Coronary sinus aneurysm associated with multiple venous anomalies
© The Author(s). 2017
Received: 3 December 2016
Accepted: 1 April 2017
Published: 5 April 2017
Congenital anomalies of the venous system are rare, involve the inferior vena cava (IVC), a persistent left superior vena cava (PLSVC), and the left hepatic vein (LHV), and can make cardiac diagnostic and therapeutic procedures difficult.
We present a 67-year-old woman without heterotaxy syndrome associated with interruption of the left IVC that continued with the hemiazygos vein system, a PLSVC, and an anomalous LHV draining the into coronary sinus (CS). The venous anomalies caused a CS aneurysm. The anomalies were demonstrated by echocardiography and the diagnosis was established by contrast-enhanced computed tomography. Three days later, a coronary artery bypass graft was performed, which confirmed the diagnosis. Half a month after surgery, the pain had been relieved and the patient was discharged from the hospital.
Echocardiography is a useful modality to diagnose and assess anomalies of the CS, including CS aneurysms. Congenital anomalies of the venous system in this case were all due to embryonic development abnormalities. Contrast-enhanced computed tomography provides a more comprehensive view of the entire course of abnormal veins.
KeywordsInterruption Left inferior vena cava Persistent left superior vena cava Hepatic vein Hemiazygos vein Coronary sinus Aneurysm
Congenital anomalies of the inferior vena cava (IVC) are rare, with a prevalence of 1%, and include interruption of the IVC, left IVC, and double IVC. Interruption of the IVC is a well-recognized, but uncommon anatomic anomaly . The most common form of this anomaly is a right IVC that continues with the azygos vein via the right superior vena cava (R-SVC) into the right atrium (RA). A left IVC is also rare. When a left IVC is interrupted, the condition is more complicated because there are numerous routes for the return of blood . In addition, interruption of the IVC is often associated with heterotaxy syndrome. If a patient does not have other features of the heterotaxy syndrome, it is easy for the examiner to miss the diagnosis. Herein, we present a patient without heterotaxy syndrome associated with interruption of the left IVC that continued with the hemiazygos vein system, a persistent left superior vena cava (PLSVC), and an anomalous left hepatic vein (LHV) connected with the coronary sinus (CS). Together, the venous anomalies caused a coronary sinus aneurysm (CSA).
Additional file 1: Movie 1. Reconstructive 3D computed tomography demonstrating association with interruption of the left inferior vena cava that continues with the hemiazygos vein system, a persistent left superior vena cava, and an anomalous left hepatic vein draining into the coronary sinus. (AVI 12,902.4 kb)
Various diagnostic and therapeutic procedures involving the right side of the heart, such as electrophysiologic studies, right heart catheterization, cardiopulmonary bypass surgery, IVC filter placement, and temporary pacing, have increased the need for ready access to the IVC via the transfemoral route. Anatomic anomalies of the IVC can make these procedures difficult.
The interruption of the IVC has a prevalence of 0.6%–2.0% in patients with congenital heart disease and 0.3% in healthy people . Embryologically, the normal IVC consists of five segments (hepatic, suprarenal, renal, infrarenal [subrenal], and iliac), which are mainly derived from the right vitelline vein (hepatocardiac canal), the right subcardinal vein, the subcardial and supracardial anastomosis, the supracardial vein, and the persistent posterior cardinal veins. Suprarenal interruption of the IVC in the current case was due to a failed connection between the right vitelline and right subcardinal veins. The blood circulating in the caudal segments of the IVC reaches the azygos/hemiazygos system by way of a persistent right/left supracardinal vein. The interruption of the IVC can be associated with cardiac abnormalities, heterotaxy syndrome (polysplenia and asplenia), deep vein thrombosis, and renal vein anomalies, and has also been reported as an asymptomatic incidental finding. Of patients associated with interruption of the IVC without other features of the heterotaxy syndrome, 62.7% do not have any cardiac malformations, which easily lead to misdiagnosis of this anomaly during echocardiography .
Summary of literature involving with anomalous LHV connection with CS
First modality for diagnosis
Associated cardiovascular anomalies
M, 3 m
van der Horst RL
PLSVC, ASD, PS
PLSVC, atrial fibrillation
PDA, ASD, ductus venosus to the CS
PLSVC, PS, bifurcation of IVC
ASD, VSD, PDA, pulmonary mass
PLSVC, subaortic valve stenosis, absence of R-SVC
Three-vessel coronary artery disease
In patients with interruption of the IVC, the hepatic veins usually drain directly into the RA. Sometimes, the hepatic veins may connect with a residual IVC that eventually drains into the RA, pulmonary vein, or left atrium [9–11]. Our case is the first report involving multiple systemic vein anomalies.
The above-mentioned multiple venous anomalies involve the vast majority of venous blood draining into the CS. Thus, intimal hyperplasia and loss of smooth muscle and elastic tissue with replacement by connective tissue with age may aggravate the formation of CSA . CSA should be diagnosed as early as possible because the CSA in children may compress the mitral annulus and limit atrioventricular flow into the left heart, eventually causing left ventricle, aortic hypoplasia, and obstructive lesions in the left ventricle .
This is the first report involving these three co-existing systemic vein anomalies. Dilated CS by echocardiography is an important diagnostic clue for systemic anomalous venous pathways. Contrast-enhanced computed tomography provides a more comprehensive view of the entire course of abnormal veins. These congenital anomalies can be explained by the theory of embryonic development abnormalities.
Coronary sinus (CS)
Coronary sinus aneurysm
Inferior vena cava
Left hepatic vein
Persistent left superior vena cava
Right superior vena cava
Availability of data and material
All data is available in the manuscript.
GS drafted the manuscript and acquired the images. MD acquired the CT images. WR acquired the echocardiography images and revised the manuscript. KZ revised the manuscript. LS drafted the manuscript. All authors read and approved the final manuscript. All authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and interpretation.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent for the publication was obtained from the patient of this case report and any accompanying images and movies.
Ethics approval and consent to participate
The publication of this case report was in accordance with the Declaration of Helsinki and approved by the ethics committee of Shengjing Hospital of China Medical University.
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