Right ventricular endomyocardial fibrosis – echocardiographic profile with video

Right ventricular endomyocardial fibrosis – echocardiographic profile with video

Echocardiogram with narration:

Echocardiogram without narration:


Endomyocardial fibrosis is type of restrictive cardiomyopathy seen mostly in the tropics and sub tropics. There is progressive fibrosis and obliteration of the right ventricle, predominantly the inflow tract and the apex. The right ventricular outflow tract (RVOT) is relatively spared and is often dilated. This leads to the physical finding of RV outflow pulsations, a wavy pulsation in the third left intercostal space close to the sternum. The restriction of inflow into the right ventricle elevates the venous pressure greatly. The right atrium is grossly dilated. Features of systemic venous congestion predominate in advanced cases with hepatomegaly, ascites and generalized edema.

Echocardiogram in right ventricular endomyocardial fibrosis
Echocardiogram in right ventricular endomyocardial fibrosis

Echocardiogram in apical four chamber view demonstrating salient features of right ventricular endomyocardial fibrosis. The right atrium is grossly dilated and the interatrial septum is pushed to the left. The left ventricle and left atrium are normal, though the left atrium appears compressed by the huge right atrium. Right ventricular cavity is very small, seen just distal to the tricuspid valve, beyond the right atrium. Dense fibrosis with calcification of right ventricular cavity and apex is the hallmark of endomyocardial fibrosis. A corresponding dimple will be seen near the apex on the surface. Clinically the grossly enlarged right atrium can be percussed out to the right of the sternal border.

Echocardiogram in right ventricular endomyocardial fibrosis with tricuspid regurgitation
Echocardiogram in right ventricular endomyocardial fibrosis with tricuspid regurgitation

Colour Doppler evaluation demonstrates significant tricuspid regurgitation (TR). Doppler interrogation will reveal that it is a low velocity TR, unlike in pulmonary hypertension, where the TR velocity is high. A high velocity TR jet can be seen in left ventricular endomyocardial fibrosis (LVEMF) with pulmonary hypertension or if there is associated mitral stenosis (see below). Even though the TR in this case is low velocity, it shows aliasing and variance with multiple colors (mosaic) because the Nyquist limit of the color Doppler is set at 60 cm / sec (see the color bar at the top right corner) which corresponds to a gradient of only 1.44 mm Hg. That means aliasing will occur if the gradient is just above 1.44 mm Hg.

Right atrial thrombus in right ventricular endomyocardial fibrosis
Right atrial thrombus in right ventricular endomyocardial fibrosis

The grossly dilated right atrium causes stasis of blood in the right atrium. This often causes thrombus formation in the right atrium as illustrated above. The thrombus can be either mobile or adherent to the atrial wall. A mobile thrombus can embolize producing pulmonary embolism and its sequelae, sometime presenting as pulmonary hypertension. Right ventricular apical fibrosis is evident in the right panel. In this case, the involvement of right ventricular cavity is lesser than in the previous case which has a documented history of over 20 years.

X-ray chest PA view in right ventricular endomyocardial fibrosis with pericardial effusion
X-ray chest PA view in right ventricular endomyocardial fibrosis with pericardial effusion

X-ray chest in an advanced case of right ventricular endomyocardial fibrosis often shows gross enlargement of the cardiac silhouette, predominantly contributed by the right atrial enlargement. But it can also be partly due to associated pericardial effusion as in this case. Varying degrees of pericardial effusion is a common association of severe right ventricular endomyocardial fibrosis with systemic venous congestion and anasarca.

X-ray chest PA view in right ventricular endomyocardial fibrosis and rheumatic mitral stenosis
X-ray chest PA view in right ventricular endomyocardial fibrosis and rheumatic mitral stenosis

Features of pulmonary arterial hypertension in endomyocardial fibrosis (EMF) could be either due to left ventricular involvement and consequent elevation of left atrial and pulmonary venous pressures or due to pulmonary embolism from a right atrial thrombus as demonstrated earlier. But the x-ray shown above shows pulmonary venous hypertension (prominent upper lobe veins), bulging of the left atrial appendage and prominence of main pulmonary artery. Though this could be due to associated left ventricular EMF, but in this case there was a history of previous closed mitral valvotomy for rheumatic mitral stenosis, now presenting with restenosis. The presence of associated right ventricular endomyocardial fibrosis was documented by echocardiography. Another possibility for explaining the right atrial enlargement in this case is mitral stenosis with tricuspid valve disease, both of rheumatic etiology.

X-ray chest PA view in left ventricular endomyocardial fibrosis
X-ray chest PA view in left ventricular endomyocardial fibrosis

Finally and X-ray chest PA view in left ventricular EMF, mimicking and X-ray in mitral stenosis, with prominent left atrial appendage, double atrial contour (shadow in shadow), right atrial enlargement, pulmonary venous congestion and main pulmonary artery prominence. Echocardiography showed that there was LVEMF and no mitral stenosis. Pulmonary arterial hypertension was also present.

Treatment of EMF is restricted to symptomatic measures to relieve pulmonary and venous congestion as the case may be. Severe ascites may require periodic paracentesis to relieve tension and give symptomatic relief. Endocardiectomy and mitral / tricuspid valve replacements were being done earlier. Prosthetic valve thrombosis is an important complication of tricuspid valve replacement. They present with worsening of systemic venous congestion and ascites.

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