Pericardial Effusion and Multiple Organ Involvement Are Independent Predictors of Mortality in Patients with Systemic Light Chain Amyloidosis

Yuda, Satoshi; Hayashi, Toshiaki; Yasui, Kohichiroh; Muranaka, Atsuko; Ohnishi, Hirofumi; Hashimoto, Akiyoshi; Ishida, Tadao; Tsuchihashi, Kazufumi; Shinomura, Yasuhisa; Watanabe, Naoki; Miura, Tetsuji

doi:10.2169/internalmedicine.54.3500

Cited by 16 publications

(10 citation statements)

References 41 publications

(82 reference statements)

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“…It is, therefore, suggested in the current guidelines as a standard assessment in the evaluation of patients with HF and should, therefore, also be part of a detailed echocardiographic examination in patients with CA [ 7 ]. One study reported on the predictive effect of pericardial effusion in patients with light chain amyloidosis (AL); however, the studied cohort was small ( n = 31) and only analysed the effects of effusion on all-cause death rather than cardiac death [ 8 , 9 ]. There has recently been evidence that pericardial effusion may also be a sign of myocardial edema following local amyloid infiltration and consecutive inflammation in patients with AL and ATTR [ 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

Prognostic implications of pericardial and pleural effusion in patients with cardiac amyloidosis

et al. 2020

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Background Pericardial and pleural effusion are common findings in patients with cardiac amyloidosis (CA). It is not known, whether effusions correlate with right ventricular (RV) function in these patients. Furthermore, data on the prognostic significance of pleural and pericardial effusion in CA is scarce. Methods Patients with transthyretin (ATTR) and light chain (AL) CA were included in a clinical registry. All patients underwent transthoracic echocardiography at baseline. The presence of pericardial and pleural effusion was determined in every patient. The clinical endpoint was defined as cardiac death or heart failure hospitalization. Results In total, 143 patients were analysed. Of these, 85 patients were diagnosed with ATTR and 58 patients with AL. Twenty-four patients presented with isolated pericardial effusion and 35 with isolated pleural effusion. In 19 patients, both pericardial and pleural effusion were found and in 65 patients no effusion was present at baseline. The presence of pleural effusion correlated well with poor RV function, measured by global RV free-wall strain (p = 0.007) in patients with AL, but not in ATTR. No such correlation could be found for pericardial effusion in either amyloidosis subtype. Patients with AL presenting with pleural effusion had worse outcomes compared to patients with pericardial effusion alone or no effusion at baseline. In the ATTR group, there was no difference in outcomes according to presence and type of effusion. 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 in AL, we were not able to detect this association with pericardial effusion.

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Section: Introductionmentioning

confidence: 99%

Prognostic implications of pericardial and pleural effusion in patients with cardiac amyloidosis

et al. 2020

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“…In the absence of confirmed plasma cell dyscrasia, immunohistochemical techniques are the most useful for classifying the type of amyloidosis [ 8 ]. Several studies concluded that cardiac involvement in amyloidosis is the major determinant of mortality in patients with AL amyloidosis, and the prognosis is very poor [ 9 – 11 ]. However, Quevedo et al [ 12 ] insisted that early diagnosis and prompt therapy are crucial to the reversal of AL-related cardiac dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Amyloid Light-Chain Amyloidosis Manifesting as Heart Failure with Preserved Ejection Fraction in a Patient with Hyper-Immunoglobulin E-emia

et al. 2016

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Patient: Male, 53Final Diagnosis: Acute heart failure • primary AL amyloidosis • hyper IgE-emiaSymptoms: Progressive breathlessnessMedication: Angiotensin-converting enzyme inhibitors and beta blockersClinical Procedure: Skin and endomyocardial biopsySpecialty: CardiologyObjective:Rare co-existence of disease or pathologyBackground:Considering the increased prevalence of heart failure with preserved ejection fraction (HFpEF) as a result of the aging population, the pathophysiology of HFpEF needs to be examined. Furthermore, many comorbidity profiles in patients with HFpEF have been reported. Hypertrophic cardiomyopathy is a well-known specific etiology of HFpEF. Cardiac amyloidosis, which mimics infiltrative and hypertrophic cardiomyopathy, resulting from intensive amyloid deposition, is easily overlooked.Case Report:A 53-year-old man with a 2-week history of persistent breathlessness was referred to our hospital. Upon admission, transthoracic echocardiography showed concentric mild left ventricular (LV) hypertrophy without a characteristic granular sparkling appearance or pericardial effusion, preserved ejection fraction, and bi-atrial enlargement with normal ventricular chambers. Doppler-derived LV diastolic filling demonstrated a prominent restrictive pattern indicating LV stiffness and elevated LV filling pressure. Blood tests revealed severe elevation of B-type natriuretic peptide and marked elevation of immunoglobulin E without eosinophilia. He was diagnosed with primary amyloid light-chain (AL) amyloidosis via skin and endomyocardial biopsy.Conclusions:We encountered a rare case of hypertrophic cardiomyopathy with HFpEF and identified a Doppler-derived restrictive filling pattern suggestive of early-stage heart failure in infiltrative cardiomyopathies. We suggest that infiltrative cardiomyopathies, such as cardiac amyloidosis, should be considered if hypertrophic cardiomyopathy is observed in a patient with HFpEF.

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“…In most cases, pericardial effusions are small and not hemodynamically relevant. There are divergent data on the prognostic significance of pericardial effusions in patients with CA [27]. Pleural effusion: In approximately 40% of patients with AL, pleural effusions are present.…”

Section: Echocardiographymentioning

confidence: 99%

Diagnosis and supportive therapeutic management of cardiac light chain amyloidosis—a cardiologist’s perspective

Binder

Duca

2021

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SummaryCardiac amyloidosis is caused by deposition of abnormally folded proteins (amyloid). The most common forms of amyloidosis which present with cardiac involvement are light-chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Even with novel treatments emerging, the prognosis of these patients remains poor once amyloid deposits in the heart. Therefore, knowledge on clinical and imaging features of cardiac amyloidosis is crucial to make an early diagnosis and improve patient outcomes. This article reviews the most important diagnostic findings of cardiac amyloidosis and gives an overview on the therapeutic management of these patients, including supportive-, device- and disease-specific drug therapies focusing on AL amyloidosis.

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Pericardial Effusion and Multiple Organ Involvement Are Independent Predictors of Mortality in Patients with Systemic Light Chain Amyloidosis

Cited by 16 publications

References 41 publications

Prognostic implications of pericardial and pleural effusion in patients with cardiac amyloidosis

Prognostic implications of pericardial and pleural effusion in patients with cardiac amyloidosis

Amyloid Light-Chain Amyloidosis Manifesting as Heart Failure with Preserved Ejection Fraction in a Patient with Hyper-Immunoglobulin E-emia

Diagnosis and supportive therapeutic management of cardiac light chain amyloidosis—a cardiologist’s perspective

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