Eisenmenger Syndrome with Dextrocardia, Ectopic Left Kidney and Right sided Infantile Scoliosis: A Rare Case Report

Abstract

Eisenmenger syndrome is defined as any untreated congenital cardiac defect with intracardiac communication resulting in pulmonary hypertension, flow reversal, and cyanosis.  The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease. Young male presented progressive dyspnoea, chest pain and anasarca and having cyanosis, dextrocardia, right scoliosis and ectopic kidney. A favourable prognosis for ES is achieved with early diagnosis and surgical intervention, whereas a poor prognosis is achieved with a late diagnosis and the onset of heart failure and pulmonary hypertension. Most patients die from heart failure, cardiac arrhythmia, and thromboembolic cerebrovascular disease. Patients with ventricular septal defects may benefit from taking medications like sildenafil and furosemide to improve their prognosis and quality of life. The longevity of these patients with functional limitations is still grim, despite therapy advancements. Further therapeutic interventions need to be made to reduce the symptoms.