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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 31  |  Issue : 3  |  Page : 175-178

Atretic Coronary sinus orifice and a diverticulum of coronary sinus associated with persistent left superior vena cava and accessory pathway


1 Vascular and Endovascular Surgery Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
2 Vascular and Endovascular Surgery Research Center, Mashhad University of Medical Sciences, Mashhad, Iran

Date of Submission24-May-2020
Date of Decision29-Dec-2020
Date of Acceptance16-Jun-2021
Date of Web Publication26-Oct-2021

Correspondence Address:
Hedieh Alimi
Vascular and Endovascular Surgery Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcecho.jcecho_55_20

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  Abstract 


Coronary orifice atresia and diverticula are considered rare congenital conditions. Our understanding of the coronary sinus (CS) and venous drainage anatomy plays a crucial role in performing interventions and surgical procedures required to improve treatment options and related prognosis for these patients. The case study described herein involves a 29-year-old female patient who was diagnosed with coronary orifice atresia and diverticula. The patient's electrocardiography revealed normal sinus rhythm, short PR interval, and delta wave. Transthoracic echocardiography results showed the subject also suffered from mild CS dilation associated with coronary orifice atresia and diverticula. Our clinical intervention included the use of agitated saline injection which was administered intravenously into the patient's left arm. Our clinical observations during the administration of agitated saline solution did not indicate the presence of any detectable air bubbles entering into the CS. The results of two-dimensional and color flow Doppler study showed that CS connection to the right atrium appeared to be atretic. During our examination, we were also successful in detecting a pulsatile free echo space which was connected through an isthmus to the CS. Electrophysiology study and coronary angiogram performed on the subject confirmed our clinical diagnosis and findings of CS atresia, diverticula, and left superior vena cava.

Keywords: Accessory pathway, atresia, coronary sinus, diverticula, left-sided superior vena cava


How to cite this article:
Tayyebi M, Alimi H. Atretic Coronary sinus orifice and a diverticulum of coronary sinus associated with persistent left superior vena cava and accessory pathway. J Cardiovasc Echography 2021;31:175-8

How to cite this URL:
Tayyebi M, Alimi H. Atretic Coronary sinus orifice and a diverticulum of coronary sinus associated with persistent left superior vena cava and accessory pathway. J Cardiovasc Echography [serial online] 2021 [cited 2021 Dec 8];31:175-8. Available from: https://www.jcecho.org/text.asp?2021/31/3/175/329313




  Introduction Top


Atretic coronary sinus (CS) orifice is a rare congenital anomaly and usually is associated with other cardiac malformations including atrial septal defects, abnormalities of systemic, pulmonary venous drainage, and persistent left superior vena cava (LSVC). Clinical conditions resulting from LSVC that are associated with the CS orifice atresia may be responsible for blood drainage that occurs retrograde from the CS system into the innominate vein as well as the right superior vena cava and ultimately terminates into the right atrium. Furthermore, patients diagnosed with such anatomical abnormalities also experience drainage of coronary venous blood into the right atrium without exhibiting any hemodynamic or physiological effect.[1],[2] LSVC is the only path for drainage of CS blood into the right atrium. In certain clinical cases, patients may also suffer from an interruption in drainage of CS which may result in coronary venous hypertension, myocardial congestion, and considerable patient morbidity and mortality.[3]

CS aneurysm or diverticula is considered another form of rare congenital abnormality of the CS. Our case study shows that a greater understanding of the CS congenital abnormalities is required in order to improve the standard of care for procedures requiring access through the CS in these patients.[2] Our clinical evaluation involves a patient diagnosed with a rare case of atretic orifice and diverticulum of the CS, which are both associated with LSVC and accessory pathway.


  Case Report Top


A 29-year-old white female patient who suffered from palpitation was referred to Quaem Hospital for further evaluation. The patient's vital signs at the time of admission indicated an O2 saturation level of 98% prior to receiving oxygen therapy, while all other vital signs appeared normal.

The patient's electrocardiography revealed normal sinus rhythm, short PR interval, and delta wave.

Transthoracic echocardiography results showed normal left ventricle size and systolic function with mild CS dilation. A pulsatile echo-free space was observed at the interventricular septum base and posterior side of the left ventricle indicating a maximum diameter of 1.96 cm × 1.33 cm [Figure 1] and Movie 1] which was connected through isthmus to the CS. Two-dimensional and color flow Doppler study results indicated that CS connection to the right atrium appeared to be atretic. The diagnosis was further confirmed using agitated saline injection. We did not observe any air bubbles entering from the right atrium into the CS during the agitated saline injection procedure [Figure 2].
Figure 1: Two-dimensional echocardiography showed diverticula in inferior side of the left ventricle with diameter of 1.96 cm × 1.33 cm

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Figure 2: During agitated saline injection to the left hand, no bubble passed from the right atrium to the coronary sinus. Diverticulum was connected through an isthmus to the coronary sinus. CS, coronary sinus; D, diverticula; I, isthmus

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The accessory pathway electrophysiology procedure at the time of CS cannulation was challenging. Therefore, we elected to perform a coronary angiogram procedure which focused on the venous phase. Our evaluation showed the presence of CS atresia, diverticula (which was pulsatile echo-free space in echocardiography), and LSVC [Figure 3].
Figure 3: In coronary angiogram involving the venous phase, coronary sinus atresia (white arrow) was noted. Blood drainage direction was retrograde from coronary sinus to the left superior vena cava. Diverticula (d) was also noted

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Based on our clinical investigations as described herein, we have determined that LSVC is the only pathway for CS blood drainage when considering the anatomy of the disease. We have successfully shown that the diseased anatomy is responsible for the incorrect and reversed blood drainage in the patient. Such disorders can be diagnosed through echocardiography procedures using color flow Doppler and pulse Doppler studies. The combination of echocardiography alone with agitated saline injection would not have resulted in detecting the air bubbles entering into the CS due to retrograde blood drainage and CS atresia [Figure 4].
Figure 4: Echocardiography procedure using color flow Doppler and pulse Doppler studies showed blood flow direction in the left superior vena cava was retrograde

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Our study results suggest a conservative clinical approach, and imaging modality is required for diagnoses and management of patients suffering from similar abnormal anatomy as described herein.[4] However, patients suffering from accessory pathway, and arrhythmias should be considered as potential candidates for additional ablation therapy.


  Discussion Top


Patients suffering from CS orifice atresia with a persistent left-sided superior vena cava may not demonstrate any signs of clinical complications unless they have been subjected to cannulation procedures. These procedures include mapping, ablation of arrhythmias, retrograde cardioplegia, targeted drug delivery, stem cell therapy, placement of central catheters, pacemakers, and defibrillators leads; or ligation of the left-sided superior vena cava in the Fontan; or when Bidirectional Glenn procedures are performed.[1],[2],[3],[4],[5]

Patients with abnormal anatomical conditions as stated earlier may experience further complications including CS thrombosis. However, existing standards of care and management guidelines for such patients do not emphasize the administration of thromboprophylaxis therapy during CS cannulation at this time.

CS aneurysms or diverticula are also considered rare forms of congenital anomalies. Diverticula associated with a posteroseptal accessory atrioventricular connection was first described in 1985 by Gerlis et al.[6]

Sudden death caused by a large CS diverticulum was previously reported by Ho et al. However, additional clinical investigations are required to study the possible relation between the rate of such occurrences in the cases with aneurysms and possible morbidity and sudden death.[7]

Our findings were similar to previous reports (e.g. LM Gerlis et a.l, 1984; Amin Sabet et al., 2015; Salah D. Qanadli, 2014; Ching-Pei Chen et al., 2007; Yokota M, 1989; Paul Chun Yih Lim, 2013) regarding the association of CS orifice atresia with LSVC.

In addition to the CS orifice atresia and LSVC described in previous studies, this patient also suffered from diverticula and accessory pathway. The clinical cases with diverticula and accessory pathway in the presence of other medical conditions such as CS orifice atresia and LSVC have not been previously well described in any published clinical investigations to date.

Although echocardiography is a modality for routine assessment of patients with congenital heart diseases, computed tomography (CT) and cardiovascular magnetic resonance imaging (MRI) have an adjunct role in the assessment of more complex congenital heart diseases.[8]

In conclusion, our information about the anatomy of the CS, cardiac venous drainage, and related congenital anomalies could significantly reduce clinical complications related to the diagnosis and treatment of such abnormalities. We believe that our clinical observations may considerably increase the rate of clinical success in the treatment of such patients who had multiple ablation failures of the accessory pathway.[9]

Improved knowledge of the anatomy of the CS may provide significant clinical benefits in dealing with difficulties encountered during cannulation of the CS. Therefore, angiography can be performed with special attention to the venous phase of the angiogram when treating patients with such anatomical abnormalities. Alternatively, we suggest to use a multidetector CT[10] or magnetic resonance imaging of the heart to further assist with the diagnosis of the disease.[6]

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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgments

We would like to extend our gratitude to Mashhad University of Medical Sciences for the support of this study.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sabet A, Karimi A, DeMaria A. Atretic coronary sinus orifice with a persistent left superior vena cava. Rev Esp Cardiol (Engl Ed) 2015;68:527.  Back to cited text no. 1
    
2.
Chen CP, Lee KL, Liao CY, Huang CL. Coronary sinus atresia with cardiac veins draining into both the left superior vena cava and large coronary fistulae, treated by percutaneous coil embolization. Acta Cardiol Sin 2007;23:119-24.  Back to cited text no. 2
    
3.
Yokota M, Kyoku I, Kitano M, Shimada I, Mizuhara H, Sakamoto K, et al. Atresia of the coronary sinus orifice. Fatal outcome after intraoperative division of the drainage left superior vena cava. J Thorac Cardiovasc Surg 1989;98:30-2.  Back to cited text no. 3
    
4.
Tham EB, Ross DB, Giuffre M, Smallhorn J, Noga ML. Images in cardiovascular medicine. Cardiac magnetic resonance imaging of a coronary sinus diverticulum associated with congenital heart disease. Circulation 2007;116:e541-4.  Back to cited text no. 4
    
5.
Lim PC, Baskaran L, Ho KL, Teo WS, Ching CK. Coronary sinus ostial atresia and persistent left-sided superior vena cava: Clinical significance and strategies for cardiac resynchronization therapy. Int J Angiol 2013;22:199-202.  Back to cited text no. 5
    
6.
Gerlis LM, Davies MJ, Boyle R, Williams G, Scott H. Pre-excitation due to accessory sinoventricular connexions associated with coronary sinus aneurysms. A report of two cases. Br Heart J 1985;53:314-22.  Back to cited text no. 6
    
7.
Ho SY, Gupta I, Anderson RH, Lendon M, Kerr I. Aneurysm of the coronary sinus. Thorax 1983;38:686-9.  Back to cited text no. 7
    
8.
Baumgartner H, De Backer J, V Babu-Narayan S, Budts W, Chessa M, Diller GP, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. European Heart Journal 2021;42:563-645. doi:10.1093/eurheartj/ehaa554.  Back to cited text no. 8
    
9.
Payami B, Shafiee A, Shahrzad M, Kazemisaeed A, Davoodi G, Yaminisharif A. Posteroseptal accessory pathway in association with coronary sinus diverticulum: Electrocardiographic description and result of catheter ablation. J Interv Card Electrophysiol 2013;38:43-9.  Back to cited text no. 9
    
10.
Ozturk E, Kafadar C, Dogan M, Uz O. RE: Anomalous cardiac venous connection to the left atrium associated with coronary sinus atresia. Korean J Radiol 2014;15:879-81.  Back to cited text no. 10
    


    Figures

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



 

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