Abstract

Abstract Background Pericardial and pleural effusion are common findings in patients with cardiac amyloidosis (CA). While this might be suggestive of a sign of right heart failure, it is not known, whether effusions correlate with right ventricular (RV) function in patients with CA. Furthermore, data on the prognostic significance of pleural and pericardial effusion in CA is scarce. Methods Patients with cardiac transthyretin (ATTR) and light chain (AL) amyloidosis were included in a clinical registry at our dedicated CA outpatient clinic. CA was diagnosed according to current guidelines. All patients underwent a comprehensive transthoracic echocardiography (TTE) exam at the time of study inclusion. The TTE protocol included standard and advanced parameters describing left ventricular and RV function, including speckle-tracking imaging to assess global longitudinal left ventricular and RV free wall (RV-FW) strain. The presence of pericardial and pleural effusion was determined in every patient and was verified by cardiac magnetic resonance imaging when present. The size of pericardial effusion was measured at the point of its maximum extension. The clinical endpoint was defined as all-cause death. Results Between March 2012 and February 2019, 177 patients were included in our CA registry, however, TTE image quality was only sufficient for analysis in 143 patients. Of these, 83 patients (59.4%) were diagnosed with ATTR and 60 patients (42.0%) with AL. In total, 23 patients (16.1%) presented with isolated pericardial effusion and 36 (25.2%) with isolated pleural effusion. In 17 patients (11.9%) both pericardial and pleural effusion were found and in 66 patients (46.2%) no effusion was present at baseline. Overall, there was no significant difference in the type of effusion between patients with AL and ATTR. In general, pericardial effusions were small (median diameter 7.1 mm (IQR 5.2–9.7) and none were hemodynamically compromising. Interestingly, the presence of pleural effusion correlated well with poor RV function, measured by RV-FW strain (p=0.034). However, no such correlation could be found for pericardial effusion and RV-FW strain (p=0.319). Kaplan Meier analysis showed that patients presenting with pleural effusion had a worse prognosis compared to patients with pericardial effusion alone or no effusion at baseline (p=0.001, Figure 1). Figure 1 Conclusion More than 50% of patients with CA presented with pleural and/or pericardial effusions. While pleural effusion was clearly associated with poor RV function, we were not able to detect this association with pericardial effusion. In addition, patients with pleural effusions had an exceptionally poor prognosis compared to patients with pericardial effusions or no effusion at baseline.

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