P243 A rare association of isolated persistent left superior vena cava(PLSVC) with anomalous right coronary artery (RCA) in a symptomatic patient. Role of saline contrast echo (SCE) with multimodal imaging
Authors: Fady G , Komaranchath A M , Al Bakshy F B , Gibreel M G 
Published: January 2020

Funding Acknowledgements

Nil

Introduction

PLSVC with absent right superior vena cava (RSVC) is an extremely rare venous anomaly. Most of the patients with this anomaly have other associated cardiac anomalies.

However, presence of an anomalous RCA has not been one of these anomalies.

Case report

A 36-year-old Asian healthy female with no significant medical history presented with over 3 years symptoms of chest heaviness and shortness of breath on walking two blocks.

Examination was unremarkable. 2D echocardiogram revealed a 36mm dilated coronary sinus (CS), grade II tricuspid regurgitation (TR) with estimated right ventricular systolic pressure (RVSP) of 40 mmhg. Normal right and left side of the heart.

SCE using 50:50 agitated saline and Gelofusine injected intravenously through the left arm showed opacification of the dilated CS first before opacifying the right atrium (RA). Right arm injection depicted the same pattern of contrast opacification which denotes presence of PLSVC with congenital absence of RSVC.

No detected intracardiac shunts by SCE. No partial anomalous pulmonary venous connection (PAPVC).

However, findings did not justify patient’s symptoms.

Seven days loop recorder ruled out any arrhythmias that might be associated with isolated PLSVC.

An exercise stress ECG was equivocal with chest discomfort but no ECG changes.

We opted for CT coronary angiography (CTCA) to rule out obstructive coronary artery disease (CAD).

CTCA confirmed our diagnosis of isolated PLSVC with absence of RSVC with huge CS.

In addition, there was an anomalous origin of RCA from sinotubular junction above the commissure between left and right aortic CS and not from LM CS proper, taking a malignant interarterial course of a slit origin RCA with first part of it showing intramural course within the aortic wall.

In view of symptoms we as a heart team explained the present risk of sudden cardiac death and possible indication for corrective surgery.

A month later patient again presented with chest discomfort. We decided to do Coronary angiography to delineate actual size and dominance of RCA.

RCA was a non-dominant small 1.75 mm vessel. There was a left anterior descending artery myocardial bridge.

We started a beta blocker (BB) for the patient after which her symptoms improved. Patient followed up for two years later with no symptoms.

We attributed her chest pain to the myocardial bridge since she improved on BB. We found there was no solid role for surgery in view of a very small non dominant RCA with no further chest pain.

Conclusions

Isolated PLSVC is a very rare condition. It is usually asymptomatic. But since our patient had symptoms, a search for another diagnosis was convenient.

A dilated CS should always alert us to search for PLSVC.

SCE is a simple, inexpensive and reproducible diagnostic tool that might help in solving a challenging diagnosis.

Multimodal imaging has a leading role in both proper diagnosis and in management of this condition

Abstract P243 Figure. Anomls. RCA - PLSVC to CS -Contrast echo