Cor triatriatum sinistrum: presentation of syncope and atrial tachycardia

Abstract

We present a rather unusual cause for syncope associated with atrial tachycardia. A man aged 39 years presented with an episode of syncope and narrow complex tachycardia. Further investigations, including transoesophageal echocardiography, identified cor triatriatum sinistrum (CTS), a rare congenital abnormality characterised by the atrium being divided by a fibrous membrane. Although it is rare, there has been an increase in diagnosis due to developments in diagnostic imaging techniques. Symptoms are related to the size of fenestrations within the fibrous membrane. Presenting symptoms can mimic those seen in mitral stenosis. It is a condition that can occur in isolation, but it can also be associated with other cardiac abnormalities such as an atrial septal defect (ASD) (as in this case). Surgery is the definitive treatment (this man had surgical repair of CTS and closure of ASD) and should be considered at any age if there are any associated symptoms or complications.

A rare case of pacemaker induced tachycardia in an elderly woman with cor triatriatum sinistrum

“…Currently, there are studies showing that anatomic and hemodynamic alterations consecutive to cor triatriatum sinistrum predispose to atrial arrhythmia, such as atrial fibrillation [ 4 , 6 – 8 ] or atrial tachycardia [ 4 , 9 , 10 ], but there is no data in the literature describing the case of a PIT in a patient with cor triatriatum sinistrum.…”

A Systematic Review of a Long-forgotten Cause of Atrial Fibrillation and Stroke: Cor Triatriatum

“…Cor triatriatum has also been classified based on size and fenestrations in the septa, with the first category having no opening, the second having one or more openings, and the third having a wide opening [3]. Embryologic malseptation, or entrapment, is thought to result in cor triatriatum [3,7].…”

“…Cor triatriatum has also been classified based on size and fenestrations in the septa, with the first category having no opening, the second having one or more openings, and the third having a wide opening [3]. Embryologic malseptation, or entrapment, is thought to result in cor triatriatum [3,7]. The clinical presentation of cor triatriatum depends on the size and number of fenestrations, with a large single fenestration being asymptomatic whereas narrow openings present earlier as respiratory distress [8].…”

“…1 2 3 1 4 The incidence of cor triatriatum ranges from 0.1% to 0.4% in patients with clinically diagnosed cardiomyopathy and congenital heart disease [2]. The male-to-female ratio is 1.5:1, with no genetic association [3]. This abnormality is labeled cor triatriatum sinister (CTS) when the left atrium is involved and cor triatriatum dexter (CTD) when the right atrium is involved.…”

“…This abnormality is labeled cor triatriatum sinister (CTS) when the left atrium is involved and cor triatriatum dexter (CTD) when the right atrium is involved. In CTS, the posterosuperior chamber receives blood from four pulmonary veins, whereas the anteroinferior chamber communicates with the left ventricle through the mitral valve [3,4]. CTD, however, is caused by the persistence of the right valve of the right horn of the sinus venosus [5].…”