Incidentally diagnosed Cor Triatriatum Sinistrum in a patient presenting with pleuritic chest pain (QF_FR_098)

Friday, January 31, 2025

8:10 AM – 8:20 AM East Coast USA Time

Location: Blue Room PreFunction

Presenting Author(s)

LD

Lorena Doctor, MD

Advanced Cardiac Imaging Fellow
Rutgers Robert Wood Johnson Medical School

Primary Author(s)

LD

Lorena Doctor, MD

Advanced Cardiac Imaging Fellow
Rutgers Robert Wood Johnson Medical School

Co-Author(s)

AM

Aliaa Mousa, MD

Advanced cardiovascular imaging
Rutgers/ Robert Wood Johnson University Hospital/ New Brunswick NJ
SB

Sabahat Bokhari, MD

Professor of Cardiology/Director of Advanced Cardiac Imaging
Rutgers Robert Wood Johnson Medical School
AM

Anthony Mendoza, MD

Advanced cardiovascular imaging attending
Rutgers/Robert wood johnson University Hospital/ New brunswick NJ
KM

Kameswari Magneti, MD

Section Chief, Director Proessor of Medicine Non-Invasive Cardiology Echocardiography Lab
Rutgers Robert Wood Johnson Medical School
PS

Partho Sangupta, MD

Professor of Cardiology/Chief of Cardiology
Rutgers Robert Wood Johnson Medical School
YH

Yasmin Hamirani, MD

Associate Professor of Medicine/Director of Structural Imaging
Rutgers Robert Wood Johnson Medical School

Description of Clinical Presentation: Description of Clinical Presentation: Clinical Presentation: 50 yo male with h/o HTN & hyperlipidemia presented c/o intermittent episodes of sharp chest pain with diaphoresis. He denied any precipitating events or other associated symptoms. Physical exam: Normal.

Diagnostic Techniques and Their Most Important Findings:
Diagnostic techniques and Most Important Findings:

Echo: unremarkable

Exercise Sestamibi: Mild CAD

CTA Heart and Coronaries revealed mild coronary artery disease. A branching linear filling defect dividing the left atrium was noted and was suspected to possibly represent Cor Triatriatum.

MRI w/wo contrast performed to evaluate myocardial morphology and function. MRI performed before and after administration of gadolinium. Bright-blood cine imaging acquired for the assessment of global and regional left ventricular function. Native T1 mapping performed and Delayed enhancement images acquired to assess myocardia viability. This revealed the p</span>resence of a membrane (Cor triatriatum sinistrum) dividing the left atrial cavity with left upper pulmonary vein entering the proximal (accessory) left atrium on the left side of the membrane while the flow from both the right sided pulmonary veins and the left lower pulmonary vein enters the right side of the membrane into the true left atrium. The membrane appeared to be partial and visualized on the inter-atrial septum side. No evidence of ASD or VSD noted with normal biventricular size and function.

Learning Points from this

Case:
Cor Triatriatum is a very rare Congenital heart disease (0.1-0.4 % of all infants with CHD) in which the left atrium (Cor triatriatum sinistrum, most common) or right atrium (Cor triatriatum dexter/dextrum) is subdivided by a thin fibromuscular membrane resulting in 3 atrial chambers. In classic Cor triatriatum (cor tritriatum sinistrum), there is abnormal incorporation of pulmonary venous structures in the left atrium with a fibromuscular membranous subdivision throughout the atrial chamber. Clinical presentation depends on the degree of stenosis in the fibromuscular membrane. the integrity of the IAS, and the presence of associated CV malformations. Typical presentation includes dyspnea, heart failure, and in children failure to thrive or unexplained PHTN. An echocardiogram typically demonstrates a linear density subdividing the atrium into proximal (inferior) and distal (superior) chambers. The membrane extends from the posteroinferior to the anterosuperior wall. Proximal chamber typically receives the pulmonary venous drainage. Distal chamber usually communicates with the mitral valve and atrial appendage.
MRI allowed more direct visualization of membrane & chambers for the accurate & definitive diagnosis in this adult with rare CHD presenting with recurrent chest pain.