Journal of Rare Cardiovascular Diseases

Eisenmenger syndrome represents the extreme manifestation of pulmonary arterial hypertension associated with congenital heart defects (CHD). Adults with CHD represent an increasing population which will require management in the long term. We present a case of a 38 year old female who presented with progressive dyspnea along with cyanosis at rest. Echocardiography showed a dilated right ventricle and an ostium secundum ASD with bidirectional shunting. Catheterization revealed severe pulmonary hypertension and evidence of partial anomalous pulmonary venous return. Warden procedure for PAPVR repair was performed along with a fenestrated pericardial ASD patch to close the shunt. A Maze Cox III procedure was also performed to treat the atrial fibrillation. Both procedures were performed successfully with significant improvement in patient’s clinical status. This article provides details regarding etiology, pathogenesis, clinical features, and diagnostic evaluation of Eisenmenger syndrome. It covers recent guidelines for screening and therapy as well as information regarding innovations in social and healthcare. JRCD 2014; 1 (6): 33–35

Eisenmenger; atrial septal defect; congenital heart disease; pulmonary hypertension

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