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'Hot phase' non-dilated left ventricular cardiomyopathy with atypical onset and recurrence: a case report
Nicola Gonano1,2, Vincenzo Nuzzi3, Daniela Pavan2
1Department of Cardiothoracovascular, Azienda Sanitaria Universitaria Integrata Giuliana Isontina (ASUGI), University of Trieste, Trieste, Italy.
Insights
This case highlights non-dilated left ventricular cardiomyopathy (NDLVC) overlapping with inflammatory heart disease (InHD). A multimodality approach is crucial for diagnosing and managing this complex cardiac condition.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Genetics
Background:
- Non-dilated left ventricular cardiomyopathy (NDLVC) is a recently defined phenotype characterized by normal left ventricular volumes.
- Inflammatory heart disease (InHD) presents heterogeneously, sometimes overlapping with NDLVC presentations.
Observation:
- A 26-year-old woman presented with complete heart block and elevated troponin, with normal echocardiography and coronary angiography.
- Cardiac magnetic resonance revealed extensive edema and late gadolinium enhancement; endomyocardial biopsy excluded active myocarditis.
Findings:
- Steroid therapy restored atrioventricular conduction, but a mild recurrence with troponin relapse occurred.
- Genetic testing was negative for mutations relevant to the patient's condition.
Implications:
- This case underscores the importance of a precise, multimodality diagnostic work-up, including genetic testing, for NDLVC with InHD.
- Early detection and treatment of recurrences are vital for patient outcomes in this challenging cardiomyopathy.
- A comprehensive approach is essential for accurate diagnosis, prognosis, and therapeutic strategies in NDLVC and InHD.
Abstract:
Non-dilated left ventricular cardiomyopathy (NDLVC) is a newly categorized cardiomyopathy phenotype includingseveral aetiologies with a linking characteristic represented by the normal left ventricular volume. Inflammatory heart disease (InHD) is a heterogeneous process with variegate clinical manifestations, sometimes in overlap with NDLVC. A 26-year-old woman was admitted forcomplete heart block (CHB) and persistently raised troponin. Echocardiography and coronary angiography were normal. Extensive oedema and late gadolinium enhancement was found at cardiac magnetic resonance. Endomyocardial biopsy showed no signs of active myocarditis. Steroid therapy was started with restoration of atrioventricular conduction but subsequently the patient experienced a mild recurrence with a new troponin relapse. Genetic test was negative for mutations related with the clinical scenario. In this case of NDLVC with InHD the precise diagnostic work-up, including genetic test, was crucial for diagnostic, prognostic andtherapeutic purposes. Multimodality approach is crucial to detect and treat possible recurrences.
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