Endocardial calcification of left atrium, tracheobronchopathia osteoplastica, and calcified aortic arch in a patient with dyspnea.

A 69-year-old female patient was admitted to our hospital for further cardiac evaluation with symptoms of dyspnea (New York Heart Association class III) and peripheral edema. Her history revealed artificial mitral valve replacement due to mitral stenosis 8 years earlier, severe chronic pulmonary hypertension, and severe tricuspid valve incompetence. Electrocardiogram showed normofrequent atrial fibrillation. Transthoracic echocardiography indicated moderate biatrial enlargement, although both ventricles had normal dimensions and normal global systolic function. The transmitral prosthetic valve gradient was 14/6 mm Hg. We documented severe tricuspid regurgitation and only mild mitral regurgitation. Nevertheless, echocardiography was difficult to perform because of lack of an adequate acoustic window and metal artifacts of the mitral valve prosthesis. Reflections at the epicardial surface close to the mitral valve were therefore interpreted as calcifications of pericardial layer, and constrictive pericarditis was expected to be the most likely cause of the patient’s symptoms and diastolic left ventricle dysfunction. By right heart catheterization, severe postcapillary hypertension was confirmed (systolic/diastolic/mean pulmonary artery pressure, 65/23/43 mm Hg; mean pulmonary wedge pressure, 29 mm Hg). In all cardiac cavities, an end-diastolic pressure equilibration was measured (Figure 1). Pressure curves did not show a dip-plateau phenomenon. Therefore, constrictive pericarditis was excluded. However, in a 64-row multidetector computed tomography scan, the endocardial layer of the left atrium (LA) and the aortic arch were heavily calcified (Figure 2), whereas the pericardium itself did not show any relevant calcifications. As an additional finding, the patient showed tracheobronchopathia osteoplastica (Figure 2A and 2B). Cardiac MRI was performed to further assess ventricular and atrial function (Figure 3). Any acute or persistent cardiac inflammation or interstitial storage cardiomyopathy could be excluded by edema-sensitive T2weighted short T1 inversion recovery (STIR) images and T1-weighted images early and late after intravenous GdDTPA according to the protocol previously described.1 The