Lee Simon,  Krishnamurthy Rajesh,  Krishnamurthy Ramkumar,  Johnston Thomas,  Hor Kan

Final Pr. ID: Poster #: SCI-009

Duchenne muscular dystrophy (DMD) is a myopathy with a natural history of progressive cardiomyopathy and vasogenic edema, fatty infiltration, and myocardial fibrosis. We recently identified a group of patients with DMD who presented with acute onset of chest pain (ACP), troponin leak (TL), and new late gadolinium enhancement (LGE), similar in presentation to acute viral myocarditis (AM). It is unclear if these patients have suffered an episode of AM or if this is a different disease process. Given the presumed acute myocardial necrosis in both processes, we hypothesize that native T1 and T2 values would be elevated in AM and DMD patients with ACP compared to asymptomatic DMD patients. Read More

Authors:  Lee Simon , Krishnamurthy Rajesh , Krishnamurthy Ramkumar , Johnston Thomas , Hor Kan

Keywords:  Parametric Mapping, Cardiomyopathy

Navallas Maria,  Zuccarino Flavio,  Inarejos Clemente Emilio J,  Marie Eman,  Gerrie Samantha,  Ladera Gonzalez Enrique,  Barber De La Torre Ignasi

Final Pr. ID: Poster #: EDU-010

Learning objectives:
1.Review the main pediatric cardiomyopathy phenotypes.
2.Clarify the role of MRI across the care pathway.
3.Walk through the key MRI sequences we actually use in kids.
4.Recognize the essential imaging findings that help us differentiate among them.

- Introduction:
Pediatric cardiomyopathies are a rare heterogeneous group of myocardial diseases but are the first cause of heart transplantation in children.
Causes are diverse—genetic mutations, coronary anomalies, infections, toxins, arrhythmias—and sorting out phenotypes can be tricky because adult criteria are often not applicable to kids and because many kids don’t tolerate long exams.

- Imaging techniques:
Echocardiography represents the first first-line modality but it is often complemented with CMR as it allows a radiation-free differentiation between different phenotypes with unique myocardial tissue characterization, playing a key role in diagnosis, risk stratification, and treatment assessment.

- Key MRI sequences:
Conventional cardiomyopathies protocols in children may use 1.5 or 3 Tesla field strengths and include the following sequences:
Initial localizers.
Cine imaging.
Phase contrast.
Parametric mapping.
LGE.

- Dilated Cardiomyopathy:
Most common.
Leading cause of heart transplant.
Dilated chambers.
Reduced systolic function.
LGE: Patchy or longitudinal mid-wall, transmural, subepicardial, or diffuse subendocardial involvement. Presence of LGE indicates poor prognosis and increased risk of SCD.
Native T1 mapping is suggested to be the strongest independent predictor of diffuse myocardial disease, allowing for the identification of patients at risk for adverse outcomes.

- Hypertrophic Cardiomyopathy:
2nd most common.
LV hypertrophy z-score >2.5 (no family history) or >2 (with family history/genetic test).
LVOT obstruction and SAM.
LGE: Independent risk factor for SCD.
Differential diagnosis with athlete's heart.

- Arrhythmogenic Cardiomyopathy:
RV and/or LV dilatation and reduced EF.
Wall motion abnormalities – most important finding in kids.
Myocardial fibrosis.
Fatty infiltration rare in children.

- Restrictive Cardiomyopathy:
Rarest CMP.
Non-dilated biventricular failure and biatrial dilatation.
Ddx: constrictive pericarditis.

- Excessive trabeculation/NCCM:
Non-compacted/Compacted myocardium ratio > 2.3 measured in diastole.
In the absence of dilation or systolic dysfunction, can be considered a phenotypic trait not necessarily associated with CMP. Read More

Authors:  Navallas Maria , Zuccarino Flavio , Inarejos Clemente Emilio J , Marie Eman , Gerrie Samantha , Ladera Gonzalez Enrique , Barber De La Torre Ignasi

Keywords:  Cardiomyopathy, Cardiac MRI

Sharma Priya,  Rajderkar Dhanashree

Final Pr. ID: Poster #: EDU-114

Learning Objectives: This educational exhibit will review glycogen storage diseases and the imaging findings of these disease processes using a multimodality approach. We will also review complications and discuss the treatment and role of imaging as a measure of treatment response. Read More

Authors:  Sharma Priya , Rajderkar Dhanashree

Keywords:  Glycogen Storage Diseases, Hepatic Adenoma, Hepatocellular Carcinoma, Cardiomyopathy, GSD

Zucker Evan

Final Pr. ID: Poster #: EDU-093

Cardiomyopathy is rare in children but associated with significant morbidity and mortality when symptomatic. It also comprises a large proportion of cardiac MR imaging referrals in typical pediatric radiology practice. The purpose of this educational exhibit is to review the major types of cardiomyopathy in children, focusing on cardiac MRI. Read More

Authors:  Zucker Evan

Keywords:  Cardiomyopathy, Cardiac, MRI, Pediatric, Late gadolinium enhancement

Johnston Thomas,  Lee Simon,  Krishnamurthy Ramkumar,  Krishnamurthy Rajesh,  Hor Kan

Final Pr. ID: Poster #: SCI-010

Cardiomyopathy is the leading cause of mortality for Becker Muscular Dystrophy (BMD).Risk stratification is challenging due to the wide variation of cardiomyopathy onset, progression, and severity; with cardiomyopathy developing independent of skeletal myopathy progression. Current guidelines recommend “complete cardiac evaluation” at ten years of age, but do not specify the type of imaging modality. We have been performing cardiac magnetic resonance imaging (CMR) routinely by 10 years of age. We sought to determine the progression of BMD cardiomyopathy during the adolescent years among patients who have evidence of fibrosis by late gadolinium enhancement(LGE). Read More

Authors:  Johnston Thomas , Lee Simon , Krishnamurthy Ramkumar , Krishnamurthy Rajesh , Hor Kan

Keywords:  Cardiomyopathy, LGE