Objective: restrictive cardiomyopathy (RCM) is characterized by impairment of ventricular filling during diastole with preserved systolic function. The clinical and histopathological profile on endomyocardial biopsy of 25 consecutive patients with RCM was analyzed in this study.
Methods: twenty-five patients with diagnosis of RCM and underwent endomyocardial biopsy (EMB) were enrolled in the study. The clinical characteristics, electrocardiogram, serum chemistry, right heart catheter and cardiac pathology results were obtained.
Results: heart failure symptom was present in all 25 patients and left ventricular size and function were normal or near normal while serum brain natriuretic peptide (577 pg/ml) was moderately elevated. Right atrial and ventricular end-diastolic as well as pulmonary capillary wedge pressures derived from right heart catheter examination were increased. Amyloid deposition were evidenced in 16 and eosinophilic myocarditis in 2 patients upon pathological examination of EMB. In the remaining 7 patients, 3 were diagnosed idiopathic RCM, 2 were diagnosed as amyloidosis by biopsy from non-cardiac tissue and etiology remained unknown in 2 patients. Thus, conclusive diagnosis was made on EMB samples in 84% (21/25) patients of RCM.
Conclusion: RCM may result from various local and systemic disorders. EMB is helpful for identifying the underlying etiology.
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Cureus
December 2024
Internal Medicine, Weiss Memorial Hospital, Chicago, USA.
A previously healthy, 28-year-old man presented with a two-day history of diarrhea and chest pain, suggestive of infectious myocarditis. Initial workup revealed elevated troponin-I levels and diffuse ST-segment elevations on electrocardiogram (ECG). Transthoracic echocardiography showed a reduced left ventricular ejection fraction (40-45%), posteroinferior wall akinesis, and a small pericardial effusion.
View Article and Find Full Text PDFCardiovasc Pathol
December 2024
Chazov National Medical Research Center of Cardiology, 121552, Academician Chazov str., 15a, Moscow, Russian Federation.
Aim: to assess the relation of focal and diffuse left ventricular (LV) fibrosis to left bundle branch block (LBBB).
Materials And Methods: 60 patients with dilated cardiomyopathy and LBBB (DCM-LBBB), 50 DCM-nonLBBB patients, 15 patients with LBBB and structurally normal heart (idiopathic LBBB) and 10 healthy volunteers (HV) underwent cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE). LGE LV images were post-proceed for core scar (CS) and gray zone (GZ) calculation.
Respir Med Case Rep
November 2024
Department of Pulmonary and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA.
Ochsner J
January 2024
Division of Cardiology, Baylor Scott & White Medical Center, Temple, TX.
Waldenström macroglobulinemia is a rare cancer of plasma cells characterized by the excessive production of immunoglobulin M (IgM). IgM-associated systemic amyloid light chain (AL) amyloidosis is a rare complication of Waldenström macroglobulinemia, characterized by the misfolding of lambda light chains that deposit in various organs, including the heart. We describe a case of progressive nonischemic cardiomyopathy secondary to Waldenström macroglobulinemia and IgM-associated AL amyloidosis that was refractory to medical therapy and highlight the challenges in diagnosis and management.
View Article and Find Full Text PDFSAGE Open Med Case Rep
December 2024
Department of Pediatrics, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, AB, Canada.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic necrotizing vasculitis marked by eosinophilia and extravascular granulomas, predominantly affecting the respiratory tract. This report details a unique EGPA case in a 6-year-old girl with extensive cardiac involvement, featuring an atypical intracardiac mass suggestive of endomyocardial fibrosis and a concomitant thrombus. The clinical course unfolded in three phases: an initial prodrome with asthma; subsequent peripheral hypereosinophilia; and ultimately systemic vasculitis.
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