Clinical Pediatrics: Open Access
Open Access

Abstract

Insights into Pathophysiology of Sickle-cell Disease Cardiomyopathy by 3D Echocardiography

Francesca Coppi, Francesca Tampieri, Giovanni Palazzi, Matteo Boschini, Gianluca Pagnoni, Francesca Ferrara, Antonio Manenti*, Anna Vittoria Mattioli, Lorenzo Iughetti and Giuseppe Boriani

In sickle cell disease cardiomyopathy, 3D transthoracic Echocardiography (3D Echo) is an essential diagnostic tool for accurate diagnosis and further understanding of its pathophysiology. To this end, we performed a retrospective observational study in a cohort of 46 pediatric patients with homozygous or heterozygous sickle cell disease. In particular, we assessed that an increased right ventricular end-diastolic volume is the earliest sign of this disease progression, promoted by a high pulmonary artery systolic pressure and followed by uncoupling with the pulmonary artery. Over time, the dysfunction of the right ventricle also affects the left ventricle, leading to global heart failure, which can be considered "right ventricle-driven". In addition, 3D echocardiography is an essential tool in the follow- up of this disease and together with the reduced incidence of acute chest syndrome or peripheral vaso-occlusive events to choose the optimal medical treatment.

Published Date: 2024-11-21; Received Date: 2024-10-21