Polymorphism of the clinical picture

Area of Science:

• Cardiology
• Pathology

Background:

• A growing body of evidence suggests a specific cardiomyopathy characterized by right ventricular (RV) involvement.
• This condition presents with unique segmental wall motion abnormalities and adipose or fibro-adipose tissue infiltration.

Observation:

• The spectrum of clinical conditions associated with this RV cardiomyopathy ranges from malignant ventricular tachyarrhythmias to precordial pain without arrhythmias.
• Frequent premature ventricular beats (PVBs), often originating in the RV, and T-wave abnormalities are common features.
• In milder forms, physical examination and non-invasive tests are frequently unremarkable.

Findings:

• Diagnosis in subtle cases relies heavily on right ventriculography and RV biopsy, though criteria are not fully established.
• The condition involves infiltration of adipose or fibro-adipose tissue within the RV myocardium.
• Peculiar segmental wall motion abnormalities are a hallmark of this RV cardiomyopathy.

Implications:

• The diagnostic dilemma often necessitates invasive investigations to confirm the condition.
• Understanding this cardiomyopathy is crucial for managing patients with unexplained arrhythmias or chest pain.
• Further research is needed to refine diagnostic criteria and non-invasive assessment methods.