日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

We report a rare case of a patient with pheochromocytoma (PCC) and Eisenmenger syndrome (ES). The patient was a female born to healthy parents. Although double outlet right ventricle (DORV) was diagnosed in the neonatal period, she displayed ES pathology from infancy. At the age of 30 years, PCC complications were diagnosed. An operation was contraindicated because of high perioperative risk. She died at the age of 32 years owing to advanced heart failure and renal failure. Her pathologic anatomy revealed DORV, severe pulmonary vascular obstruction, PCC in the left adrenal gland, multiple paraganglioma (PGL) in the bladder, and cyanotic nephropathy. In recent years, hypoxia has been reported as a risk factor for PCC/PGL. In our case, PCC/PGL was associated with DORV and ES. The diagnosis of PCC/PGL in congenital heart disease patients tends to be delayed as the symptoms of PCC/PGL overlap with those of heart disease. The harmful effects of hypertension and/or tachycardia on cardiac hemodynamics due to PCC were difficult to manage as she was contraindicated for surgery. PCC/PGL should be considered as a complication of congenital heart disease.