ABC | Volume 110, Nº1, January 2018

Clinicoradiological Session Atik & Dezn Preponderant left ventricular restrictive syndrome Arq Bras Cardiol. 2018; 110(1):95-96 Figure 1 – Chest X-ray in AP emphasizes normal cardiac area with a clear increase of the left atrium size in a double contour in the lower right arch and slightly congested pulmonary vascular weave in the upper fields. The echocardiographic images highlight the left atrial enlargement in longitudinal and 4-chamber projection. Angiocardiograms below show right ventricular hypertrophy (A), left atrial emptying delay (B) compatible with left ventricular restrictive syndrome and this cavity with smooth and normal-sized internal borders (C). 1. Gallego-Delgado M, Delgado JF, Brossa-Loidi V, Palomo J, Marzoa-Rivas R, Perez-Villa F, et al Idiopathic restrictive cardiomyopathy is primary a genetic disease. J Am Coll Cardiol.2016;67(25):3021-3. Doi:10.1016/j. jacc.2016.04.024. 2. HaasNA, LaserKT , BachS, KantzisM, HappelCM, FischerM. Decompressive atrioseptostomy (DAS) for the treatment of severe pulmonary hypertension secondarytorestrictivecardiomyopathy. IntJCardiol. 2016Jan15;203:845-7. Doi:10.1016/j.sjcard.2015.11.059. References pulmonary hypertension, allowing to postpose the indication for transplantation. 2 It is the least common cardiomyopathy of all types. The genetic spectrum points to mutations in sarcomeric genes in half of the cases. 1 Evolutionarily, sudden death occurs in 80%, heart failure in 15%, infective endocarditis in 5%, predominantly below 20 years of age. This is an open-access article distributed under the terms of the Creative Commons Attribution License 96