A Comparison of the Clinical, Haemodynamic and Angiographic Features in Right Ventricular Endomyocardial Fibrosis and Ebstein’s Anomaly of the Tricuspid Valve

Abstract

The clinical, haemodynamic and angiographic features of 18 patients with right ventricular endomyocardial fibrosis (RVEMF) and 8 patients with Ebstein’s anomaly of the tricuspid valve (EATV) have been compared. Diagnosis was confirmed by selective angiography. The position of the tricuspid annulus was identified from selective right ventricular angiograms and confirmed by selective right coronary angiography. In 83% of RVEMF patients the tricuspid annulus was displaced to the left of the spine. A false impression of displacement of the tricuspid leaflet can thus be created. However, a tricuspid leaflet displaced away from the tricuspid annulus was found only in patients with EATV.A considerable overlap exists between the wide spectrum of clinical presentations of the two conditions. Helpful distinguishing features that favour EATV were, the presence of a scratchy diastolic murmur and polyphasic QRS complexes in the ECG. Atrial fibrillation in the ECG, and myocardial calcification or pericardial effusion, whenever present, favour RVEMF.

Cardiovascular magnetic resonance evidence of myocardial fibrosis and its clinical significance in adolescent and adult patients with Ebstein's anomaly

“…The typical LGE pattern found in this study was mostly localized in the endocardium of the aRV septum. This finding was in accordance with a previous case report [ 36 ]. In one case, mid myocardial wall LGE (non-ischemic pattern) was observed in the basal to mid septum.…”

Tapioca Cardiomyopathy: Curse of Cassava Endomyocardial Fibrosis

“…JNP Davies first coined the term EMF. The triad of elevated JVP, ascites and hepatomegaly formed the hallmark of RV EMF, which can present with cyanosis and clubbing because of stretch opening of foramen ovale [ 2 ]. Mild cardiomegaly, loud left ventricular third heart sound, short systolic murmur and severe pulmonary hypertension formed the hallmark for the diagnosis of left ventricular EMF.…”

Restrictive cardiomyopathy in India: the story of a vanishing mystery

“…Occasional patients may have cyanosis with digital clubbing because of stretched open foramen ovale. This type of presentation should be differentiated from Ebstein’s anomaly and the former from constrictive pericarditis 20 21. Clinical presentation of the disorder may vary from asymptomatic incidental detection to cardiac cachexia.…”