Hypertrophic cardiomyopathy (HCM) and storage diseases – clarifications

Cardiac magnetic resonance imaging (cardiac MRI) plays a key role in the diagnosis of a pathological increase in the mass and thickness of the heart muscle. Such changes in the heart muscle can either be genetic (HCM) or can occur as part of storage diseases in which substances are deposited in the heart muscle. These substances include iron (e.g. in hemochromatosis), pathological proteins (e.g. in amyloidosis) or building blocks of cell membranes such as sphingolipids in Fabry disease.

Using cardiac MRI, the effects of HCM and storage diseases on the structure (hypertrophy or fibrosis) and function of the heart muscle can be examined in detail.

Typical image of cardiac amyloidosis in cardiac MRI with concentric hypertrophy (left), significantly increased T1 mapping (center) and pronounced myocardial late gadolinium enhancement (LGE, right).

Typical image of cardiac amyloidosis in cardiac MRI with concentric hypertrophy (left), significantly increased T1 mapping (center) and pronounced myocardial late gadolinium enhancement (LGE, right).

In addition, the magnetic relaxation properties of the heart muscle change in a characteristic way in storage diseases. A particular strength of cardiac MRI is its ability to quantify these relaxation characteristics (T1, T2 and T2*) and thus provide important information for diagnosis, particularly in the early stages of the disease.

When diagnosing patients with storage diseases, the radiology department works closely with an interdisciplinary team of specialists to ensure optimal care(amyloidosis network).

Cardiac MRI of a patient with HCM. There is clear hypertrophy of the septum (left) and typical fibrosis, emphasized at the base of the right ventricle (right, red arrows).

Cardiac MRI of a patient with HCM. There is clear hypertrophy of the septum (left) and typical fibrosis, emphasized at the base of the right ventricle (right, red arrows).

Responsible squad

Hatem Alkadhi, Prof. Dr. med.

Senior Attending Physician, Vice Director of the Institute, Institute for Diagnostic and Interventional Radiology

Tel. +41 44 255 36 62
Specialties: Multimodal cardiovascular imaging, Emergency radiology, Computed tomography

Robert Manka, Prof. Dr. med.

Senior Attending Physician, Institute for Diagnostic and Interventional Radiology
Senior Attending Physician, Department of Cardiology

Tel. +41 44 255 12 51
Specialties: Cardiac imaging (heart MRI & heart CT), Interventional cardiology, Consultation hours for complex coronary interventions

Malgorzata Polacin, PD Dr. med.

Attending Physician, Institute for Diagnostic and Interventional Radiology

Tel. +41 43 253 15 21
Specialties: Cardiovascular imaging (cardiac MRI, cardiac CT), Oncological imaging

Alexander Gotschy, Dr. med.

Attending Physician, Department of Cardiology
Attending Physician, Institute for Diagnostic and Interventional Radiology

Tel. +41 43 253 78 90
Specialties: Interventional cardiology, Cardiac magnetic resonance imaging (cardiac MRI), Imaging of congenital heart defects

Oliver Müggler, Dr. med.

Attending Physician, Institute for Diagnostic and Interventional Radiology

Tel. +41 43 253 78 69

Verena Charlotte Wilzeck, Dr. med.

Attending Physician, Institute for Diagnostic and Interventional Radiology
Attending Physician, Department of Cardiology

Tel. +41 43 253 50 37

For patients

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For referrer

University Hospital Zurich
Institute for Diagnostic and Interventional Radiology
Rämistrasse 100
8091 Zurich

Tel. +41 43 254 41 10
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