Steroid Therapy in a Surgical Treated Case of Ventricular Septal Defect with Congenital Adrenocortical Insufficiency / 日本心臓血管外科学会雑誌

A very rare case of congenital adrenocortical insufficiency accompanied with ventricular septal defect, is presented. Surgical treatment together with glucocorticoid replacement therapy was performed. The patient, a 4-month-old girl with congenital adrenocortical insufficiency, had been treated with a long-term adrenocortical hormones replacement. Surgical treatment for ventricular septal defect was applied in order to reduce the risk of heart failure. The administration dose of glucocorticoid was determined according to the body surface area and chronologic change of serum cortisol. Following the surgical treatment, and with adequate glucocorticoid replacement, the patient showed a good clinical outcome. In conclusion, we showed a beneficial treatment protocol with adequate glucocorticoid replacement in open heart surgery for a case of congenital adrenocortical insufficiency.

A Case of Primary Right Atrium Angiosarcoma Involving Cardiac Tamponade / 日本心臓血管外科学会雑誌

Primary cardiac angiosarcoma is very rare and its prognosis was reported to be very poor (average survival period 7 months). A 46-year-old woman with angiosarcoma was admitted for recurrent symptoms of cardiac tamponade. Surgical excision of the tumor was performed 5 months after initial presentation and irradiation therapy was added. Thereafter, immunotherapy, and transcatheter arterial embolization were performed for liver metastasis. Despite this multidisciplinary therapy, she passed away 355 days after surgery. In our report, we described our multidisciplinary approach to this highly malignant tumor and the treatment strategy was discussed.