Association of Fallot Tetralogy with Carney's complex

The Carney complex is an inherited, autosomal disease of multicentric tumors in many organs. Some components of Carney's complex are cardiac myxoma, spotty pigmentation, and endocrine overactivity. Primary pigmented nodular adrenocortical dysplasia (PPNAD) is an exceedingly rare cause of Cushing's syndrome in infants, children, and young adults. PPNAD occurs sporadically or as part of a familial syndrome called Carney's complex. Up to our knowledge, the association of Fallot Tetralogy with Carney's complex has not been previously reported. We presented, a 20-year-old woman, who had been operated for Fallot Tetralogy at the age of 3 years, had Carney's complex, i.e. left atrial myxoma, two facial spotty pigmented areas, and PPNAD.