Cor-triatriatum in an adult - a rare finding
Location
Auditorium Pond Side
Start Date
26-2-2014 10:30 AM
Abstract
Background: Cor-triatriatum is a rare congenital cardiac anomaly with incidence ranging from 0.1-0.4%. In this condition, a fibromuscular membrane divides the left atrium into two chambers: superior and inferior. This fibromuscular membrane may be obstructive (75%) or non-obstructive. The superior chamber may variously receive oulmonary venous drainage. Symptoms depend on the degree of obstruction and associated anomalies. Occasionally, it may be diagnosed incidentally in adults but this is exceedingly rare.
Case History:We present the case of a 55 year old hypertensive lady who presented with left sided weakness. She was diagnosed with right middle cerebral artery stroke. Her electrocardiogram revealed left atrial enlargement and left ventricular hypertrophy. Echocardiography was performed as part of stroke and hypertension assessment. Transthoracic echocardiography revealed a membrane which was dividing the left atrium into two chambers, without evidence of significant obstruction.
Two (right sided) pulmonary veins were visualized, the remaining two could not be identified. In addition, aneurysmal interatrial septum with a small atrial septal defect with left to tight shunt was noted, which was confirmed by agitated saline contrast study. These findings were highly suggestive of cor-triatriatum.
A trans-esophageal echocardiogram (TEE) was also performed to further delineate membrane characteristics and pulmonary venous drainage. TEE additionally demonstrated that two right sided and one left sided pulmonary veins were opening into the upper chamber while one left sided pulmonary vein was probably opening into the lower chamber. The communication between the superior and inferior left atrial chambers was not visualized but it was likely non-obstructive in view of the absence of symptoms, turbulent flow, and pulmonary hypertension. The patient was advised anticoagulation in view of stroke in the presence of aneurysmal inter-atrial septum with a septal defect. However, the presence of cor-triatriatum in this patient was considered to be non-contributory. This patient was referred for cardiac surgery consultation regarding ASD closure and resection of the left atrial membrane. However, the patient and family were unwilling for any intervention and were discharged and subsequently lost to follow up.
Conclusion: Cor-triatriatum is an unusual congenital cardiac malformation. Incidental diagnosis in an adult is an exceedingly rare presentation.
Cor-triatriatum in an adult - a rare finding
Auditorium Pond Side
Background: Cor-triatriatum is a rare congenital cardiac anomaly with incidence ranging from 0.1-0.4%. In this condition, a fibromuscular membrane divides the left atrium into two chambers: superior and inferior. This fibromuscular membrane may be obstructive (75%) or non-obstructive. The superior chamber may variously receive oulmonary venous drainage. Symptoms depend on the degree of obstruction and associated anomalies. Occasionally, it may be diagnosed incidentally in adults but this is exceedingly rare.
Case History:We present the case of a 55 year old hypertensive lady who presented with left sided weakness. She was diagnosed with right middle cerebral artery stroke. Her electrocardiogram revealed left atrial enlargement and left ventricular hypertrophy. Echocardiography was performed as part of stroke and hypertension assessment. Transthoracic echocardiography revealed a membrane which was dividing the left atrium into two chambers, without evidence of significant obstruction.
Two (right sided) pulmonary veins were visualized, the remaining two could not be identified. In addition, aneurysmal interatrial septum with a small atrial septal defect with left to tight shunt was noted, which was confirmed by agitated saline contrast study. These findings were highly suggestive of cor-triatriatum.
A trans-esophageal echocardiogram (TEE) was also performed to further delineate membrane characteristics and pulmonary venous drainage. TEE additionally demonstrated that two right sided and one left sided pulmonary veins were opening into the upper chamber while one left sided pulmonary vein was probably opening into the lower chamber. The communication between the superior and inferior left atrial chambers was not visualized but it was likely non-obstructive in view of the absence of symptoms, turbulent flow, and pulmonary hypertension. The patient was advised anticoagulation in view of stroke in the presence of aneurysmal inter-atrial septum with a septal defect. However, the presence of cor-triatriatum in this patient was considered to be non-contributory. This patient was referred for cardiac surgery consultation regarding ASD closure and resection of the left atrial membrane. However, the patient and family were unwilling for any intervention and were discharged and subsequently lost to follow up.
Conclusion: Cor-triatriatum is an unusual congenital cardiac malformation. Incidental diagnosis in an adult is an exceedingly rare presentation.