The treatment of systemic light-chain (AL) amyloidosis with symptomatic cardiac involvement at diagnosis remains a challenge. We report the results of 40 consecutive newly diagnosed AL cardiac patients who were not candidates for stem cell transplant and therefore received monthly oral melphalan and dexamethasone. Median survival was 10.5 months and baseline predictors of survival included gender, troponin I and interventricular septal thickness. The most significant predictor of survival was response to therapy. The haematological response rate was 58% (23/40) with 13% (5/40) complete responses; most responses were noted in <3 cycles. Achievement of a rapid response to therapy extends survival.

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BackgroundThe significance of right ventricular ejection fraction (RVEF), independent of left ventricular ejection fraction (LVEF), following isolated coronary artery bypass grafting (CABG) and valve procedures remains unknown. The aim of this study is to examine the significance of abnormal RVEF by cardiac magnetic resonance (CMR), independent of LVEF in predicting outcomes of patients undergoing isolated CABG and valve surgery.MethodsFrom 2007 to 2009, 109 consecutive patients (mean age, 66 years; 38% female) were referred for pre-operative CMR. Abnormal RVEF and LVEF were considered <35% and <45%, respectively. Elective primary procedures include CABG (56%) and valve (44%). Thirty-day outcomes were perioperative complications, length of stay, cardiac re-hospitalizations and early mortaility; long-term (> 30 days) outcomes included, cardiac re-hospitalization, worsening congestive heart failure and mortality. Mean clinical follow up was 14 months.FindingsForty-eight patients had reduced RVEF (mean 25%) and 61 patients had normal RVEF (mean 50%) (p<0.001). Fifty-four patients had reduced LVEF (mean 30%) and 55 patients had normal LVEF (mean 59%) (p<0.001). Patients with reduced RVEF had a higher incidence of long-term cardiac re-hospitalization vs. patients with normal RVEF (31% vs.13%, p<0.05). Abnormal RVEF was a predictor for long-term cardiac re-hospitalization (HR 3.01 [CI 1.5-7.9], p<0.03). Reduced LVEF did not influence long-term cardiac re-hospitalization.ConclusionAbnormal RVEF is a stronger predictor for long-term cardiac re-hospitalization than abnormal LVEF in patients undergoing isolated CABG and valve procedures.

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Journal of Cardiovascular Magnetic Resonance 2009, 11(Suppl 1):O1Introduction: Prophylactic implantation of a cardioverter/ defibrillator (ICD) has been shown to reduce mortality in patients with chronic myocardial infarction (CMI) and an increased risk for life threatening ventricular arrhythmia (VA). The use of ICDs in this large patient population is still limited by high costs and possible adverse events including inappropriate discharges and progression of heart failure. VA is related to infarct size and seems to be related to infarct morphology. Contrast enhanced cardiovascular magnetic resonance imaging (ceCMR) can detect and quantify myocardial fibrosis in the setting of CMI and might therefore be a valuable tool for a more accurate risk stratification in this setting. Hypothesis: ceCMR can identify the subgroup developing VA in patients with prophylactic ICD implantation following MADIT criteria. Methods: We prospectively enrolled 52 patients (49 males, age 69 ± 10 years) with CMI and clinical indication for ICD therapy following MADIT criteria. Prior to implantation (36 ± 78 days) patients were investigated on a 1.5 T clinical scanner (Siemens Avanto © , Germany) to assess left ventricular function (LVEF), LV end-diastolic volume (LVEDV) and LV mass (sequence parameters: GRE SSFP, matrix 256 × 192, short axis stack; full LV coverage, no gap; slice thickness 6 mm). For quantitative assessment of infarct morphology late gadolinium enhancement (LGE) was performed including measurement of total and relative infarct mass (related to LV mass) and the degree of transmurality (DT) as defined by the percentage of transmurality in each scar. (sequence parameters: inversion recovery gradient echo; matrix 256 × 148, imaging 10 min after 0.2 μg/kg gadolinium DTPA; slice orientation equal to SSFP). MRI images were analysed using dedicated software (MASS © , Medis,

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e19524 Background: Patients with systemic amyloidosis involving the heart have generally a poor prognosis. Elevated cardiac troponin (cTn) has been shown to predict poor survival in patients with AL cardiac amyloidosis (CA). However, cardiac structure and function associated with an elevated cTn in patients with CA remains uncertain. Methods: Eighty patients with CA who had an echocardiogram and cTnI measured at the time of diagnosis between March 1997 May 2008 were included in the study. The patients were divided into 2 groups: normal cTnI ( <0.06 ng/ml, n = 28) or elevated cTnI (≥0.06 ng/ml; n = 52). Results: The elevated cTnI group was slightly older with higher brain natriuretic peptide (BNP) level and creatinine clearance than the normal cTnI group. There was a trend towards higher prevalence of congestive heart failure (CHF) in the elevated cTnI group than the normal group at time of diagnosis. The elevated cTnIgroup had thicker interventricular septum and higher left atrial (LA) volume than the normal group. LV ejection fraction, left atrial systolic force (LASF) and stroke volume were lower in the elevated cTnI group than the normal group. Mean survival was significantly shorter in the elevated cTnI group than the normal group (10 vs. 47 months). Conclusions: Elevated troponin in patients with AL cardiac amyloidosis is associated with worse left atrial and ventricular structure and function. The raised troponin level reflects myocardial injury due to the amyloid deposition and correlates with more abnormal alteration of cardiac structure and function. Routine measurement of troponin is useful in identifying high risk patients with CA. [Table: see text] No significant financial relationships to disclose.