Accelerated junctional rhythm (AJR) revealing light-chain cardiac amyloidosis: A case report with literature review.

Introduction and importance: Cardiac amyloidosis (CA) is a rare condition, characterized by fibrillary proteins infiltration in the extracellular space of the heart. Even though many types of cardiac amyloidosis exist, light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) remain the most described forms. The diagnosis of amyloidosis represents a real challenge for clinicians, requiring both invasive and non-invasive investigations. Conduction defects and atrial arrhythmias are well known complications of cardiac amyloidosis. However, only a few studies have reported junctional rhythm a primary presentation of light chain cardiac amyloidosis (AL). An early diagnosis and proper management are crucial to improve the prognosis of this disease. Case presentation: Here, we report a rare case of a 48 year-old patient, in acutely decompensated heart failure, presenting an accelerated junctional rhythm (AJR) as initial presentation of light-chain cardiac amyloidosis. The diagnosis was made based on clinical, biological, radiological and histological findings. This case shows diagnostic difficulties and management of this rare disease.

Complete heart block in a young adult with non-isolated congenitally corrected transposition of the great arteries: Case report.

Introduction and importance: Congenitally corrected transposition of the great arteries (ccTGA) or L-looped transposition of the great arteries (L-TGA) is a very rare and complex form of congenital heart disease. The majority of patients with ccTGA have at least one or more associated congenital heart disorders, essentially ventricular septal defects. Patients with ccTGA can remain asymptomatic for a long time and the diagnosis can sometimes be made late in life at the stage of complications. Case presentation: Here, we report a rare case of a 19-year-old patient, with no medical or surgical history, presenting a complete heart block as initial presentation of a ''non-isolated'' ccTGA. The diagnosis is made essentially by echocardiography.This case aims to show diagnostic difficulties of this rare congenital heart disease and be aware of the risk of its relative complications.