MANAGEMENT OF EISENMENGER SYNDROME IN A LATE PRESENTATION OF TRANSPOSITION OF THE GREAT ARTERIES ASSOCIATED WITH INCIDENTAL ADRENAL TUMOR. CASE REPORT
We present the case of a 25-year-old patient with transposition of the great arteries (TGA), ventricular septal defect (VSD), without surgical correction, severe pulmonary hypertension and Eisenmenger syndrome, under vasodilatation therapy with Bosentan and Sildenafil, which presented for hemoptysis, shortness of breath at mild effort, myalgia, headaches and vertigo. Blood test showed important erythrocytosis and thrombocytopenia. CT examination revealed the presence of an adrenal incidentaloma (AI), which was proved to be nonfunctional after hormonal testing. Management of the patient included phlebotomy for the treatment of the hyper viscosity syndrome and recommending the addition of a prostacyclin analogue to the vasodilators scheme. The dimensions of the AI raised the question regarding the need of surgical excision, due to the risk of being an adrenal gland carcinoma. Due to the high risk of general anesthesia in Eisenmenger patients, a multidisciplinary team (endocrinologist, cardiologist, surgeon, and anesthesiologist) decided on a watch and wait strategy regarding the tumor, with regular monitoring.
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