Intraventricular Septation in the Context of Dilated Cardiomyopathy Associated With TTN Mutation

Karl R Khandalavala; Jean A Ballweg; Stanley J Radio; Jeffrey A Robinson

doi:10.1016/j.jaccas.2021.09.002

Intraventricular Septation in the Context of Dilated Cardiomyopathy Associated With TTN Mutation

JACC Case Rep. 2021 Nov 3;3(15):1674-1676. doi: 10.1016/j.jaccas.2021.09.002.

Authors

Karl R Khandalavala¹, Jean A Ballweg^{2

3}, Stanley J Radio⁴, Jeffrey A Robinson^{2

3}

Affiliations

¹ College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska, USA.
² Division of Cardiology, Department of Pediatrics, College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska, USA.
³ The Dr. C. C. and Mabel L. Criss Heart Center, Children's Hospital and Medical Center, Omaha, Nebraska, USA.
⁴ Department of Pathology, College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska, USA.

Abstract

A 6-month-old infant boy presented with symptomatic heart failure. Dilated cardiomyopathy was found in association with a mutation in TTN. Structural heart disease included novel septation of the left ventricle with a fenestrated membrane resulting from aberrant congenital mitral valve apparatus formation. (Level of Difficulty: Advanced.).

Keywords: BP, blood pressure; EFE, endocardial fibroelastosis; LV, left ventricle; TTN, titin gene; congenital heart disease; dilated cardiomyopathy; endocardial fibroelastosis; intraventricular septation; titin gene.