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Start free with EleplanTropical endomyocardial fibrosis
ORPHA:75565Disease
Also called Davies disease · TEMF
What it is
Tropical endomyocardial fibrosis is a restrictive cardiopathy, occuring almost exclusively in children and young adults in tropical and subtropical regions, characterized by endocardial fibrosis, affecting the apices and the inflow tract of the right or left ventricle (or both) and manifesting with a restrictive cardiomyopathy and atrioventricular regurgitation leading to severe pulmonary hypertension, very high systemic venous pressure and congestive cardiac failure. Suspected etiologies include helminth and protozoal infestation and malnutrition.
Key facts
- Age of onset
- Adolescent, Adult, Childhood
- Inheritance
- Not applicable
- Classified as
- Disease
Signs and symptoms
Common30–79%
21- Abnormal ST segment
- Arrhythmia
- Ascites
- Atrioventricular block
- Cachexia
- Cardiomegaly
- Decreased QRS voltage
- Fever
- Hepatomegaly
- Hypoalbuminemia
- Left atrial enlargement
- Left bundle branch block
- Left ventricular diastolic dysfunction
- Malnutrition
- Orthopnea
- Peripheral edema
- Prolonged QRS complex
- Pulmonary venous hypertension
- Quadruple gallop rhythm
- Right bundle branch block
- Systolic heart murmur
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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