A hitherto unreported combination of pulmonary stenosis, single coronary artery anomaly, and coronary sinus to left atrial communication.

BACKGROUND

We report a hitherto unreported combination of pulmonary stenosis, single coronary artery anomaly and coronary sinus to left atrial communication. Our case highlights the important value of coronary computed tomographic angiography and transthoracic echocardiography for the diagnosis of such anomalies and guidance for proper management.

METHODS AND RESULTS

A 64-year-old male presented chest tightness and shortness of breath for 2 days. Transthoracic echocardiography revealed a thickened pulmonary valve leaflet and subvalvular outflow tract stenosis, colour flow Doppler showed a significant accelerated blood flow in the pulmonary artery cavity originating from the subvalvular outflow tract, continuous wave Doppler revealed the transpulmonary valvular pressure gradient of 63mmHg. Computed tomographic angiography image reveals thickened pulmonary valve leaflets and subvalvular outflow tract stenosis, single coronary artery anomaly and levoatriocardinal vein. The patient underwent percutaneous pulmonary valve balloon dilatation, the post-procedural course was uneventful.

DISCUSSION

Pulmonary stenosis can occur as part of more congenital cardiac malformations or as rare primary isolated pulmonary stenosis, which includes the valvular, sub-valvular, or supra-valvular pulmonary stenosis. Single coronary artery anomalies are very rare, anomalous right coronary artery originates from proximal to mid-left anterior descending coronary artery is one such single coronary artery anomaly, in most cases, it is asymptomatic, diagnosed incidentally, and a benign entity has a better prognosis except if the right coronary artery is passing between the aorta and pulmonary artery. This course of the right coronary artery anomaly is malignant. Coronary sinus to left atrial communication includes a direct or indirect communication. The direct communication is described as a partial or complete absence of the roof between the coronary sinus and left atrium, as it is well known as the unroofed coronary sinus syndrome. The indirect communication is an anomalous bridging vein communicating the coronary sinus to the left atrium, which can be distinguished from classical unroofed coronary sinus syndrome. The venous collateral channel communication between the coronary sinus to the left atrium by a bridging vein is also categorized as a variant type of unroofed coronary sinus syndrome. Understanding coronary venous variations has significant clinical implications particularly in the realm of electrophysiology. The anatomical variations can have important consequences for procedures such as biventricular pacing and trans-coronary vein ablations.

CONCLUSION

Pulmonary stenosis combined with single coronary artery anomaly and bridging vein communication between the coronary sinus and the left atrium is an extremely rare. Coronary computed tomographic angiography and transthoracic echocardiographyplay an important role the diagnosis of such anomalies and guidance for clinical Treatment.