Anatomical Variants of Venous Circulation in Congenital Heart Disease

Congenital heart disease often presents with a spectrum of systemic and cardiac venous anomalies that originate from embryological deviations in the cardinal vein system. The most common finding is a persistent left superior vena cava (PLSVC), reflecting failure of normal regression of the left anterior cardinal vein, which may co-exist with absence or hypoplasia of the right superior vena cava. Other recognised variants include duplication of the superior vena cava, anomalous courses of the left brachiocephalic vein beneath (subaortic) or behind (retroesophageal) the great vessels, coronary sinus ostial atresia, and diverticula or aneurysms of the coronary sinus. These anomalies affect central venous return pathways, can alter haemodynamics, and carry implications for catheterisation, pacemaker or cardiac device implantation and surgical repair. Modern cross-sectional imaging—echocardiography, computed tomography and magnetic resonance—has enhanced the detection and characterisation of such variations, informing clinical decision-making and guiding interventions. Understanding the prevalence, embryological basis and clinical impact of venous anomalies is essential for optimising outcomes in patients with complex congenital heart disease across diverse global settings.

Research from Nature Portfolio

Recent foundational work using fetal cardiac MRI has demonstrated the feasibility and accuracy of prenatal identification of subaortic and retroesophageal anomalous courses of the left brachiocephalic vein in congenital heart disease. The study retrospectively reviewed over 7 000 scans, correctly diagnosing eight cases of anomalous subaortic left brachiocephalic vein and two retroesophageal variants, with confirmation by postnatal imaging. These findings highlight the value of high-resolution MRI as a complementary tool to echocardiography for detailed venous anatomy mapping prior to postnatal management.

Anatomical Variants of Venous Circulation in Congenital Heart Disease publication trend

The graph below shows the total number of articles in anatomical variants of venous circulation in congenital heart disease across all publications each year (not limited to Nature Index journals).

Technical terms

Persistent left superior vena cava (PLSVC): failure of the left anterior cardinal vein to regress, resulting in a left-sided superior vena cava that most often drains into the coronary sinus.

Left brachiocephalic vein (LBCV): the venous channel formed by convergence of the left subclavian and internal jugular veins, which may follow abnormal mediastinal pathways.

Coronary sinus (CS): the principal venous conduit collecting deoxygenated blood from the myocardium and directing it into the right atrium.

Subaortic course: an anomalous pathway of a vessel passing beneath the aortic arch instead of its usual anterior route.

Retroesophageal course: an anomalous pathway of a vessel passing posterior to the oesophagus on its way through the mediastinum.

References

1. The Challenges of Cardiac Resynchronization Therapy in Patients with Dilated Cardiomyopathy and Persistent Left Superior Vena Cava: A Case Report and Concise Literature Review. Biomedicines (2023).
2. Malposition of Central Venous Catheter into Coronary Sinus throughout the Persistent Left Superior Vena Cava and Other Complications Related to Catheterization. Diagnostics (2024).
3. Persistent left superior vena cava: clinical importance and differential diagnoses. Insights into Imaging (2020).
4. Computed Tomography and Magnetic Resonance Imaging of the Coronary Sinus: Anatomic Variants and Congenital Anomalies. Insights into Imaging (2014).
5. MR imaging of subaortic and retroesophageal anomalous courses of the left brachiocephalic vein in the fetus. Scientific Reports (2018).
6. Single left superior vena cava: antenatal diagnosis, associated anomalies and outcomes. Ultrasound in Obstetrics and Gynecology (2022).