Classical Echocardiography Features Of Cardiac Amyloidosis-National Heart Institute Of Malaysia Experience

Classical Echocardiography Features Of Cardiac Amyloidosis
National Heart Institute Of Malaysia Experience

NATIONAL HEART INSTITUTE OF MALAYSIA

2D ECHOCARDIOGRAPHY

CASE REVIEW

RESTRICTIVE CARDIOMYOPATHY

CARDIAC AMYLOIDOSIS

Myocardial infiltration by amyloid fibrils can occur in primary, familial, secondary, and senile amyloidosis. The degree of involvement is variable depending upon the type of amyloidosis. Two dimensional echocardiography remains the ideal method for identifying and following individuals with cardiac amyloidosis.

Characteristic two dimensional and Doppler echocardiographic features have been described in individuals with cardiac amyloidosis. Two dimensional features include thickening of the LV walls, increased reflectivity of these walls (the ‘‘speckled’’ or ‘‘granular’’ myocardium), biatrial enlargement, thickening of the interatrial septum, thickening and regurgitation of the mitral and tricuspid valves, and the presence of a small pericardial effusion.

Transmitral Doppler flow patterns in patients with amyloidosis exhibit evidence of diastolic dysfunction. Characteristic transmitral Doppler patterns representative of early impaired relaxation and later restrictive filling have been demonstrated in this population. An increase in both the pulmonary venous Doppler atrial reversal duration and the ratio of this atrial reversal duration to the transmitral A wave duration have been observed in these patients and reflect increased LA pressure.

Cardiac amyloidosis should be considered in individuals in whom several of these echocardiographic features are observed. The coexistence of increased thickening of the LV walls on echocardiography yet low voltage on electrocardiography is highly suggestive of amyloid infiltration of the myocardium.

Differentiating cardiac amyloidosis from hypertrophic cardiomyopathy can be difficult on echocardiography. Asymmetric septal thickening can result from focal amyloid deposition and can mimic hypertrophic cardiomyopathy. The presence of decreased LV function would argue strongly against hypertrophic cardiomyopathy. The presence of highly reflective myocardium can also be seen in individuals with primary and secondary causes of LV hypertrophy, ventricular fibrosis, and other infiltrative processes.

The integration of clinical, echocardiographic, and electrocardiographic data is essential when making the diagnosis of cardiac amyloidosis. While the long term prognosis is substantially worse inmpatients with primary amyloidosis, the echocardiographic distinction between familial, primary, and secondary amyloidosis can be difficult.

Impaired LV systolic function is more common in primary amyloidosis than in the other forms. Doppler tissue echocardiography is helpful in differentiating amyloid patients from those with similar two dimensional echocardiographic features but without amyloidosis. Abnormally low tissue Doppler diastolic velocities are present in individuals with cardiac amyloid compared with control patients.

58 years old male. 
Presents with worsening S.O.B, weight gain and fatique.
Echocardiography study done with bedside echocardiography routine post percuataneous coronary intervention to rule out pericardial effussion. Incidental finding by cardiovascular technologist on duty and noted in the preliminary echocardiography report.

CASE REVIEW

SUGGESTED PRIMARY CARDIAC AMYLOIDOSIS EARLY DIAGNOSIS CCF

[ECHO FINDING:TOSHIBA XARIO] THIKENED LV WALL (CONCENTRIC LVH) THICKENED RV WALLS (RVH) ECHO DENSED MYOCARDIUM (GRANULAR SPARKLING) SMALL LV CAVITY DEPRESSED LV SYSTOLIC FUNCTION EF (TEICH):16% EF (SIMPSON):43% EF (EST):40% DEPRESSED RV FUNCTION SMALL PERICARDIAL EFFUSSION DILATED ATRIA (LA/RA) THICKENED VALVE THICKENED ATRIAL SEPTUM THICKENED PAPILLARY MUSCLE BILATERAL PLEURAL EFFUSSION CONTRACTION WORSE FROM BASAL TO MID WALL CONTRACTION IMPROVE FROM MID TO APICAL

NOTE:PORTABLE ECHO MRI DONE

Diagnosis Confirm By Cardiac MRI

Parasternal Long Axis View

Parasternal Long Axis View

Parasternal Short Axis View At Mitral Valve Level

Parasternal Short Axis View At Mitral Valve Level

Parasternal Short Axis View At Papillary Muscle Level

Parasternal Short Axis View At Apical Left Ventricular Level

Apical 4 Chamber View

Apical 4 Chamber View

Apical 4 Chamber View Zoom

Apical 2 Chamber View

Apical 3 Chamber View

Apical Right Ventricular Inflow View

Subcostal 4 Chamber View

Expected Reduced Global Left Ventricular Systolic Strain 

With Speckle Tracking

Restrictive Filling Pattern Of Mitral Valve Inflow

Reduced Tissue Doppler Imaging Velociy