Eisenmenger Syndrome (ES) represents Pulmonary Arterial Hypertension (PAH) associated with Congenital Heart Defects (CHD). Although patients survive until their third or fourth decades of life, the symptoms include dyspnea, cyanosis, fatigue, dizziness, and syncope. In addition, cardiac arrhythmias, a late complication are causing sudden death in patients with ES. Treatment options have been limited; however, recent successes have been achieved with the use of therapies targeted against the pathophysiological pathways that underlie PAH. The dual endothelin receptor antagonist and prostacyclins demonstrated to improve hemodynamics of the patients. This is the case of a 16 year old young female with ventricular septal defect that was admitted with increasing shortness of breath and cyanosis with clubbing which are clinical features of Eisenmenger syndrome. She was medicated with Furosemide, Sildenafil which improved her functional status
