Heart Transplant for Heart Failure Secondary to Late Coronary Sinus Obliteration After Raghib Syndrome Repair

Raghib syndrome is a rare congenital anomaly involving a persistent left superior vena cava draining into the left atrium, absence of the coronary sinus, and an atrial septal defect; long-term outcomes after repair are unclear.

We report a 57-year-old man who developed heart failure with preserved ejection fraction (HFpEF) 4 decades after childhood repair, secondary to progressive coronary sinus obliteration from patch calcification. The resulting impairment of cardiac venous drainage likely contributed to chronic myocardial congestion and diastolic dysfunction. The patient was successfully treated with orthotopic heart transplantation.

Coronary sinus obstruction can elevate venous pressure, impair myocardial perfusion, and reduce diastolic compliance. Chronic obstruction is rare and under-recognized. This case demonstrates coronary venous outflow obstruction as a potential mechanism of heart failure with preserved ejection fraction.

Consider coronary venous pathology in unexplained heart failure with preserved ejection fraction, particularly after Raghib syndrome repair.