Abstract
BACKGROUND: Coronary artery fistulas (CAFs) are a rare congenital heart disease. Large fistulas can lead to coronary steal phenomena presenting with angina, heart failure, and in rare cases, cardiogenic shock. CASE SUMMARY: A 55-year-old woman acutely presented at our heart center with nonhemorrhagic pericardial tamponade and cardiogenic shock. A large CAF of an aneurysmatic right coronary artery to the coronary sinus was diagnosed. Pericardiocentesis was used for initial hemodynamic stabilization. On cardiopulmonary bypass, perforations connecting right coronary artery and coronary sinus were closed via suture. DISCUSSION: In cases of CAF, cardiogenic shock with pericardial tamponade is usually caused by rupture of an aneurysmatic vessel with successive hemopericardium. In this case, the pericardial tamponade was caused by acute right heart decompensation after long-term right ventricular overload. TAKE-HOME MESSAGES: In adult right heart failure, early comprehensive cardiac imaging is essential for diagnosis of rare underlying congenital diseases. Surgical shunt closure can drastically improve symptoms.