Non-ischemic cardiomyopathy

a) SCMR official document standardized acquisition guidelines – relevant extract

LV structure and function module
Consider T2-weighted imaging in the acute setting when necrosis/edema may be present (e.g. myocarditis)
a. Body coil should be used or alternatively functional surface coil intensity correction algorithms
b. Breath-hold, segmented fast spin-echo imaging (double inversion recovery)
c. Perform imaging prior to contrast administration
d. Selected slices based on cine imaging findings (e.g. 2 and 4-chamber long axis and 3 representative short axis slices)
e. Adjust readout to mid-diastole
f. Slice thickness at least 10 mm
g. Slice thickness of dark blood prep if used should be greater than the base-apex motion of the mitral annulus
Late Gadolinium Enhancement module
a. Analysis – Examine the "pattern" of enhancement as certain nonischemic cardiomyopathies have predilection for scarring in typical ways
Optional – adenosine stress-rest perfusion imaging or high dose dobutamine stress functional imaging (see stress protocols) to comment on the presence or absence of ischemia since a mixed cardiomyopathy may be present
For hypertrophic cardiomyopathies -LV outflow tract flow imaging for presence of turbulence, systolic anterior motion, and phase velocity measures of gradient if present.

b) SCMR official document reporting recommendations – relevant extract

No specific recommendations (but see the LV function module, LGE reporting and ARVC, next disease)

c) Standardized web based images

HCM case
Case notes: Case from Dr James Moon, the Heart Hospital, London. This case is of hypertrophic cardiomyopathy in a progressive disease phase. There is mid-cavity obliteration, and an apical aneurysm. This has thrombus in it, and there is extensive late gadolinium enhancement. Additional features are RVH, but no SAM, or LVOT obstruction.

d) Case of the Week example(s)

Number 10-21: Role of CMR in the diagnosis of ARVC
History: A 78 year old patient was admitted to the Accident and Emergency department with a syncope. The ECG showed a broad complex tachycardia (left bundle-branch morphology with superior axis) with a rate of 230 bpm. The patient received DC cardioversion under sedation, which resulted in sinus rhythm.

Number 10-15: T2* CMR to tailor chelation therapies
History: A 35 year old male with beta-thalassemia major, regularly transfused since the age of 30 months, started chelation treatment with subcutaneous desferrioxamine at the age of 4 years.

Number 10-20: Electrophysiologic and CMR features of ARVC
History: A 19-year old caucasian female with documented ventricular tachycardia was referred to electrophysiology service.

Number 10-12: Spiral Hypertrophic Cardiomyopathy
History: This is a case of HCM with asymmetrical hypertrophy which is distributed in a spiral or helical way from base to apex.

Number 10-09: Acute Pulmonary Edema in an Active Duty Officer in Iraq
History: A 27-year-old female active duty soldier with no significant past medical history was evacuated from Iraq for flash pulmonary edema.

Number 10-08: Perfusion Abnormalities in Cardiac Amyloidosis
History: A 68 year-old hypertensive male patient presented with typical chest pain. The ECG showed new characteristic ischemic changes and troponin dosage was positive. An urgent coronary angiogram revealed unobstructed epicardial coronary arteries. The echo showed a significant degree of septal wall thickening, out of proportion with his well-controlled blood pressure profile, suggesting possible hypertrophic cardiomyopathy.

Number 09-06: Heterogeneous etiologies affecting a patient with cardiac amyloidosis
History: 81 year-old male with history of atrial fibrillation

Number 09-03: Coincident Hypertrophic Cardiomyopathy and Left Ventricular Non-Compaction
History: Woman, 44 y/o, diagnosed by echocardiography of Non-Obstructive Hypertrophic Cardiomyopathy (HCM)

Number 10-11: Cardiac Sarcoidosis
History: A 32-year-old man presented with chest pain, fever, erythema nodosum of the lower extremities and swelling of both ankles. ECG showed ST-elevation in leads I and aVL and troponin was raised 40-fold.

Number 06-02: High Risk HCM
History: A 33 year old man with HCM with a family history of sudden death. Seven years previously, a single Holter had shown 3 beats of NSVT. Cine CMR shows HCM with ASH, no SAM, no LVOTO (left or right), and normal systolic function. After gadolinium, there was very extensive late enhancement, (44% of LV myocardium, arrowed). The scan triggered re-evaluation of cardiac risk, and the patient was offered an ICD.

Number 06-05: Apical HCM by CMR and echo
History: A 64 year old women presented with chest pain and palpitations. The ECG showed Extensive T wave inversion. Echocardiography (L) was reported as showing diastolic dysfunction and an akinetic apex. Cine CMR confirmed the clinical suspicion of apical hypertrophic cardiomyopathy (aHCM).

Number 07-07: HCM with LVOT obstruction by CMR
History: 62 year old man with HCM with obstruction referred for alcohol septal ablation (ASA) refractory symptoms of angina and dyspnea. This was a baseline study done prior to ASA.,

Number 08-02: Apical HCM, fibrosis and sustained VT
History: An 82 yr man admitted with palpitations, presyncope and sustained monomorphic VT. Family history, his brother died age 6 of unknown cause.

Number 08-07: Left Ventricular Trabeculae, Non-Compaction and DCM
History: 18 Y/O male presented with dyspnea and palpitations. His father had died suddenly at age 32.

Number 08-13: Viral Myocarditis by CMR
History: A 22-year-old college student noted chest pain one week after recovering from flu-like symptoms. His ECG revealed inferolateral ST-elevation and his cardiac biomarkers were elevated.

Number 08-15: Danon Disease - A rare cause of LV hypertrophy
History: 20 years old male, history of RF ablation for WPW syndrome, without prior history of heart failure.

Number 08-17: Isolated Left Ventricular Apical Hypoplasia
History: 35yr male. Known previous DCM diagnosed via echocardiography. Currently stable, NYHA Class I, and well controlled. No significant arrhythmias noted. Referred for CMR.

Number 08-18 'Bystander' ST elevation MI in DCM
History: A 52 yr old lady, previously asymptomatic with no previous medical history, underwent a primary PCI for acute chest pain with inferior STEMI and LBBB. The left coronary system was unobstructed and RCA was occluded distally. PCI successfully established flow into the postero-lateral branch but not posterior descending branch of the RCA.

Number 08-19 Diagnosing RV dilatation by CMR
History: ** Case of the year winner, 2008

Number 08-20: Imaging Restrictive cardiomyopathy by CMR
History: A 23 year old male presented with increasing dyspnea, abdominal distension, pedal edema and orthopnea over 18 months.

Number 08-21: Myocardial scarring from 2 distinct pathologies
History: Asymptomatic 62 yr ?. Diagnosed with HCM more than 20 with an abnormal ECG. Recent echo demonstrated concentric LVH. CMR to further assess this.

Number 08-23: A tricky diagnostic challenge
History: A man in his 40s presenting to respiratory medicine with 6 months breathlessness and hemoptysis.

e) Expert opinion – ‘How we do’

"How I do" CMR in DCM (Sanjay Prasad, Royal Brompton Hospital)
"How I do" CMR in HCM (Sanjay Prasad, Royal Brompton Hospital)

f) Relevant Online Talks

Free talks

ARVD the Imager View
By Francisco Alpendurada - Amsterdam, The Netherlands
Recorded at EuroCMR 2009 Athens

Dilated Cardiomyopathies
By Albert van Rossum - Amsterdam, The Netherlands
Recorded at EuroCMR 2009 Athens

Hypertrophic Cardiomyopathies
By Rory O'Hanlon - London, UK
Recorded at EuroCMR 2009 Athens

CMR in HCM
By James Moon - The Heart Hospital, London
Recorded at The 2007 UK Cardiomyopathy Association meeting - HCM:

Diagnostic challenges: CMR for LV non-compaction
By James Moon - The Heart Hospital, London
Recorded at HCM working group of the Spanish cardiology society 2008

CMR for heart failure
By Dudley Pennell
Recorded at LondonCMR

CMR in ARVC
By James Moon - The Heart Hospital, London
Recorded at The 2008 UK Cardiomyopathy Association meeting - ARVC:

Cardiomyopathies in Latin America; are we different?
By Horacio Di Nunzio - Argentina
Recorded at Second Latin American SCMR Chapter Meeting, 2008

ARVC and sudden death
By Matthias Friedrich
Recorded at EuroCMR 2008

Members only talks - general
Systemic Disease
By Sophie Mavrogeni - Onassis Cardiac Surgery Center
Recorded at SCMR 2010
Iron Overload
By Sophie Mavrogeni - Onassis Cardiac Surgery Center
Recorded at SCMR 2010
Technical challenges in tissue characterisation beyond LGE
By Anthony Aletras - NHLBI-National Institutes of Health
Recorded at SCMR 2010
Myocardial Tissue Characterisation in Heart Failure - Late Gadolinium Enhancement and Beyond
By Christopher Kramer - University of Virginia Health Systems
Recorded at SCMR 2009 Plenary Session 1: Heart Failure MRI - The Gold Standard
Recognizing Myocardial Iron Overload
By Sophic Mavrogeni - Onassis Cardiac Surgery Centre
Recorded at SCMR 2009 Physician preconference
CMR in Thalassemia
By Massimo Lombardi - CNR Clinical Physiology Institute
Recorded at SCMR 2009 Clinical Non-ischemic - CMR in systemic disease
CMR in myocarditis
By Peter Hunold - University Hospital Schleswig-Holstein
Recorded at SCMR 2009 Clinical Non-ischemic - CMR in systemic disease
Cardiomyopathies and Right Ventricular Dysplasia
By Ian Paterson - University of Alberta
Recorded at SCMR 2008: Technicians Workshop

g) Relevant papers (starting point):

Moon JC, McKenna WJ, McCrohon JA et al. Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol 2003;41:1561–7.
McCrohon JA, Moon JC, Prasad SK et al. Differentiation of heart failure related to dilated cardiomyopathy and coronary artery disease using gadolinium enhanced cardiovascular magnetic resonance. Circulation 2003;108:54–9.
Moon JC, Reed E, Sheppard MA et al. The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Am Coll Cardiol 2004;43:2260–4.
Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I, Sheppard MN, Poole-Wilson PA, Hawkins PN, Pennell DJ.Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation. 2005;111:186-93.
Sharkey SW, Lesser JR, Zenovich AG et al. Acute and Reversible Cardiomyopathy Provoked by Stress in Women From the United States. Circulation. 2005;111:472-479.
S. Petersen, J. Selvanayagam, F. Wiesmann, M. Robson, J. Francis, R. Anderson, H. Watkins, S. Neubauer. Left Ventricular Non-CompactionInsights From Cardiovascular Magnetic Resonance Imaging. J Am Coll Cardiol, 2005;46:101-105