Isolated right ventricular infarction with massive dilation: acquired Uhl's disease?

Area of Science:

• Cardiology
• Pathology

Background:

• Right ventricular infarction is less common than left ventricular infarction.
• Uhl's disease is a rare congenital condition characterized by the absence of normal right ventricular myocardium.

Observation:

• A patient presented with extensive isolated right ventricular infarction.
• The infarction resulted from proximal occlusion of a small right coronary artery within a dominant left circumflex system.
• Minimal left-sided coronary artery disease was noted.

Findings:

• The patient's condition presented with clinical and morphologic features similar to healed right ventricular infarction and Uhl's disease.
• The sequence of events suggests a potential link between coronary artery occlusion, myocardial infarction, and the development of a parchment-like right ventricle.
• Right ventricular myocardial damage may lead to chamber dilation and morphologic changes resembling Uhl's anomaly.

Implications:

• This case highlights a potential, previously unrecognized association between myocardial infarction and the "parchment heart" appearance.
• It expands the differential diagnosis for conditions presenting with right ventricular abnormalities.
• Further research is warranted to explore the pathophysiological mechanisms linking infarction to these specific morphologic changes.