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Rapid progression of right ventricular dysfunction: a case report
Guoliang Li1, Changying Zhao2, Lingmin Wu3
1Department of Cardiovascular Medicine, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
Insights
Arrhythmogenic cardiomyopathy (ACM) can rapidly progress, suggesting a possible link to myocarditis. This inflammation may drive the transformation of heart tissue in some ACM patients.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Arrhythmogenic cardiomyopathy (ACM) is a genetic heart muscle disease.
- It involves myocyte loss and fibrofatty tissue replacement, leading to arrhythmias and impaired function.
- The connection between ACM and myocarditis is increasingly recognized.
Purpose of the Study:
- To investigate the potential role of myocarditis in rapid ACM progression.
- To explore the inflammatory mechanisms underlying ACM pathogenesis.
Main Methods:
- Case presentation of a 28-year-old female with advanced biventricular ACM.
- Electrocardiogram (ECG) and echocardiogram for diagnosis and monitoring.
- Histopathological examination of heart tissue post-transplantation.
Main Results:
- The patient presented with edema and palpitations, showing advanced biventricular ACM on ECG.
- Despite treatment, right ventricle (RV) function rapidly declined, requiring heart transplantation.
- Postoperative analysis revealed significant lymphocytic infiltration, particularly in the RV.
Conclusions:
- Rapid ACM progression may indicate concomitant myocarditis.
- Myocarditis could be a factor in the fibrofatty transformation of heart tissue in ACM.
- Inflammation may be integral to the natural history of some ACM cases.
Background:
Arrhythmogenic cardiomyopathy (ACM) is a genetic myocardial disease characterized by progressive myocyte loss and fibrofatty (fibrous and adipose) tissue replacement to predispose these patients to fatal ventricular arrhythmias and impairment of ventricular systolic function. The relationship of ACM and myocarditis has gained significant attention.
Case Presentation:
This case presented a 28-year-old female who was admitted to the hospital with complaints of recurrent lower limb edema and palpitations for 6 months. Her electrocardiogram revealed a typical manifestation of an advanced form of biventricular arrhythmogenic cardiomyopathy (ACM). Despite systematic medical management, her right ventricle (RV) function deteriorated rapidly, necessitating heart transplantation. Postoperative histopathological examinations confirmed the RV involvement as reflected in the electrocardiogram. Especially, multiple foci of lymphocytic infiltration were observed throughout the heart, with the RV being the most severe.
Conclusion:
When a rapid progression of ACM occurs, a concomitant myocarditis should be considered. ACM may be an inflammation-mediated transformation from myocardial tissue to fibrofatty tissue, and myocarditis may be a part of the natural history in some ACM cases.
