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A rare case: adult cor triatriatum sinister with tricuspid regurgitation

Published online by Cambridge University Press:  24 July 2025

Onur Benli Open the ORCID record for Onur Benli [Opens in a new window] ,
Mete Kubilay Kasap Open the ORCID record for Mete Kubilay Kasap [Opens in a new window]  and
Mehmet Sah Topcuoglu Open the ORCID record for Mehmet Sah Topcuoglu [Opens in a new window]
Onur Benli*
Affiliation:
Cardiovascular Surgery, Department of Cardiovasculary Surgery, Adana City Training and Research Hospital, Adana, Turkey
Mete Kubilay Kasap
Affiliation:
Cardiovascular Surgery, Department of Cardiovasculary Surgery, Adana City Training and Research Hospital, Adana, Turkey
Mehmet Sah Topcuoglu
Affiliation:
Cardiovascular Surgery, Department of Cardiovasculary Surgery, Cukurova University, Adana, Turkey
*
Author for correspondence: Onur Benli, Cardiovascular Surgery, Department of Cardiovasculary Surgery, Adana City Training and Research Hospital, Adana, Turkey. E-mail: dronurbenli@gmail.com
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Abstract

Background:

Cor triatriatum sinister is a rare congenital cardiac anomaly, occurring in approximately 1 in 1,000 congenital cases. Although typically diagnosed in infancy because of significant haemodynamic consequences, cases in adulthood are uncommon and may present with nonspecific symptoms.

Case presentation:

A 31-year-old male presented with progressive dyspnoea and haemoptysis. Transthoracic echocardiography revealed a fibrous membrane dividing the left atrium, with a 5-mm fenestration permitting communication between an accessory chamber and the main left atrial cavity; four pulmonary veins drained into the accessory chamber. In addition, an atrial septal defect and pulmonary hypertension were identified. The patient underwent surgical correction, which included resection of the membrane, closure of the atrial septal defect using a Dacron patch, and DeVega tricuspid annuloplasty. Intraoperative transesophageal echocardiography confirmed absence of residual shunt and tricuspid regurgitation with improved pulmonary artery pressures.

Conclusion:

This case underscores the necessity for early recognition and timely surgical intervention in adult cor triatriatum sinister cases to prevent complications such as right ventricular failure and severe pulmonary hypertension.

Keywords

Cor triatriatum sinistertricuspid regurgitationatrial septal defectpulmonary hypertensioncardiac surgery

Information

Type
Case Report
Information
Cardiology in the Young , Volume 35 , Issue 6 , June 2025 , pp. 1305 - 1307
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Creative Commons
Creative Common License - CCCreative Common License - BY
This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use, distribution and reproduction, provided the original article is properly cited.
Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Adult presentation of cor triatriatum sinister is extremely rare, especially when associated with severe tricuspid regurgitation. In cor triatriatum sinister, a fibromuscular membrane subdivides the left atrium into a posterosuperior chamber—receiving the pulmonary veins—and an anteroinferior chamber, which houses the mitral valve and left atrial appendage. Although the condition usually manifests in infancy, cases with an adequately sized fenestration may remain undiagnosed until adulthood. Common clinical presentations in such patients include dyspnoea and haemoptysis, with echocardiography serving as the primary diagnostic tool.Reference Rudienė, Hjortshøj and Glaveckaitė1, Reference Kilkenny and Frishman2

Case presentation

A 31-year-old male with no significant past medical history presented to the emergency department with a one-month history of progressively worsening dyspnoea and recurrent haemoptysis. Vital signs on admission were stable with a blood pressure of 120/70 mmHg and a heart rate of 70 bpm in sinus rhythm. Physical examination revealed a mid-systolic murmur audible over the left clavicular area. Chest radiography demonstrated mild pulmonary congestion with a normal cardiothoracic ratio.

Transthoracic echocardiography identified an abnormal septum within the left atrium (Figure 1). Detailed evaluation revealed that the left atrium was divided by a fibrous membrane forming an accessory chamber; a 5-mm perforation allowed communication between the chambers, and all four pulmonary veins were seen draining into the accessory chamber (Figure 2). In addition, an atrial septal defect was documented.

Figure 1.

Transthoracic echocardiography demonstrating an abnormal septum (arrow) within the left atrium. Abbreviations: LA = left atrium; LV = left ventricle.

Figure 2.

(a) Four pulmonary veins draining into the accessory chamber. (b) Communication between the accessory chamber and the main left atrium via a 5-mm fenestration (yellow arrow).

Surgical procedure

Under general anaesthesia, a median sternotomy was performed. Intraoperative findings included a persistent left superior vena cava with the absence of an innominate vein. Cardiopulmonary bypass was instituted with arterial cannulation of the ascending aorta and venous cannulation of the inferior vena cava, superior vena cava, and persistent left superior vena cava. The patient was cooled to 32°C and cardiac arrest was achieved using blood cardioplegia.

A right atriotomy revealed a normal-sized coronary sinus, a large atrial septal defect, and the fibrous membrane dividing the left atrium. The mitral valve was intact, and the persistent left superior vena cava drained into the posterior left atrial compartment (Figure 3). Subsequently, a left atriotomy performed anterior to the right pulmonary veins exposed a 5-mm fenestration in the fibrous membrane that permitted limited blood flow between the two chambers. Resection of the membrane was carried out to restore a single left atrial cavity. The atrial septal defect was closed with a Dacron patch, ensuring that persistent left superior vena cava drainage remained directed into the right atrium. Owing to annular dilatation, a DeVega tricuspid annuloplasty was also performed.

Figure 3.

Intraoperative view from the surgeon’s perspective. The fibrous septum and the 5-mm fenestration (arrow) between the left atrial chambers are clearly visible.

Intraoperative transesophageal echocardiography confirmed the successful repair with no residual atrial septal defect or tricuspid regurgitation. Notably, pulmonary artery pressure improved from 60 mmHg preoperatively to 35 mmHg postoperatively, with left atrial pressure recorded at 17 mmHg.

Discussion

Adult cor triatriatum sinister is an exceptionally rare condition, often leading to delayed diagnosis due to its nonspecific clinical manifestations. Cases that are asymptomatic in early life due to adequate fenestration may present later in adulthood with symptoms such as dyspnoea and haemoptysis. Cor triatriatum sinister is frequently associated with other congenital anomalies including atrial septal defect, patent ductus arteriosus), total anomalous pulmonary venous return, ventricular septal defect, and persistent left superior vena cava.

Echocardiography is paramount for diagnosis, while surgical intervention is essential to prevent the progression to right ventricular failure and severe pulmonary hypertension. Management of such complex cases should be undertaken in specialised centres with expertise in congenital cardiac surgery.

Conclusion

This case highlights the importance of early diagnosis and surgical management in adult patients with cor triatriatum sinister to avert life-threatening complications. The combined surgical approach—comprising membrane resection, atrial septal defect repair, and tricuspid annuloplasty—resulted in significant haemodynamic improvement, with the patient experiencing an uncomplicated recovery and remaining asymptomatic at six-month follow-up.Reference Ishida, Yagami, Fujita and Mutsuga3, Reference Kumar, Singh, Mishra and Thingnam4

References

Rudienė, V, Hjortshøj, CMS, Glaveckaitė, S, et al. Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review. Heart 2019; 105: 1197–1202.Google ScholarPubMed
Kilkenny, K, Frishman, W. Cor triatriatum: a review. Cardiol Rev. 2023 Nov 15. doi: 10.1097/CRD.0000000000000626. Epub ahead of print. PMID: 37966218. Google ScholarPubMed
Ishida, S, Yagami, K, Fujita, T, Mutsuga, M. Cor triatriatum in adulthood with mitral valve regurgitation and atrial fibrillation. J Card Surg 2021; 36: 309–311.CrossRefGoogle ScholarPubMed
Kumar, V, Singh, RS, Mishra, AK, Thingnam, SKS. Surgical experience with cor triatriatum repair beyond infancy. J Card Surg 2019; 34: 1445–1451.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1. Transthoracic echocardiography demonstrating an abnormal septum (arrow) within the left atrium. Abbreviations: LA = left atrium; LV = left ventricle.

Figure 1

Figure 2. (a) Four pulmonary veins draining into the accessory chamber. (b) Communication between the accessory chamber and the main left atrium via a 5-mm fenestration (yellow arrow).

Figure 2

Figure 3. Intraoperative view from the surgeon’s perspective. The fibrous septum and the 5-mm fenestration (arrow) between the left atrial chambers are clearly visible.