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Atypical left bundle branch block in dilative "burned-out" phase of hypertrophic cardiomyopathy
Juan Benezet-Mazuecos1, Borja Ibanez, Jeronimo Farre
1Servicio de Cardiología, Fundación Jiménez Díaz, Universidad Autónoma de Madrid, Madrid, Spain. jbenezet@yahoo.es
Insights
This case study highlights a rare instance of hypertrophic cardiomyopathy (HC) progressing to an end-stage, or "burned-out," phase. Unusual electrocardiogram (ECG) findings may signal this advanced stage of heart failure.
Area of Science:
- Cardiology
- Internal Medicine
- Pathophysiology
Background:
- Hypertrophic cardiomyopathy (HC) is a genetic heart condition.
- Left ventricular remodeling is a key feature in HC pathophysiology.
- Some HC patients paradoxically develop left ventricular wall thinning and systolic dysfunction.
Observation:
- A 70-year-old male presented with congestive heart failure.
- The patient had a 22-year history of hypertrophic cardiomyopathy.
- Electrocardiogram (ECG) revealed a unique left bundle branch block (LBBB).
- 2D-echocardiogram showed a dilated left ventricle with a 25% ejection fraction.
Findings:
- The patient's presentation represents the rare "end-stage" or "burned-out" phase of HC.
- This phase is characterized by systolic dysfunction and heart failure, even without coronary artery disease.
- The specific LBBB pattern observed is a unique manifestation of this advanced HC stage.
Implications:
- ECG changes, particularly rare LBBB patterns, may serve as early indicators of the "burned-out" phase in HC.
- Understanding these ECG alterations can aid in early detection and management of advanced hypertrophic cardiomyopathy.
- Further research into the mechanisms of left ventricular wall thinning in HC is warranted.
Abstract:
We present the case of a 70-year-old man admitted in congestive heart failure. The patient was diagnosed 22 years ago of hypertrophic cardiomyopathy (HC). ECG showed a very peculiar and pathological form of left bundle branch block (LBBB). 2D-echocardiogram revealed a dilated left ventricle (LV) and ejection fraction of 25%. LV remodeling represents an important component of the pathophysiology of HC and, paradoxically, some patients develop LV wall thinning, systolic dysfunction, and congestive heart failure (in the absence of coronary artery disease). This evolution is designated as "end-stage" or "burned-out"phase. We present this rare LBBB and his pathological evolution along the time as unique manifestation of this "burned-out" phase. The mechanism of this wall thinning remains unclear but changes in ECG may alert us about it.
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