Non-ischemic Primary and Secondary Cardiomyopathy

1354976 - Prevalence of asymmetric septal hypertrophy (ASH) in hypertrophic cardiomyopathy (HCM) and mimics

Thursday, January 26, 2023

1:00 PM – 2:00 PM

Location: Pacific Jewel

Presenting Author(s)

HS

Hunain Shiwani, MD

Clinical Research Fellow
University College London and Barts Heart Centre, United Kingdom

Primary Author(s)

HS

Hunain Shiwani, MD

Clinical Research Fellow
University College London and Barts Heart Centre, United Kingdom

Co-Author(s)

Joao B. Augusto, MD

Consultant Cardiologist
Hospital Prof Doutor Fernando Fonseca
Lisbon, Portugal
SR

Stefania Rosmini, MD

Consultant Cardiologist
King's College Hospital NHS Foundation Trust, England, United Kingdom
AI

Adam Ioannou, MBBS BSc

Doctor
National Amyloidosis Center, United Kingdom
RH

Rebecca Hughes, MD

Clinical Research Fellow
University College London and Barts Heart Centre
London, United Kingdom
Kristopher Knott, MD

Cardiology Registrar
Kings College Hospital
London, England, United Kingdom
JA

Jessica Artico, MD

Clinical Research Fellow
St Bartholomew's Hospital, England, United Kingdom
IP

Iain Pierce, PhD

Scientist
Barts Heart Centre at St Bartholomew's Hospital, United Kingdom
GJ

George Joy, MBBS

Research Fellow
University College London, United Kingdom
Rebecca Kozor, MD, PhD

Cardiologist
Royal North Shore Hospital
Sydney, New South Wales, Australia
RS

Richard P. P. Steeds, MD

Honorary Professor of Cardiovascular Medicine
University of Birmingham
Birmingham, England, United Kingdom
PK

Peter Kellman, PhD

Senior Scientist
National Institutes of Health, Maryland, United States
HX

Hui Xue, PhD

Director, Imaging AI Program
National Institutes of Health
Bethesda, Maryland, United States
LL

Luis R. Lopes, PhD

Associate Professor
University College London and Barts Heart Centre, United Kingdom
SM

Saidi A. Mohiddin, MD, PhD

Consultant Cardiologist in Inherited and Acquired Heart Muscle Disease and in CMR
Barts Heart Centre at St Bartholomew's Hospital, England, United Kingdom
Charlotte Manisty

Consultant Cardiologist
University College London and Barts Heart Centre
London, England, United Kingdom
Gabriella Captur, n/a

Consultant Cardiologist, Senior Clinical Lecturer
UCL
London, England, United Kingdom
Marianna Fontana, MD, PhD

Consultant Cardiologist, Director UCL CMR unit at the RFH
University College London
London, England, United Kingdom
Thomas A. Treibel, MD, PhD

Consultant Cardiologist
University College London, England, United Kingdom
James C. Moon, MD

Clinical Director, Imaging
Barts Heart Centre and UCL
London, England, United Kingdom
Rhodri Davies, MD, PhD

Associate clinical professor
University College London
London, Wales, United Kingdom

Disclosure(s):

Charlotte Manisty: No relevant disclosure to display

Marianna Fontana, MD, PhD: No relevant disclosure to display

Thomas A. Treibel, MD, PhD: No relevant disclosure to display

Background: Asymmetric involvement of the interventricular septum in left ventricular hypertrophy (LVH) is typically seen in hypertrophic cardiomyopathy (HCM) but is also seen in HCM mimics including hypertension, aortic stenosis, ATTR amyloidosis and Fabry disease. We analysed wall thickness and asymmetry in HCM and its mimics.

Methods: Left ventricular (LV) structure and function was automatically analysed using a clinically validated AI algorithm (1, 2) to measure the wall thickness in 8 cohorts: healthy volunteers (n=100), veteran athletes (n=275), hypertension (n=376), Fabry disease (n=242), aortic stenosis (n=125), ATTR (n=148) and AL cardiac amyloidosis (n=130) and hypertrophic cardiomyopathy (HCM) (n=490). A ratio of the thickest septal segment and thickest lateral segment >1.3 defined asymmetry (septal-lateral ratio; SLR) (3). Left ventricular hypertrophy (LVH) was defined by a maximum wall thickness (MWT) measurement of >15mm. Each patient was grouped into one of four categories: normal (no LVH, symmetric), asymmetric remodelling (no LVH, asymmetric), symmetric hypertrophy (LVH, symmetric) and asymmetric hypertrophy (LVH, asymmetric).

Results: The prevalence of asymmetric hypertrophy amongst groups are healthy volunteers (0%), veteran athletes (1.8%), hypertension (4.0%), Fabry disease (14.0%), AL amyloid (23.8%), aortic stenosis (26.4%), ATTR amyloid (37.2%) and HCM (61.4%).

Conclusion: Whilst asymmetric hypertrophy is the most common morphological variant in HCM, it is non-specific and present in all forms of LVH measured in this study including ATTR and AL amyloidosis and aortic stenosis.