Eisenmenger Syndrome with Unilateral Renal Agenesis: A Rare Case Report

Abstract

The most advanced form of Pulmonary Atrial Hypertension (PAH), linked with Congenital Heart Abnormalities (CHD), is Eisenmenger Syndrome (ES). This report is about a 26-year-old female who presented with ES treated with Phosphodiesterase type 5 (PDE5) inhibitors, cardiac glycosides, Angiotensin-Converting Enzyme (ACE) inhibitors, loop diuretics, antipyretics, iron and vitamin supplements. Investigations revealed unilateral renal agenesis of left kidney, pulmonary hypertension, ES with large ventricular septal defect and bidirectional shunt. Loop diuretics and PDE5 inhibitors remained the mainstay of the treatment which induced hearing loss and the patient self reported hearing improvement after ceasing furosemide administration. Additional treatment strategy is to be used to improve the symptoms and quality of life of patients.

Keywords

• loop diuretics
• pulmonary atrial hypertension
• sildenafil
• ventricular septal defect