Surgically Treated Giant Right Atrium in Isolated Severe Tricuspid Regurgitation

Severe tricuspid regurgitation (TR) can lead to significant enlargement of the right atrium (RA) and poses unique clinical challenges. We report this case of a 17-year-old boy previously misdiagnosed with Ebstein anomaly who presented with dyspnea and palpitations. Initial examination revealed irregular heart rhythm, distended neck veins, and a significant murmur. Echocardiogram and computed tomography showed severe TR due to noncoaptation of the tricuspid valve, causing massive RA enlargement (1916 mL). The patient underwent successful surgical intervention, including tricuspid valve replacement and RA reduction. Postoperative complications included atrial fibrillation, which was medically managed. To our knowledge, this case had the largest RA to be reported and surgically treated in the literature. The case underscores the diagnostic challenges in differentiating severe TR from Ebstein anomaly and the progression from TR to significant RA enlargement. Accurate diagnosis and timely surgical management are critical in such cases to achieve favorable outcomes.