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Chronic inflammatory cardiomyopathy: diagnosis, management and long-term treatment – a case report
Session:
Prémio Melhor Caso Clínico
Speaker:
Débora Da Silva Correia
Congress:
CPC 2026
Topic:
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Theme:
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Subtheme:
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Session Type:
Sessão de Prémios
FP Number:
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Authors:
Débora da Silva Correia; Raquel Montalvão; Rui Gomes; Rita Carvalho; Rita Amador; Mariana Paiva; Francisco Albuquerque; Pedro Lopes; Christopher Strong; Sérgio Maltês; Bruno Rocha
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Introduction:</strong> Chronic inflammatory cardiomyopathy (ICM) is an uncommon cause of heart failure (HF), characterized by persistent immune-mediated inflammation leading to myocyte injury and ventricular remodeling. Diagnosis requires an integrated approach using biomarkers, multimodality imaging, and, in selected cases, endomyocardial biopsy (EMB), which remains as the gold standard to confirm active inflammation and to guide immunosuppressive therapy.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Case Description:</strong> A 79-year-old woman with a history of hypertension and dyslipidemia was evaluated for pre-syncope and mild fatigue. Initial transthoracic echocardiography (TTE) revealed a left ventricular ejection fraction (LVEF) ~25% with diffuse hypokinesia. With subsequent clinical deterioration, she was hospitalized for <em>de novo</em> HF and markedly elevated NT-proBNP levels (36,523 pg/mL). Guideline-directed medical therapy for HF was initiated. After discharge, cardiac magnetic resonance (CMR) showed severe biventricular dysfunction and tissue characterization suggestive of ICM. EMB was undertaken, revealing active inflammatory cardiomyopathy with T-lymphocyte and macrophage infiltration, cardiomyocyte necrosis, and interstitial fibrosis. Polymerase chain reaction analysis for RNA/DNA common cardiotropic virus on biopsy samples was negative.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Despite optimized HF therapy, repeat TTE showed persistently reduced LVEF. Based on the combined findings of clinical history, EMB, TTE and CMR, a presumptive diagnosis of chronic ICM was established, and immunosuppressive therapy with oral prednisolone (0.5 mg/kg/day) was initiated with appropriate infection prophylaxis, resulting in partial improvement (LVEF 39%). However, follow-up CMR after four weeks demonstrated an incomplete imaging response, with persistent inflammatory activity. Immunosuppression was then intensified with IV methylprednisolone pulses (1g/day for 3 consecutive days), followed by gradual taper until 0.5 mg/kg/day.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">The patient had significant clinical improvement (NYHA I). Follow-up imaging with TTE demonstrated normalization of left ventricular volumes (LVEDVi 110 to 41 mL/m²) and substantial improvement in systolic function (LVEF 50%; and global longitudinal strain from −4.8% to −11.6%). Repeat CMR confirmed complete resolution of the myocardial inflammation (normalization of T1 and T2 mapping values) and recovery of biventricular function, leaving only minimal residual focal fibrosis.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Conclusion:</strong> This case illustrates the importance of EMB in unexplained cardiomyopathy and highlights the potential reversibility of chronic ICM with timely and appropriately intensified immunosuppressive therapy. Multimodality imaging played a pivotal role in diagnosis, therapeutic guidance, and follow-up, emphasizing that even severe ventricular dysfunction may substantially recover after appropriate management.</span></span></p> <p> </p>
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