Overview
Endomyocardial fibrosis (EMF) is a rare clinicopathological condition characterized by fibrotic thickening of the endocardium, often leading to restrictive cardiomyopathy and valvular dysfunction, predominantly observed in tropical regions though sporadic cases occur globally 4.Diagnosis
Clinical Presentation: Exertional breathlessness, orthopnea, syncope, and recurrent symptoms 13.
Diagnostic Imaging: Echocardiography may be limited by acoustic shadowing; chest CT provides better visualization of calcification extent 1.
Electrocardiography: Serial ECGs can show minor changes indicative of disease progression 5.
Laboratory Findings: Blood eosinophilia is common in sporadic cases 4.Management
First-Line Treatments: Diuretics, angiotensin-converting enzyme inhibitors, and beta-blockers 1.
Surgical Interventions: Multiple operative procedures may be required for aggressive or recurrent cases 3.
Specific Therapies: No specific curative treatment exists; management focuses on symptom relief and complications 1.Special Populations
Pediatrics: Rare association with congenital bleeding disorders like Glanzmann thrombasthenia 2.
Elderly: Poor prognosis with rapid progression to organ failure 1.
Comorbidities: EMF can occur secondary to chemotherapy, e.g., daunorubicin therapy 6.Key Recommendations
Utilize chest CT for accurate assessment of endocardial calcification extent in diagnosis 1 (Evidence: Moderate).
Implement supportive care with diuretics, ACE inhibitors, and beta-blockers for symptom management 1 (Evidence: Weak).
Consider surgical intervention for recurrent or aggressive forms necessitating multiple procedures 3 (Evidence: Weak).References
1 Yuan Y, Li Y, Xu Z. A case presentation of patient from northern China with endomyocardial fibrosis. BMC cardiovascular disorders 2019. link
2 Tullu MS, Vaideeswar P, Pandit SP, Lahiri KR. Glanzmann thrombasthenia with endomyocardial fibrosis: a thrombotic paradox. International journal of cardiology 2007. link
3 Pyrgakis VN, Shapiro LM, Donaldson RM. Unusual presentation of endomyocardial fibrosis. International journal of cardiology 1988. link90298-7)
4 Hutt MS. Epidemiology aspects of endomyocardial fibrosis. Postgraduate medical journal 1983. link
5 Lowenthal MN. Serial electrocardiograms in endomyocardial fibrosis. Medical journal of Zambia 1978. link
6 Wilcox RG, James PD, Toghill PJ. Endomyocardial fibrosis associated with daunorubicin therapy. British heart journal 1976. link