Myocardial Disease Flashcards
Definition of cardiomyopathy ?
Any structural disease of the myocardium, in absence of ischaemic or significant valvular or congenital heart disease
Different types of cardiomyopathy?
Hypertrophic cardiomyopathy Dilative cardiomyopathy Restrictive cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy (ARVC) Non compaction cardiomyopathy Takotsubo cardiomyopathy Peripartal cardiomyopathy Tachyarrhyrhmic cardiomyopathy Athlete‘s heart
Hypertrophic cardiomyopathy — role of MRI?
I1 Indication Diagnosis / Severity (volumes, function, mass, wall- thickness) Form (symmetric vs asymmetric, obstructive vs non-obstructive) Risk stratification (LGE, myocardial fibre disarray) Follow up (medical therapy, alcohol ablation, surgery)
Hypertrophic cardiomyopathy — forms and facts?
Symmetric and asymmetric phenotypes
Apical, midseptal, basal phenotype
Obstructive and non-obstructive forms
Hypertrophic cardiomyopathy — differential diagnosis?
Cardiac amyloidosis
Anderson Fabry disease
Löffler endocarditis (apical)
Cardiac hypertrophy due to valvular heart disease (aortic stenosis)
Cardiac hypertrophy due to congenital heart disease (coarctation)
Hypertrophic cardiomyopathy — MRI diagnosis?
Septal wall-thickness > 15mm
Septal wall-thickness > 13mm with known genotype
Myocardial fibre disarray with +LGE at septal hinge points
+/- myocardial edema
LVRI (left ventricular remodeling index) > 1.3
Myocardial crypts?
Outpouchings/small cavities of the ventricular wall, most often seen in the inferoseptal parts
Associated with phenotype-/genotype+ HCM
Dilative cardiomyopathy — role of MRI?
I1 Indication
Diagnosis / Severity (volumes, function, mass)
DDx (valvular disease, congenital heart disease, LVNCM, Tako-Tsubo)
Establishing the cause (myocarditis)
Prognosis (mass, LGE)
Complications (thrombi)
Dilative cardiomyopathy — MRI diagnosis?
Dilated left ventricle, short axis diameter > 60/55 mm (m/f)
EDVI>100/90 ml/m2 (m/f)
LVRI (left ventricular remodelling index) < 0.8
Decreased systolic function
Absence of hypertension or significant valvular disease
Absence of ischaemic heart disease
Differential diagnosis of dilative cardiomyopathy?
Non compaction cardiomyopathy
Peripartal cardiomyopathy
Tachyarrhythmic cardiomyopathy
Dilative cardiopathy due to valvular heart disease
Restrictive cardiomyopathy — role of MRI?
I1 Indication
Diagnosis / Severity (volumes, function, mass)
Differentiation restrictive cardiomyopathy vs constrictive pericarditis
Establishing the cause (amyloidosis, Fabry disease, hemochromatosis etc.)
Prognosis (mass, LGE)
Restrictive cardiomyopathy — diagnosis?
Diastolic dysfunction
Echocardiography — missing A-wave
Atrial dilatation
Arrhythmogenic right ventricular cardiomyopathy (ARVC) — MRI criteria ?
Demonstration of right ventricular akinesia/dyskinesia and/or right ventricular aneurysms
Major criteria: Increased right ventricular EDVI > 110/100 ml/BSA (m/f), decreased right ventricular ejection fraction (RVEF) < 40%
Minor criteria: Increased right ventricular EDVI > 100/90 ml/BSA (m/f), decreased right ventricular ejection fraction (RVEF) < 45%
ARVC — additional features in MRI?
Fibrofatty replacement of the right ventricular myocardium: often seen in free right ventricular wall and apex, rarely in septum (bad prognosis)
Scarring of the right ventricular wall with +LGE
ARVC — differential diagnosis (DDx)?
- Right ventricular (RV) infarct -usually encompasses the inferior and inferoseptal LV segments
- Valvular heart disease (PS, PI, MS, MI)
- congenital heart disease (ASD +/- PAPVR, Ebstein anomaly)
- cardiac sarcoidosis
- pulmonary hypertension
Non compaction cardiomyopathy (LVNC) — MRI diagnosis ?
- non-compacted vs compacted myocardium 2.3:1
- compacted vs compacted LV mass 40%
- non-compacted vs total LV mass > 20%
Non-compaction cardiomyopathy (LVNC) — differential diagnosis (DDx) ?
Hypertrophic cardiomyopathy (HCM) — apical form Dilative cardiomyopathy (DCM) with hypertrabeculation Congenital corrected transposition of the great arteries (L-TGA/ccTGA)
Takotsubo cardiomyopathy — role of CMR?
Confirm diagnosis Rule out (r/o) important differential diagnosis (DDx)
Takotsubo cardiomyopathy — CMR features?
„Apical ballooning“
Hypokinesia / akinesia of apical and midventricular segments
Cardiac edema, increased signal in T2w
Takotsubo cardiomyopathy — differential diagnosis (DDx)
Clinical: embolic (non-stenotic) myocardial infarction (MI), myocarditis
Image related: dilative cardiomyopathy, (Löffler endocarditis)
Athlete‘s heart — MRI features? Diagnostic clues?
Balanced increase in myocardial wall thickness and ventricular volumes
Concentric hypertrophy usually not exceeding 15mm
LVRI (left ventricular remodeling index) > 0.8 and < 1.3
No myocardial fibre disarray / LGE-
Absence of hypertension or significant valvular heart disease
Decreased extrazellular volume (ECV)
Hypertensive Heart Disease — facts?
Trias: left heart hypertrophy, myocardial fibrosis, coronary microangiopathy
Cause: long-standing untreated hypertension
Leads to diastolic dysfunction
Hypertensive heart disease — bad prognostic factor?
Left ventricular hypertrophy
Hypertensive cardiomyopathy — pathophysiology?
Left ventricular pressure overload secondary to increased vascular stiffness
Hypertensive cardiomyopathy — early left ventricular changes?
Left ventricular hypertrophy and hyperplasia
Increased inotropy
Prolonged isovolumetric relaxation
Shortened filling phase
Hypertensive cardiomyopathy / hypertrophic cardiomyopathy — clinical symptoms?
Angina — decreased coronary flow reserve
Ventricular and supraventricular arrhythmias
Hypertensive crisis (emergency)
Sudden cardiac death
Hypertensive cardiomyopathy — imaging features?
Left ventricular hypertrophy — septal/basal wall
Left atrial dilatation
Normal or mildly elevated left ventricular ejection fraction (LV-EF)
Diastolic dysfunction
Midwall septal and lateral enhancement
Corkscrew appearance of the small distal coronary segments
Cor pulmonale — definition?
Right ventricular hypertrophy and/or dilatation as a result of pressure overload / pulmonary hypertension
Pulmonary hypertension ?
Pulmonary artery pressure > 25 mmHg
Cor pulmonale / pulmonary hypertension — aetiology?
Nizza classification:
Pulmonary hypertension: idiopathic, hereditary, medications/toxins, associated with different conditions
Pulmonary hypertension associated with left heart disease: systolic / diastolic dysfunction, valvular heart disease, congenital outflow tract obstruction
Pulmonary hypertension associated with pulmonary disease or hypoxia: COPD, ILD, other obstructive/restrictive lung disease, sleep apnea etc.
Chronic thromboembolic pulmonary hypertension (CTEPH)
Dilative cardiomyopathy — definition?
LV-dilatation and LV-systolic-dysfunction in the absence of abnormal loading conditions (hypertension / valvular disease) or CAD sufficient to cause global systolic impairment.
RV-dilatation or RV-dysfunction may be present.
Dilative cardiomyopathy — aetiology?
Sporadic: myocarditis, toxic, autoimmune,metabolic
Genetic: 20-35%
Workup to exclude ischaemic heart disease?
Ideally CMR and coronary CTA / coronary angiogram
Why? — Soriano et al. 2005
~10% with myocardial infarction (LGE+) do not show coronary stenosis
~20% with significant stenosis do not have LGE+
Non compaction cardiomyopathy (LVNC) — role of MRI ?
I1 Indication
- diagnosis / severity (volumes, function, mass)
- r/o differential diagnosis (DDx)
- complications
Arrhythmogenic right ventricular cardiomyopathy (ARVC) — role of MRI ?
I1 Indication
- diagnosis / severity (volumes, function, LGE, fibrofatty deposits)
- complications
- r/o DDx (e.g. right ventricular infarction)
Dilated cardiomyopathy - origins ?
- genetic
- postpartum
- infectious (viral, lyme)
- autoimmune(sarcoidosis, LED)
- toxic (alcohol)
- metabolic (Cushing, diabetic)
ARVC - epidemiologic facts ?
- prevalence: 1/2000 - 1/5000
- more severe in males (hormon induced)
- responsible for ~ 20% of sudden cardiac deaths (SDC)
- biventricular involvement ~ 60%
ARVC - clinical symptoms ?
- arrhythmia, RBBB
- syncope
- right sided heart failure (ascites, peripheral edema)
Possible causes of cardiac deaths ?
- ARVC ~ 20%
- hypertrophic cardiomyopathy (HOCM, SAM)
- coronary anomalies (malignant)