Apical Hypoplasia of the Left Ventricle A Very Rare Congenital Heart Disease

Abstract

ABSTRACT
Background: Apical hypoplasia of the left ventricle it is characterized by a spherical truncated left ventricle, with some degree of systolic and/or diastolic dysfunction, an elongated right ventricle, which surrounds the distal left ventricle, adipose tissue can be found infiltrating the apex of the left ventricle (LV)

Case: A 6-year old male patient with a history of Zollinger-Ellison syndrome, righ renal agenesis and systolic murmur in tricuspid focus, the transthoracic echocardiogram showed situs solitus, hypoplasia of the apical portion of the LV, interventricular septum with basal and medial dyskinesia, with restrictive systolic and diastolic dysfunction, severe pulmonary hypertension, the right ventricle is dilated and elongated surrounding the LV forming the cardiac apex, already with systolic and diastolic dysfunction of the wall free of global deformation of 9.3%.

Conclusion: there is currently very little knowledge about what appears to be a rare congenital disease. Some cases reported in the literature have responded to pharmacotherapy, although many of the affected patients may require invasive procedures and even heart transplantation.