Surgical Outcomes of Malignant Primary Cardiac Tumor:A 20-Year Study at a Single Center

Background@#Malignant primary cardiac tumors are extremely rare, but have a poor prognosis. This study evaluated the surgical outcomes of patients with this disease. @*Methods@#Forty patients who underwent surgery for malignant primary cardiac tumors between January 1998 and December 2018 were enrolled. Participants were divided into 3 groups based on resection margins (R0, 14 patients; R1, 11 patients; and R2, 11 patients) and their surgical outcomes were compared. Heart transplantation was performed in 4 patients with unresectable tumors. @*Results@#Early mortality was reported in 2 cases (5%) due to postoperative bleeding and cerebral hemorrhage secondary to brain metastasis. The 1- and 2-year survival rates were 67.5% and 42.5%, respectively. The median survival time of the patients was 20.3 months (range, 9.2–37.6 months). The median survival time was 48.7, 20.3, and 4.8 months in patients with R0, R1, and R2 resections, respectively (p=0.023). Tumor recurrence occurred in 21 patients (61.7%), including 4 cases of local recurrence and 17 cases of distant metastasis. In patients who underwent heart transplantation, the median survival time was 29.5 months, with 3 cases of distant metastasis. @*Conclusion@#Although surgery for malignant primary cardiac tumors has a poor prognosis, complete resection of the tumor may improve surgical outcomes.

Heart Transplantation in Patients with Superior Vena Cava to Pulmonary Artery Anastomosis: A Single-Institution Experience

BACKGROUND: Heart transplantation (HTx) can be a life-saving procedure for patients in whom single ventricle palliation or one-and-a-half (1½) ventricle repair has failed. However, the presence of a previous bidirectional cavopulmonary shunt (BCS) necessitates extensive pulmonary artery angioplasty, which may lead to worse outcomes. We sought to assess the post-HTx outcomes in patients with a previous BCS, and to assess the technical feasibility of leaving the BCS in place during HTx. METHODS: From 1992 to 2017, 11 HTx were performed in patients failing from Fontan (n=7), BCS (n=3), or 1½ ventricle (n=1) physiology at Asan Medical Center. The median age at HTx was 12.0 years (range, 3–24 years). Three patients (27.3%) underwent HTx without taking down the previous BCS. RESULTS: No early mortality was observed. One patient died of acute rejection 3.5 years after HTx. The overall survival rate was 91% at 2 years. In the 3 patients without BCS take-down, the median anastomosis time was 65 minutes (range, 54–68 minutes), which was shorter than in the patients with BCS take-down (93 minutes; range, 62–128 minutes), while the postoperative central venous pressure (CVP) was comparable to the preoperative CVP. CONCLUSION: Transplantation can be successfully performed in patients with end-stage congenital heart disease after single ventricle palliation or 1½ ventricle repair. Leaving the BCS in place during HTx may simplify the operative procedure without causing significant adverse outcomes.

Erdheim-Chester Disease Presenting as an Anterior Mediastinal Tumor without Skeletal Involvement

Erdheim-Chester disease (ECD) is a form of non–Langerhans cell histiocytosis that most commonly involves the skeletal system. We report an unusual case of ECD presenting as an anterior mediastinal tumor without skeletal involvement. A 60-year-old man with no remarkable medical history was referred for evaluation of a mediastinal mass. The patient underwent surgical excision of the tumor via video-assisted thoracoscopic surgery. Histologic examination revealed marked proliferation of atypical histiocytes with sclerosis, and the results of immunohistochemical staining were suggestive of ECD.