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A case of coronary sinus ostium atresia with muscular obstruction in a complex univentricular heart managed in stages

Published online by Cambridge University Press:  10 October 2025

Kentaro Suzuki,
Jun SatoOpen the ORCID record for Jun Sato[Opens in a new window] and
Naoki OhashiOpen the ORCID record for Naoki Ohashi[Opens in a new window]
Kentaro Suzuki
Affiliation:
Department of Pediatric Cardiology, Children’s Heart Center, Nagoya University Hospital, Nagoya, Japan
Jun Sato*
Affiliation:
Department of Pediatric Cardiology, Chukyo Children’s Heart Center, Japan Community Health Organization Chukyo Hospital , Nagoya, Japan
Naoki Ohashi
Affiliation:
Department of Pediatric Cardiology, Children’s Heart Center, Nagoya University Hospital, Nagoya, Japan
*
Corresponding author: Jun Sato; Email: sato111.ped@gmail.com
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Abstract

We report a rare case of coronary sinus ostium atresia diagnosed due to impaired cardiac function caused by Marshall vein stenosis, which occurred during surgical treatment. Transcatheter perforation of the coronary sinus ostium and stenting of the Marshall vein stenosis improved ventricular function, enabling subsequent surgical enlargement. This staged strategy minimised surgical risks and demonstrated a reliable and effective treatment for such challenging conditions.

Keywords

Coronary sinus ostium atresiamuscular obstructionuniventricular hearttranscatheter perforationmarshal vein stenting

Information

Type
Case Report
Information
Cardiology in the Young , Volume 35 , Issue 11 , November 2025, pp. 2376 - 2378
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Coronary sinus ostium atresia is an extremely rare congenital anomaly, with an estimated incidence of 0.08% to 0.1% of the population. Reference Shum, Kim and Choe1,Reference Hiroshima, Goya and Nagashima2 The coronary sinus is usually covered by a thin, membranous-like tissue, and coronary venous blood flow returns to the innominate vein via the Marshall vein, also known as the left superior caval vein. Reference Penny, Geva, Shaddy and Penny3 Although most cases are asymptomatic and rarely diagnosed, obstruction of the coronary venous blood pathway can impair coronary circulation and ventricular function.

Case

The patient was a 5-year-old girl who had been prenatally diagnosed with single ventricle physiology. She was born at 39 weeks and 2 days of gestation, weighing 2768 g. Postnatal evaluation confirmed the diagnosis of single right ventricle, common atrioventricular valve, pulmonary atresia, and major aortopulmonary collateral arteries. She underwent unifocalization and a modified Blalock–Thomas–Taussig shunt at 7 months of age, followed by atrioventricular valvuloplasty for severe regurgitation at 2 years of age. At age 4, an additional central shunt and artificial valve replacement were performed for advanced cyanosis and uncontrollable atrioventricular valve regurgitation. During surgery, the central shunt was inadvertently connected to the Marshall vein, which was corrected immediately.

After the operation, cardiac catheterisation revealed elevated ventricular end-diastolic pressure and central venous pressure of 11 mmHg and 12 mmHg, respectively, and a reduced ventricular ejection fraction of 45%. The levophase of aortography revealed that coronary venous return drained into the innominate vein via the Marshall vein instead of the atrium, confirming the diagnosis of coronary sinus ostium atresia. The Marshall vein was stenotic at the site where the central shunt had been incorrectly connected previously (Figure 1a), causing a pressure gradient of 4 mmHg, and the pressure in the coronary sinus was 16 mmHg. Furthermore, angiography from the central shunt demonstrated a small fistula between the left pulmonary artery and the Marshall vein at the stenosis (Figure 1b). After discussion with the cardiovascular team, a decision was made to perform staged treatment: transcatheter perforation of the atretic coronary sinus ostium concomitant with covered stent implantation for Marshall vein stenosis and fistula, followed by surgical enlargement.

Figure 1.

(a ) Angiography of the Marchall vein demonstrating coronary sinus ostium atresia and stenosis in the middle. (b ) Levo phase of the central shunt angiography showing the left pulmonary artery to the Marchall vein fistula. (c) , (d ) Atretic coronary sinus was successfully perforated with Nykanen RF wire. (e ) Transesophageal cardiography confirmed newly created communication between the coronary sinus and the atrium. (f ) Final angiography after covered stent implantation showing patent coronary sinus ostium and improved Marshall vein stenosis.

Figure 2.

( a ) Schematic representation of the intraoperative findings showing the “crack” of the perforated atrial wall by catheter interventions (red arrow). There was no membranous-like tissue around it. ( b ) The crack was enlarged by resection of the atrial wall vertically.

Under general anaesthesia, a 4 French Judkins right catheter and a 2.9 French Leonis Mova steerable microcatheter (Sumitomo Bakelite, Tokyo, Japan) with a Nykanen RF wire (Baylis Medical, Ontario, Canada) system were advanced from the left jugular vein into the Marshall vein. Multiple applications of radiofrequency power were required for successful perforation of the coronary sinus ostium (Figure 1c, d). Subsequently, 2 × 20 mm Coyote ES balloon dilatation catheters (Boston Scientific Corporation, Massachusetts, US) and 5 × 20 mm Sterling balloon dilatation catheters (Boston Scientific Corporation) were used for staged dilatations. Transesophageal echocardiography confirmed newly created flow from the coronary sinus to the atrium (Figure 1e). Lastly, a 7 × 29 mm VIAVAHN VBX covered stent (W.L. Gore & Associates, Delaware, US) was successfully placed at the point of the Marshall vein stenosis and fistula (Figure 1f). All procedures were completed without any complications. The pressure gradient in the Marshall vein and the fistula completely disappeared.

A month later, catheterisation showed improved ventricular end-diastolic pressure, central venous pressure, and ventricular ejection fraction of 8 mmHg, 7 mmHg, and 63%, respectively. Therefore, surgical enlargement of the coronary sinus ostium was performed as planned. In the intraoperative macroscopic finding, the created coronary sinus ostium was surrounded by thickened muscular tissue rather than a membranous-like one (Figure 2). The postoperative course was uneventful, and she is currently awaiting the Glenn procedure.

Discussion

Congenital coronary sinus ostium atresia is usually asymptomatic, making diagnosis challenging. According to previous reports, deterioration of coronary circulation due to surgical ligation of the Marshall vein can cause severe consequences, including death. Reference Foale, Baron and Rickards4 In the setting of single ventricle circulation, it has also been reported that elevated superior caval vein pressure after the bidirectional Glenn procedure leads to worsening of ventricular function. Reference Yokota, Kyoku and Kitano5 In our case, the condition remained undiagnosed until cardiac function deteriorated due to Marshall vein stenosis.

There are several reports of creating communication between the atrium and the coronary sinus by both surgical and transcatheter interventions. Reference Ohta, Sakamoto, Kado, Nishioka and Yokota6,Reference Dalby, Mitchell, Greiten, Reemtsen, Eisenring and Zakaria7,Reference Kops, Pangburn, Barber and Seckeler8,Reference El-Said, Hegde and Moore9 Although creation of a wide and certain communication was needed in our case, the surgical approach posed a potential risk of postoperative cardiovascular collapse considering her impaired cardiac function. Additionally, identification of the atretic coronary sinus ostium from the atrial side was expected to be challenging. Therefore, we attempted to reduce coronary venous pressure as much as possible to improve coronary circulation in advance of the surgery and successfully performed transcatheter perforation of the coronary sinus ostium and stenting for Marshall vein stenosis. To eliminate a small fistula from the left pulmonary artery to the Marshall vein concomitantly, a covered stent was used.

Another noteworthy finding of our case was that the atretic coronary sinus ostium was covered with muscular tissue, not a membranous-like one. It was corroborated by the fact that we required multiple applications of radiofrequency power to perforate the coronary sinus ostium completely. Although there was an option of coronary sinus ostium stenting due to concerns about restenosis, we selected subsequent surgical enlargement for a more reliable procedure in this complex heart.

In conclusion, staged coronary sinus ostium creation can be a less invasive and more reliable option for coronary sinus ostium atresia causing cardiac dysfunction. The transcatheter approach was feasible even with muscular obstruction.

Acknowledgements

The authors would like to thank our colleagues who contributed to the care of these patients. We are also grateful to Dr Sung-Hae Kim and Dr Hiroshi Nishikawa for their extensive catheter-related technical support.

Financial support

This research received no specific grant from any funding agency, commercial or not-for-profit sectors.

Competing interests

The authors declare no conflict of interest.

Ethical standards

The authors assert that all work reported complies with the ethical standards of the Helsinki Convention, and the institutional and national research committee. Consent for publication of this report was obtained from the family members.

References

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Figure 0

Figure 1.(a) Angiography of the Marchall vein demonstrating coronary sinus ostium atresia and stenosis in the middle. (b) Levo phase of the central shunt angiography showing the left pulmonary artery to the Marchall vein fistula. (c), (d) Atretic coronary sinus was successfully perforated with Nykanen RF wire. (e) Transesophageal cardiography confirmed newly created communication between the coronary sinus and the atrium. (f) Final angiography after covered stent implantation showing patent coronary sinus ostium and improved Marshall vein stenosis.

Figure 1

Figure 2.(a) Schematic representation of the intraoperative findings showing the “crack” of the perforated atrial wall by catheter interventions (red arrow). There was no membranous-like tissue around it. (b) The crack was enlarged by resection of the atrial wall vertically.