Echocardiographic Diagnosis of Cardiac Amyloidosis and Hypertrophic Cardiomyopathy
Cardiac Amyloidosis
- The European Society of Cardiology recommends that granular sparkling myocardial appearance and thickened heart valves are key features to distinguish cardiac amyloidosis from hypertrophic cardiomyopathy 1, 2
- Concentric left ventricular hypertrophy with symmetrically thickened walls, typically without cavity dilation, is a characteristic feature of cardiac amyloidosis 1, 2
- Increased interatrial septum thickness (>6 mm) is highly specific for amyloidosis 1, 2
- Thickened atrioventricular valve leaflets (mitral and tricuspid) are commonly seen in cardiac amyloidosis 1, 2
- Increased right ventricular free wall thickness distinguishes amyloidosis from isolated HCM 1, 2
- Biatrial enlargement despite relatively preserved ventricular cavity size is a feature of cardiac amyloidosis 3
- Restrictive filling pattern with E/A ratio ≥2 and E-wave deceleration time ≤150 ms is characteristic of cardiac amyloidosis 1
- Mildly reduced ejection fraction (typically 40-50%) with restrictive physiology contrasts with hyperdynamic function in HCM 1, 2
- Severely reduced longitudinal strain affecting all segments globally, with relative apical sparing creating a "bulls-eye" pattern, is seen in cardiac amyloidosis 3
Hypertrophic Cardiomyopathy
- The American College of Cardiology recommends that maximal LV wall thickness ≥15 mm (or ≥13-14 mm in family members with confirmed HCM) measured anywhere in the left ventricle is diagnostic of HCM 4, 5
- Asymmetric septal hypertrophy is the most common pattern involving basal anterior septum and anterior free wall 4, 5
- Systolic anterior motion (SAM) of the mitral valve with mitral-septal contact is highly specific for HCM 1, 4, 5
- Left ventricular outflow tract obstruction with gradient ≥30 mmHg at rest or with provocation (Valsalva, standing, exercise) is a feature of HCM 1, 2, 7
- Normal or hyperdynamic ejection fraction (typically >65%) contrasts with mildly reduced EF in amyloidosis 1, 2
- Abnormal diastolic function with elevated E/e' ratio indicating elevated filling pressures is seen in HCM 1, 2
- Restrictive filling pattern (E/A ≥2) in advanced cases indicates poor prognosis 1