Cor Triatriatum Sinister Identified after New Onset Atrial Fibrillation in an Elderly Man

A 73-year-old man with new onset atrial fibrillation with rapid ventricular response underwent transthoracic echocardiography that revealed an echogenic linear structure along the left atrium, suggestive of cor triatriatum sinister (CTS). CTS was confirmed with transesophageal echocardiography which demonstrated a proximal accessory atrium receiving pulmonary venous flow separated from a distal true atrium by a fibromuscular membrane with a large fenestration allowing flow between the chambers. In CTS, the left atrium is divided into proximal and distal chambers by a fenestrated fibromuscular septum. This cardiac anomaly accounts for 0.1% of cases of congenital heart disease and rarely presents in adults. CTS is primarily diagnosed with echocardiography and is associated with left atrial enlargement and development of atrial fibrillation. Treatment options depend on size of the communication between proximal and distal chambers, the gradient across the membrane, and the position of pulmonary veins. In some instances, surgical resection of the membrane that divides the left atrium is warranted.

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Isolated Cor Triatriatum Sinister: A Case Report

Acta Chirurgica Latviensis ◽

10.2478/chilat-2020-0007 ◽

2020 ◽

Vol 18 (1) ◽

pp. 28-30

Author(s):

Zanda Grīnberga ◽

Pauls Sīlis ◽

Elīna Ligere ◽

Ingūna Lubaua ◽

Inta Bergmane ◽

...

Keyword(s):

Left Atrium ◽

Pulmonary Veins ◽

Surgical Excision ◽

Venous Drainage ◽

Treatment Method ◽

Cor Triatriatum ◽

Cardiac Anomaly ◽

Congenital Cardiac Anomaly ◽

Cor Triatriatum Sinister ◽

The Right

SummaryCor triatriatum sinister is a rare congenital cardiac anomaly that has been identified in 0.1% of children with congenital heart disease. It is defined as a fibromuscular membrane that divides the left atrium into two chambers: a superior (proximal) that in most cases receives drainage from the pulmonary veins and an inferior (distal) chamber that communicates with the mitral valve and the left atrium. Cor triatriatum sinister can be an isolated lesion (approximately 25% of cases), but in many cases it is associated with other congenital cardiovascular anomalies, the most common one being – atrial septal defect(3). Symptoms in patients with cor triatriatum sinister are related to obstruction of pulmonary venous drainage, pressure loading of the right side of the heart and congestive cardiac failure. Depending on the severity of the obstruction and presence of associated cardiac anomalies it can be diagnosed at any age. Diagnosis is usually achieved by echocardiography in early infancy. Elective treatment method is surgical excision of the membrane. Here we present a pediatric patient (4 months old) presenting in cardiogenic shock with a successful correction of isolated cor triatriatum sinister. To confirm diagnosis and success of surgical repair, transthoracic and transesophageal echocardiography were used.

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Isolated Cor-Triatriatum Sinister Presenting as New Onset Atrial-Fibrillation in a 53-Year-Old Female

CHEST Journal ◽

10.1378/chest.1388838 ◽

2012 ◽

Vol 142 (4) ◽

pp. 119A ◽

Cited By ~ 1

Author(s):

Priyank Shah ◽

Vipin Mittal ◽

Nishant Gupta ◽

Sharad Bajaj ◽

Fadi Alattar ◽

...

Keyword(s):

Atrial Fibrillation ◽

Cor Triatriatum ◽

Cor Triatriatum Sinister ◽

Onset Atrial Fibrillation ◽

New Onset

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New onset atrial fibrillation due to isolated cor triatriatum in a middle-aged woman

Acta Cardiologica ◽

10.1080/ac.70.2.3073519 ◽

2015 ◽

Vol 70 (2) ◽

pp. 247-247

Author(s):

Priyank Shah ◽

Vipin Mittal ◽

Fadi Alattar ◽

Donna Konlian ◽

Aderemi Soyombo ◽

...

Keyword(s):

Atrial Fibrillation ◽

Cor Triatriatum ◽

Middle Aged ◽

Aged Woman ◽

Middle Aged Woman ◽

Onset Atrial Fibrillation ◽

New Onset

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Pulmonary Vein: Embryology, Anatomy, Function and Disease

10.5772/intechopen.100051 ◽

2021 ◽

Author(s):

Chan I-Ping ◽

Hsueh Tung

Keyword(s):

Cardiovascular Diseases ◽

Left Atrium ◽

Developmental Process ◽

Case Reports ◽

Treatment Options ◽

Pulmonary Veins ◽

Fibrous Tissue ◽

Whole Body ◽

Cor Triatriatum Sinister ◽

Anomalous Pulmonary Venous Connection

Four pulmonary veins come from respective lung lobes drain oxygen-rich blood back to the left atrium. Failure of incorporation with the left atrium can lead to a condition, called Cor triatriatum sinister, that the left atrium is separated into two chambers by an abortive fibrous tissue. The venous system of lung and whole body communicate with each other in the earlier time and they will be disconnected in the following developmental process. Total or partial anomalous pulmonary venous connection refers to that there is/are some degree of the communication exists after birth, which can occur in different sites. In the veterinary field, retrospective studies and several case reports have been published to describe these rare congenital cardiovascular diseases in several species. More cases are need for better understanding their clinical manifestation, treatment options and outcomes.

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Divided left atrium with obstruction of pulmonary venous return in the setting of hypoplasia of the left heart

Cardiology in the Young ◽

10.1017/s104795110400513x ◽

2004 ◽

Vol 14 (5) ◽

pp. 553-556 ◽

Cited By ~ 5

Author(s):

Sara E. Monaco ◽

Welton M. Gersony ◽

Harshwardhan M. Thaker

Keyword(s):

Left Atrium ◽

Venous Return ◽

Pulmonary Veins ◽

Postmortem Examination ◽

Cor Triatriatum ◽

Pulmonary Venous Obstruction ◽

Left Heart ◽

Venous Obstruction ◽

Cor Triatriatum Sinister

We describe an infant with hypoplasia of the left heart diagnosed prenatally who, at birth, had signs of severe pulmonary venous obstruction. Echocardiography indicated normally connecting pulmonary veins, and showed a paradoxical right-to-left shunt across a patent oval foramen. Postmortem examination revealed that the obstruction was due to a divided left atrium, or cor triatriatum sinister, with an imperforate muscular diaphragm separating completely the two components of the divided atrium.

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Cardioembolic stroke in a young male with cor triatriatum sinister: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa096 ◽

2020 ◽

Vol 4 (3) ◽

pp. 1-6

Author(s):

Richard S Amara ◽

Rakhee Lalla ◽

Jean Jeudy ◽

Susie Nam Hong

Keyword(s):

Left Atrium ◽

Protein C ◽

Blood Stasis ◽

Young Male ◽

Cardioembolic Stroke ◽

Cor Triatriatum ◽

Cardiac Anomaly ◽

Protein C Deficiency ◽

Congenital Cardiac Anomaly ◽

Cor Triatriatum Sinister

Abstract Background Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly defined by a fibromuscular membrane which bisects the left atrium. Cor triatriatum sinister has been associated with cardioembolic stroke through mechanisms including stagnation of blood flow within the left atrium, an association with atrial fibrillation (AF), and/or an accompanying atrial septal defect (ASD) or patent foramen ovale. We describe a case highlighting the role that CTS may play in cardioembolic stroke, provide high-quality computed tomography angiography and two- and three-dimensional echocardiography of the CTS membrane, and outline management strategies for this uncommon clinical scenario. Case summary A 35-year-old man with no prior medical history presented with acute onset weakness and aphasia. He was found to have an embolic stroke with left M1 and A1 occlusions and received tissue plasminogen activator followed by mechanical thrombectomy with successful recanalization. A thorough stroke workup revealed CTS with an associated ASD as well as potential protein C deficiency. He was managed with indefinite anticoagulation with apixaban. Discussion This is the 13th reported case of CTS associated with stroke. In most previous cases evidence of blood stasis or frank thrombus was associated with the CTS membrane, and/or existing AF was noted. In this case, none of these were identified, particularly highlighting the surreptitious risk of CTS. In addition, the presence of potential protein C deficiency in this case compounded the risk for thromboembolism and factored into multidisciplinary management decisions.

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