A case of atrial cardiomyopathy in a patient with rheumatic heart disease with massive left atrial calcification

Excerpt

A 60-year-old man was admitted to our hospital with progressive heart failure. He had a medical history of mitral and aortic valve replacement with bioprosthesis for long-standing rheumatic heart disease. An electrocardiogram revealed atrial fibrillation. A chest X-ray revealed cardiomegaly, pulmonary congestion, and bilateral pleural effusion. A blood test showed a high plasma brain natriuretic peptide concentration of 336 pg/ml. Transthoracic echocardiography showed dilation of the left atrium (LA) (77 mm), and the estimated systolic pulmonary pressure was 66 mmHg with severe tricuspid regurgitation. The left ventricle (LV) end-diastolic dimension was 55 mm, and the LV ejection fraction was 61%. Evaluation of LA function by two-dimensional speckle tracking echocardiography showed a significant decrease in the LA systolic strain and strain rate (Fig. 1a). Cinefluoroscopy revealed normal bioprosthetic valve motion without leaflets dysfunction. Computed tomography confirmed the severity of LA dilation and massive calcification around the LA wall (Fig. 1b). Right-sided heart catheterization revealed pulmonary hypertension with a systolic pulmonary pressure (PAP) of 95 mmHg (mean PAP 42 mmHg), pulmonary artery wedge pressure of 41 mmHg, and a dip-and-plateau pattern of the right ventricular pressure. Despite undergoing intensive treatment, the patient died of progressive heart failure. Written informed consent for autopsy was provided by the patient’s next-of-kin.

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