e22008 Background: Clinical presentation of pediatric primary cardiac tumors is exceptionally rare throughout the world. The purpose of our study is to evaluate clinical intervention by following up on children with primary cardiac tumors. Methods: Our study retrospectively enrolled 97 children diagnosed with primary cardiac tumors from Jan 2005 to Dec 2020 in a single center, Children’s Hospital of Nanjing Medical University by confirming with clinicoradiological and non-invasive diagnostic procedures. Primary outcome was to measure clinical trends and interventions while secondary was to analyze survival of 26 (26.80%) operative and 71 (73.19%) non-operative patients. Surgical intervention patients were followed for 6 Mo in the first postoperative year, then every year. Meanwhile, non-serious, asymptomatic, and no hemodynamic changes patients were treated non-operatively and followed up every year regularly. Results: In primary outcome, median age of children was 4.72±4.10 years (1d~16y), and median follow-up was 5.60±4.97 years (1m~15y). Most enrolled children were younger than 1 year old at the time of diagnosis. Primary cardiac tumors patients consisting of 60 rhabdomyoma (61.85%), 11 myxomas (11.34%), 7 fibromas (7.21%), 3 lymphangiomas (3.09%), 2 lipomas (2.06%), 9 unclassified tumors (9.27%) and 5 malignant tumors (5.15%) were retrospectively analyzed. Major 75 patients (77.31%) were diagnosed during various treatments of clinical symptoms, whereas 18 (18.55%) were diagnosed with routine investigation and 14 (14.43%) were diagnosed by prenatal fetal echocardiography. Herein, 26 children with critical symptoms and serious hemodynamic alteration had undergone surgical intervention in our hospital. Among them, 6 (23.07%) patients had postoperative complications; 2 (7.7%) patients were unable to survive due to post-operative arrhythmia and low cardiac output syndrome; While 2 (7.7%) patients with myxomas had a reoccurrence, and 2 (7.7%) patients had been diagnosed with another rhabdomyoma after intervention, others recovered well. Meanwhile, 71 children had non-surgical management, where 8 (11.26%) were lost to follow-up; 3 (4.22%) patients with malignant tumors were unable to survive after discharge due to unknown causes, and other patients had no serious symptoms or growth of tumors during follow-up. In secondary outcome, survival between operative and non-operative patients had no significant difference ( P > 0.05). Conclusions: Clinical intervention of pediatric primary cardiac tumors may be performed one-on-one by accounting presentations, symptoms, and emergence even though majority of primary cardiac tumors in children are benign in nature, and postoperative reoccurrence is feasible, particularly for rhabdomyoma. Meanwhile, the primary goal of surgical intervention is to reinstate normal hemodynamics, and preserve significant structures and cardiac tissues.