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Infiltrative Cardiomyopathies
David Bejar1, Paolo C Colombo1, Farhana Latif1
1Division of Cardiology, Columbia University Medical Center, New York, NY, USA.
Insights
Infiltrative cardiomyopathies, often inherited or acquired, can cause severe heart problems. Early diagnosis of conditions like glycogen storage disease is crucial for potentially curative treatment and improved prognosis.
Area of Science:
- Cardiology
- Cardiovascular Medicine
- Infiltrative Diseases
Background:
- Infiltrative cardiomyopathies encompass diverse inherited and acquired conditions.
- These diseases often lead to adverse prognoses, heart failure, and arrhythmias.
- Early diagnosis is key for potentially curative interventions in specific cases like glycogen storage disease.
Purpose of the Study:
- To review the spectrum of infiltrative cardiomyopathies.
- To highlight diagnostic approaches and treatment strategies.
- To emphasize the importance of clinical suspicion for rare cardiac conditions.
Main Methods:
- Review of existing literature on infiltrative cardiomyopathies.
- Discussion of diagnostic tools from electrocardiography to advanced imaging.
- Analysis of treatment modalities including medical, device, and transplant options.
Main Results:
- Infiltrative cardiomyopathies cause ventricular wall thickening, chamber dilatation, and conduction system disruption.
- Common consequences include heart failure, atrioventricular block, and ventricular arrhythmias.
- Cardiac amyloidosis serves as a primary example of these conditions.
Conclusions:
- A high index of clinical suspicion is vital for diagnosing rare infiltrative cardiomyopathies.
- Advanced imaging like cardiac magnetic resonance (CMR) and nuclear imaging are increasingly important.
- Treatment is etiology-dependent, ranging from medications to organ transplantation.
Abstract:
Infiltrative cardiomyopathies can result from a wide spectrum of both inherited and acquired conditions with varying systemic manifestations. They portend an adverse prognosis, with only a few exceptions (ie, glycogen storage disease), where early diagnosis can result in potentially curative treatment. The extent of cardiac abnormalities varies based on the degree of infiltration and results in increased ventricular wall thickness, chamber dilatation, and disruption of the conduction system. These changes often lead to the development of heart failure, atrioventricular (AV) block, and ventricular arrhythmia. Because these diseases are relatively rare, a high degree of clinical suspicion is important for diagnosis. Electrocardiography and echocardiography are helpful, but advanced techniques including cardiac magnetic resonance (CMR) and nuclear imaging are increasingly preferred. Treatment is dependent on the etiology and extent of the disease and involves medications, device therapy, and, in some cases, organ transplantation. Cardiac amyloid is the archetype of the infiltrative cardiomyopathies and is discussed in great detail in this review.
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