and RV with normal biventricular functions with mild tricuspid regurgitation (pulmonary arterial systolic pressure = 50 mmHg). Color Doppler imaging pointed toward turbulence in dilated CS [Figure 3]. Agitated bubble saline contrast injected via left brachiocephalic vein showed initial opacification of CS, left atrium (LA), and left ventricle within three cardiac cycles followed by opacification of RA and RV, thereby confirming with diagnosis of persistent left superior vena cava (LSVC) with UCS type IV [Video 1]. Transesophageal echo (TEE) showed the absence of terminal portion of CS with left to right shunt [Figure 4] and [Video 2], i.e., blood flow from LA to CS. TEE also showed prolapse of anterior leaflet of mitral valve with moderate mitral regurgitation [Video 3]. The patient was put on antiarrhythmic drugs (amiodarone, diltiazem) and warfarin and advised electrophysiological (EP) study with radiofrequency ablation and surgery, but she refused for same. She has been on regular follow-up for the last 3 years and doing well despite experiencing intermittent episodes of atrial fibrillation.
Figure 1: 12-lead electrocardiogram showing regularly regular tachycardia with negative concordance with atrioventricular dissociation having north-west axis with negative QRS in leads II, III, avf

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Figure 2: Postcardioversion electrocardiography showing sinus rhythm with incomplete right bundle branch block

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Figure 3: Color Doppler image in parasternal long-axis view showing turbulence in dilated coronary sinus

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Figure 4: Transesophageal echocardiographic two-chamber view at mid-esophagus level showing defect in the terminal portion of the coronary sinus

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Video 1: Transesophageal echocardiography showing initial opacification of coronary sinus and left atrium, left ventricle followed by right atrium and right ventricle within three cardiac cycles of injecting agitated saline contrast in the left antecubital vein.[Additional file 1]

Video 2: Transesophageal echocardiography showing exact location of coronary sinus atrial septal defect with the absence of terminal portion of roof of coronary sinus with left to right shunt.[Additional file 2]

Video 3: Transesophageal echocardiography showing prolapse of anterior leaflet of mitral valve with moderate mitral regurgitation.[Additional file 3]

  Discussion Top

The combination of an UCS with a persistent LSVC is a rare CHD first described by Raghib et al. in 1965. CS is an anatomical structure of the heart connecting the coronary venous system to the RA. UCS is a CHD characterized by defects in the formation of the wall of CS. UCS occurs when there is a partial or complete absence of the wall separating inferior wall of LA from the CS (i.e. roof of CS), resulting in 'unroofing', allowing a communication between the LA and the CS. Since the CS drains into RA, a shunt is thus created between LA and RA. CS, as well as SVC, develop from the anterior and common cardinal veins. Normally, the LSVC regresses at the sixth fetal month, and a small portion remains as the left superior intercostal vein and CS. With the deficiency of the left arteriovenous fold, the left side of the sinus venosus maintains continuity and represents the termination of the LSVC in the LA. The LSVC does not regress in 0.5% of the general population and in 3%–10% of people with CHD. LSVC is associated with UCS in 75%.[1],[2] CS atrial septal defects (ASDs) are very rare representing less than 1% of all ASDs. These defects could be missed on echo and are often diagnosed intraoperatively or at necropsy. They should be considered if the CS is dilated, especially in the presence of LSVC and right heart dilatation.

Kirklin and Barrat-Boyes described four types of UCS: type I, completely unroofed with LSVC; type II, completely unroofed without LSVC; type III, partially unroofed midportion; type IV, partially unroofed terminal portion.[3]

TEE plays an important role in UCS evaluation, especially in unstable patients in whom cardiac magnetic resonance imaging cannot be done. There are cases in the literature in which the contribution of these techniques was essential for a confirmatory diagnosis, sometimes in obese patients or after cardiac surgery. Using TEE, best views for CS evaluation are lower esophageal four-chamber view and the mid-esophageal long-axis views.[4]

Arrhythmias are well described in patients with ASDs. Atrial tachyarrhythmias such as atrial flutter and atrial fibrillation are rare in childhood but become increasingly common with older age, larger shunt size, and comorbidities. Incidence of arrhythmias decreases after closure of ASD but remains higher in comparison to general population, particularly with late age of repair.[5] Ventricular tachycardias are rare in ASDs; only few case reports are available. The presence of ventricular tachycardia in CS ASD can be explained due to the deformation of the atrioventricular junction, Koch's triangle, and ventricular septum due to the large CS. Ventricular tachycardia possibly is a result of reentrant circuit due to chronic stretch and resultant fibrosis in the upper septum with advancing age.[6] In our case, as EP study was not done, we could not confirm the origin of tachycardia which may or may not be related to congenital defect.

  Conclusion Top

UCS is a rare CHD. Presentation in the fifth decade with wide QRS tachycardia is rarely reported. Multimodality imaging with EP mapping is recommended as an optimal approach in such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Chauvin M, Shah DC, Haïssaguerre M, Marcellin L, Brechenmacher C. The anatomic basis of connections between the coronary sinus musculature and the left atrium in humans. Circulation 2000;101:647-52.  Back to cited text no. 1
Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr. Surgical experience with unroofed coronary sinus. Ann Thorac Surg 1979;27:418-25.  Back to cited text no. 2
Kirklin JW, Barrat-Boyes BG. Cardiac Surgery. John Wiley & Sons: New York; 1986.  Back to cited text no. 3
Ootaki Y, Yamaguchi M, Yoshimura N, Oka S, Yoshida M, Hasegawa T. Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment. J Thorac Cardiovasc Surg 2003;126:1655-6.  Back to cited text no. 4
Williams MR, Perry JC. Arrhythmias and conduction disorders associated with atrial septal defects. J Thorac Dis 2018;10:S2940-4.  Back to cited text no. 5
Bohora S, Singh P, Shah K. Partial unroofed coronary sinus associated with upper septal ventricular tachycardia and atrioventricular nodal reentrant tachycardia. Indian Pacing Electrophysiol J 2015;15:68-72.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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