Cardiomyopathy and its Echo findings

S. Iswarya

Cardiac Technician, Department of OPD, Kauvery Heart city, Trichy, Tamil Nadu, India

Background

Cardio – Heart, Myo – Muscle, Pathy – Disease

Any disorder that affects the heart muscle is called a cardiomyopathy. Cardiomyopathy causes the heart to lose its ability to pump blood well. Cardiomyopathy can lead to heart failure. In some cases, the heart rhythm also becomes disturbed. This leads to arrhythmias (irregular heartbeats).

Types of Cardiomyopathy

  1. Hypertrophic cardiomyopathy
  2. Dilated cardiomyopathy
  3. Restrictive cardiomyopathy
  4. Left ventricular noncompaction
  5. Arrhythmogenic right ventricular cardiomyopathy
  6. Takotsubo cardiomyopathy, or broken heart syndrome

1. Hypertrophic cardiomyopathy (HCM)

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Hypertrophic cardiomyopathy is a disease in which the heart muscle becomes thickened (hypertrophied). The thickened heart muscle can make it harder for the heart to pump blood.

There are two main types of HCM,

  1. Obstructive hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy typically affects the muscular wall (septum) between the two bottom chambers of the heart (ventricles). The thickened wall might block blood flow out of the heart

  1. Non-obstructive hypertrophic cardiomyopathy

The heart muscle thickens but doesn’t block blood flow.

Echo findings

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  1. Maximal wall thickness >15 mm in any myocardial segment (or)
  2. Septal/posterior wall thickness ratio > 1.3 in normotensive patients.

Assessment of the mitral valve

Systolic anterior motion (SAM) of the mitral valve was first described as a feature of HCM. Anterior motion of mitral leaflets in systole resulting in movement of leaflets into the LVOT and thus impediment to ejection of the stroke volume out the aortic valve. Varying degrees; mild, moderate, severe (septal contact for >30% of systole).

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Mitral valve apparatus

  1. Anterior leaflet elongation (AML > 30 mm (17 mm/m2)
  2. Posterior leaflet elongation (Absolute height of PL >15 mm)
  3. Papillary muscle abnormalities (Anterior displacement of AL PM)
  4. Aorto-mitral angle <120o
  5. Mitral chordae (Elongation/thickening/buckling)

Systolic function

Left ventricular (LV) systolic function is characteristically normal to hyper dynamic in HCM. However, prior studies have suggested that 49% of patients develop systolic dysfunction, defined by LV ejection fraction (LVEF) <50% (herein referred to as HCM with LV systolic dysfunction).

Key points

Systolic longitudinal dysfunction

  1. Tissue Doppler imaging (TDI) < 4 cm/s
  2. Global longitudinal Strain (GLS) > 10.6% d
  3. Paradoxical apical strain (apical HCM)

Diastolic Dysfunction

HCM has LV diastolic dysfunction which occurs early in the disease evolution and is due to increased LV mass and stiffness. Diastolic dysfunction with elevated LVEDP is present in HCM patients if > 50% of the variables meet the cut-off values

  1. E/e 14
  2. LA volume index > 34 mL/m2
  3. Pulmonary vein atrial reversal velocity (Ar-A duration 30 ms)
  4. TR peak velocity of 2.8 m/s.

LA enlargement

LA is often enlarged in HCM patients, because of diastolic dysfunction and MR. It is important to recognize that use of linear dimensions may mispresent true LA size, because of asymmetric dilatation

2. Dilated cardiomyopathy (DCM)

The disease starts in the left ventricle, the heart’s main pumping chamber. The heart muscle begins to dilate, stretching and becoming thinner. As a result, the inside of the chamber enlarges. The problem often spreads to the right ventricle and then to the atria,

There are two types of DCM

  1. Dilated cardiomyopathy
  2. Ischemic dilated cardiomyopathy

Echo findings

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  1. Wall motion – uniform global hypokinesia. Global decrease in systolic contractile function with eventual dilatation of the ventricles
  2. Dilated chambers – LV, LA, RV, RA
  3. Usually normal LV wall thickness
  4. Systolic function – reduced systolic function. decreased EF, < 45%
  5. Diastolic function – impaired – may reflect volume overload
  6. Usually present Mitral regurgitation
  7. RV dysfunction – high incidence (true myopathy do not differentiate RV and LV).

Ischemic dilated cardiomyopathy echo findings

Ischemic cardiomyopathy (CM) is a type of dilated cardiomyopathy. When coronary artery disease is present, or after a heart attack, an artery to the heart can become blocked for a short time, preventing oxygen-rich blood from entering the heart. When this happens, the heart muscle can become enlarged, dilated and weak.

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  1. Wall motion – Global hypokinesia with regional variation
  2. Dilated chambers – LV, LA dilatation predominant
  3. Mitral regurgitation – Often present (Eccentric jet common due to differential papillary muscle involvement)
  4. Diastolic dysfunction – Grade 1 or 2
  5. Less common (RVD is due to spiral muscle sharing between RV and LV).

3. Restrictive Cardiomyopathy (RCM)

Restrictive cardiomyopathies are associated with impaired ventricular filling and increased LV end diastolic pressure. Echocardiographic features include bi-atrial dilatation, hypertrophied ventricles with decreased compliance, initially small LV cavities, and normal to depressed systolic function.

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LV Function

Restrictive left ventricular (LV) physiology is produced by an increased myocardial stiffness, causing a rapid rise in ventricular pressure at the beginning of the diastolic phase with only small increases in filling volumes or a critical reduction of ventricular volumes up to a near obliteration, caused by massive.

Systolic Function

Systolic function is usually remains normal, atleast early in the disease. The entity is the subset of what has been referred to as a heart failure with preserved ejection fraction.

Diastolic Function

Restrictive cardiomyopathy is characterized by noncompliant ventricular walls that resist diastolic filling; one (most commonly the left) or both ventricles may be affected. Symptoms include fatigue and exertional dyspnea. Diagnosis is by echocardiography and cardiac catheterization

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ECHO Findings

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  1. A low or normal diastolic volume
  2. Normal or only mildly reduced LV ejection fraction, atrial enlargement
  3. Abnormal diastolic function
  4. Diastolic dysfunction frequently with an elevated peak mitral inflow velocity. reduced isovolumic relaxation time (<70m/s). Increased E velocity (>1 m/s). Reduced deceleration time (< 160 ms). Reduced A velocity (< 0.5 m/s). Reduced A wave duration. Increased E: a ratio (- >2)
  5. Rapid early mitral inflow deceleration, and reduced doppler tissue imaging early annular velocity. Reduced Em velocity (lateral annulas). < 8 – 10 cm/ s
  6. Pulmonary vein -Reduced systolic flow, increased diastolic flow, reduced systolic: diastolic flow ratio, increased peak atrial reversal velocity and duration

4. Left ventricular non-compaction

Left ventricular non – compaction (LVNC) is a very rare congenital cardiomyopathy. It is a disease of endomyocardial trabeculations that increase in number and prominence. This cardiomyopathy carries a high risk of malignant arrhythmias, thromboembolic phenomenon and left ventricular dysfunction.

 

Echo findings

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  1. Abnormal segmental myocardial thickening of LV or RV due to hypertrabeculation with two – layered pattern
  2. Thickened noncompacted (N) endocardial layer with a compacted (C) thin epicardial layer
  3. NC/C ratio > 2 in PSLX end systole
  4. Segmental or global cardiac dysfunction
  5. No septal hypertrophy
  6. Trabecular meshwork in apex or midventricular segments of inferior and lateral wall
  7. Deep Recesses

5. Arrhythmogenic cardiomyopathy (ACM)

ACM is a genetic heart muscle condition which affects the right ventricle, left ventricle, or both. ACM has been referred to in the past as Arrhythmogenic Right Ventricular Cardiomyopathy as it was initially thought that it exclusively affected this pumping chamber of the heart.

Echo findings

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  1. Regional dyskinesia, akinesia, or aneurysm of the right ventricle
  2. Increased end-diastolic right ventricular outflow tract (RVOT) diameter. greater than or equal to 32 mm (parasternal long-axis measurement). 36 mm or greater when measured in a parasternal short-axis. respective cutoffs when corrected for body surface area are 19 and 21 mm/m2
  3. A fractional area change of 33% or less.

6. Takotsubo cardiomyopathy or “Broken Heart Syndrome”

Takotsubo cardiomyopathy or “Broken Heart Syndrome” is when the heart muscle becomes suddenly stunned or weakened

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Echo findings

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  1. Wall motion: Basal akinesis and apical normo-hyperkinesis. Key echocardiographic features during the acute phase in takotsubo cardiomyopathy are usually characterized by symmetrical regional abnormalities involving the mid-ventricular segments of the anterior, inferior, and lateral walls
  2. Left ventricular ejection fraction < 35 %
  3. Dilated ventricles (octopus appearance)
  4. LVOTO Intraventricular gradient 25 mmHg.

Conclusion

Cardiomyopathy and its Echo findings are described

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S. Iswarya

Cardiac Technician

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