Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience.
Endomyocardial fibrosis (EMF) is a fascinating disease entity of unknown etiology. It is prevalent in the tropical zone. Its essential features are the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of the inflow tract and apex of one or both ventricles. It results in endocardial rigidity, atrioventricular valve incompetence secondary to papillary muscle involvement, and progressive reduction of the cavity of the involved ventricle leading to restriction in filling and atrial enlargement. This article will present 21 patients with EMF who were initially referred to our hospital from 1979 to 2004 with different diagnoses: rheumatic heart disease with mitral and or tricuspid regurgitation (n = 9), constrictive pericarditis (n = 6), restrictive cardiomyopathy (n = 1), hypertrophic cardiomyopathy apical type (n = 2), dilated cardiomyopathy (n = 2), and Ebstein malfunction of the tricuspid valve (n = 1). The clinical, echocardiographic, hemodynamic, and angiographic findings in these 21 patients are presented; echocardiographic findings lead to the right diagnosis. The presence of a small ventricle with obliteration of the apex and large atrium shown on two-dimensional echocardiography is highly suggestive of EMF.
Hassan
Walid M
WM
Cardiology Section, King Faisal Heart Institute (MBC 16), King Faisal Specialist Hospital and Research Center, PO Box 3354, Riyadh 11211, Saudi Arabia. hassanw@kfshrc.edu.sa
Fawzy
Mohamed E
ME
Al Helaly
Sumaya
S
Hegazy
Hesham
H
Malik
Shahid
S
D000293:Adolescent; D000328:Adult; D000790:Angiocardiography / Q000379:methods; D001707:Biopsy, Needle; D015331:Cohort Studies; D003952:Diagnostic Imaging / Q000379:methods*; D004359:Drug Therapy, Combination; D004452:Echocardiography / Q000379:methods; D004562:Electrocardiography / Q000379:methods; D004719:Endomyocardial Fibrosis / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality*; D005260:Female; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D015996:Survival Rate; D014665:Vasodilator Agents / Q000627:therapeutic use
D014665:Vasodilator Agents