Sleeping Giants: Anomalous Coronary Anatomy Laying Dormant for Decades
Raheja S, Wang DD, and Basir M. SLEEPING GIANTS: ANOMALOUS CORONARY ANATOMY LAYING DORMANT FOR DECADES. J Am Coll Cardiol 2019; 73(9):2775.
J Am Coll Cardiol
Background: Congenital coronary anomalies have varied forms, presentations and malignant potential. Understanding the dynamics of flow is essential for management. Case: A 73 year old female with new angina was found to have cardiomyopathy and severely elevated pulmonary artery pressures. A coronary angiogram revealed severely dilated and tortuous vessels (Fig 1A-B). Contrast coursed through the left cusp ostium into an enlarged coronary system, then into collateral vessels before retrograde filling a right sided artery. Dye drained into the pulmonary trunk. No other coronary ostium was found in the aorta. Decision-making: Coronary anomalies result from malrotation of the spiral septum dividing the truncus arteriosus, malpositioning the coronary buds. CT scans and 3D reconstruction confirmed the anomalous RCA from the pulmonary artery (ARCAPA) (Fig 1C-G). Diastolic pressure differences initially allow left to right flow of oxygenated blood through collaterals to meet myocardial demand. If pulmonary vascular resistance or filling pressures rise (as in this case), lower inter-coronary gradients compromise flow and myocardial blood supply, resulting in angina, heart failure, or cardiac arrest. Accordingly, coronary re-implantation is recommended to establish an arterial origin, and was performed here. Conclusion: ARCAPA is a rare entity, and patients may be asymptomatic for decades. Pathophysiology develops when myocardial oxygen delivery is lost, with potential for severe complications. [Figure presented]
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