A rare cause of cardiac decompensation in a patient with left ventricular hypertrophy

Abstract

Amyloidosis, a disease characterized by the deposition of extracellular proteinaceous material known as amyloid, commonly involves more than one organ system. When cardiac amyloidosis is the isolated or even the predominant manifestation of undiagnosed multiorgan amyloidosis, diagnose is often not confirmed until the late course of disease [1]. The different types of amyloidosis, e.g., AL, senile, familial or secondary amyloidosis, progress at different speeds, but if cardiac amyloidosis is present, the prognosis is determined by cardiac symptoms and complications [1, 2]. A 72-year-old woman was admitted with progressive dyspnoea on exertion during the last month (NYHA class III), unspecific chest discomfort and recurrent syncope (twice). Her previous history included cardiac surgery in 2005 with bioprosthetic aortic valve replacement, mitral valve repair, and aortocoronary bypass grafting (LIMA bypass to the LAD and single vein graft to the right coronary artery). The electrocardiogram documented bradycardic atrial fibrillation about 50/min, lost R-wave in V1–V4 with persistent ST-elevation in V1–V3 and T-wave inversion in I, II, III, aVF as well as V5–V6. Echocardiography confirmed normal aortic valve prosthesis and a mild regurgitation of the reconstructed mitral valve with adequate mitral valve opening area. However, a remarkable concentric left ventricular hypertrophy was detected (left ventricular mass index, 181 g/m; relative wall thickness, 0.8). The myocardial texture showed a striking ‘‘speckled’’ appearance involving both papillary muscles (Fig. 1a, b). Systolic and diastolic function was impaired in both the left and the right ventricle: The left ventricular ejection fraction (LV-EF 40 %) and peak systolic strain were significantly impaired. Right ventricular function as measured by tricuspid annular plane systolic excursion (TAPSE: 8 mm) was reduced. Echocardiographic examination of diastolic function pointed to elevated LV-enddiastolic pressures under the circumstances of atrial fibrillation: Emax. 1.8, deceleration time 144 ms, E/Em at lateral mitral annulus 25.9, E/Em at septal mitral annulus 53.3. An inconspicuous pericardium, the lack of a breathdependent flow pattern of the mitralor tricuspid valve inflow and higher lateral Em (7 cm/s) than septal Em (3 cm/s) made the diagnosis of constrictive pericarditis unlikely. For further tissue characterization, we performed cardiac magnetic resonance imaging (CMR) including late gadolinium enhancement (LGE). CMR confirmed left ventricular hypertrophy (Fig. 1c), atrial enlargement, reduced left ventricular ejection fraction and demonstrated bilateral pleural effusion. No focal or generalized edema, but increased global relative enhancement was present. Furthermore, LGE demonstrated a diffuse enhancement (Fig. 1d). It was impossible to perfectly null the left Electronic supplementary material The online version of this article (doi:10.1007/s00392-012-0478-5) contains supplementary material, which is available to authorized users.