Treatment of Endomyocardial Fibrosis
Surgical intervention with endocardial decortication and valve replacement/repair is the definitive treatment for endomyocardial fibrosis, as medical therapy alone has no curative benefit and the disease carries a very poor prognosis without surgery. 1, 2
Medical Management: Temporizing Only
Medical therapy for endomyocardial fibrosis (EMF) serves only to mitigate symptoms temporarily and does not alter the disease course or provide curative benefit. 2 Standard heart failure management with diuretics and supportive care can provide symptomatic relief but should be viewed as a bridge to definitive surgical treatment rather than standalone therapy. 1, 2
Surgical Treatment: The Definitive Approach
Indications for Surgery
Surgery should be considered for all patients with EMF who are symptomatic (NYHA functional class III or IV), which represents the vast majority of patients at presentation. 3, 4 The procedure should not be delayed, as untreated EMF has a very poor prognosis and early surgical intervention may improve survival. 2, 5
Surgical Technique Components
The surgical approach combines three key procedures performed during cardiopulmonary bypass: 3, 4
- Endocardiectomy: Removal of the fibrotic endocardium from the right ventricle, left ventricle, or both depending on disease distribution 3, 4
- Valve replacement: Required in most cases due to severe atrioventricular valve regurgitation (tricuspid and/or mitral valve replacement) 6, 3
- Valve repair: Possible in select cases, particularly in younger patients with less severe valvular involvement 6, 3
Expected Outcomes
Surgical mortality ranges from 16-20% in experienced centers, with most deaths occurring from low cardiac output in the postoperative period. 3, 4 Among survivors, functional improvement occurs in approximately 96% of patients, with most moving to NYHA class I or II. 3, 4 Late mortality (5-17%) is primarily valve-related. 3, 4
Critical Pitfalls to Avoid
Do not rely on medical management alone, as this approach has no curative benefit and the natural history of untreated EMF is uniformly poor. 2 The disease predominantly affects children and young adults living in extreme poverty in tropical regions, and delayed surgical referral significantly worsens outcomes. 2, 5
Recognize that complete heart block is a potential surgical complication, particularly with right ventricular endocardiectomy, though technical modifications have reduced this risk substantially. 3 Modern surgical techniques have essentially eliminated this complication when performed by experienced teams. 3
Disease-Specific Context
EMF presents as restrictive cardiomyopathy with characteristic ventricular fibrosis causing diastolic dysfunction and severe atrioventricular valve regurgitation. 1, 5 The diagnosis is confirmed by echocardiography showing endocardial thickening (mean 3000 µm), restrictive filling patterns, and valve regurgitation, with findings correlating well with surgical and histopathologic examination. 6
Endomyocardial biopsy can confirm the diagnosis when EMF is suspected on clinical grounds (eosinophilia >1500/μL, geographic origin from endemic regions, restrictive physiology), though the procedure should only be performed when results will meaningfully affect therapeutic decisions. 1
Prognosis and Long-Term Management
Surgery should be considered palliative rather than curative, as recurrence of endocardial fibrosis remains possible and patients require lifelong valve prosthesis management. 4 However, operation remains the treatment of choice because: 4
- EMF has a grave prognosis with ineffective medical therapy alone
- Only the heart is primarily affected (other organ involvement is secondary to passive congestion)
- Systolic function is usually only mildly depressed
- The surgical procedure, while complex, yields acceptable mortality in experienced centers
Early surgical intervention is critical, as outcomes are better when surgery is performed before severe decompensation occurs. 2, 5 Access to surgical care remains limited in most EMF-endemic regions, representing a major barrier to improving survival. 2