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A case report of cardiac amyloidosis presenting with chronic pericardial effusion and conduction block.

ABSTRACT: Background:Amyloidosis is caused by the deposition of abnormal proteins in the extracellular space of various organs. The clinical features of amyloidosis depend on the type of amyloid protein and the organ system involved. Case summary:A 51-year-old woman developed complete heart block which warranted a permanent pacemaker insertion. She was referred for evaluation of chronic pericardial effusion. The patient had stable vital signs and muffled heart sounds on examination of the cardiovascular system. Her chest X-ray film showed a permanent pacemaker in situ, and echocardiogram showed a chronic pericardial effusion without features of tamponade. On further evaluation, she was found to have an M band on serum electrophoresis, elevated free light chain ratio and amyloid deposits in bone marrow biopsy. Technetium pyrophosphate (Tc-PYP) scintigraphy was consistent with cardiac amyloidosis. Discussion:Cardiac amyloidosis can have diverse clinical presentations. Chronic pericardial effusion and conduction block can be a rare presentation of cardiac amyloidosis and needs to be considered while evaluating the same. Cardiac magnetic resonance imaging and Tc-PYP imaging can be used in establishing the diagnosis of cardiac amyloidosis, if endomyocardial biopsy is not feasible.

SUBMITTER: John KJ

PROVIDER: S-EPMC6939806 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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A case report of cardiac amyloidosis presenting with chronic pericardial effusion and conduction block.

John Kevin John KJ Mishra Ajay Kumar AK Iyyadurai Ramya R

European heart journal. Case reports 20190924 4

<h4>Background</h4>Amyloidosis is caused by the deposition of abnormal proteins in the extracellular space of various organs. The clinical features of amyloidosis depend on the type of amyloid protein and the organ system involved.<h4>Case summary</h4>A 51-year-old woman developed complete heart block which warranted a permanent pacemaker insertion. She was referred for evaluation of chronic pericardial effusion. The patient had stable vital signs and muffled heart sounds on examination of the c ...[more]

PMID: 31911988

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Project description:BackgroundErdheim-Chester disease (ECD) is a rare multisystem disorder that primarily affects adults. It is characterized by the excessive production and accumulation of histiocytes, a type of white blood cell, within multiple tissues and organs, including the cardiovascular system. The infiltration of histiocytes can cause a range of cardiovascular symptoms, including pericardial effusion, myocardial infiltration, and heart failure, among others. Despite the potential severity of these cardiovascular manifestations, ECD is often misdiagnosed or underdiagnosed, leading to delays in appropriate treatment and poor outcomes for patients. As such, there is a pressing need for increased awareness and understanding of ECD's cardiovascular manifestations among clinicians and researchers. This article aims to highlight the importance of considering ECD as a potential underlying cause of cardiovascular complaints and to encourage further investigation into this uncommon but potentially life-threatening condition.Case summaryA 63-year-old man presented as outpatient complaining of dyspnoea on exertion during the last 3 weeks (New York Heart Association functional class III). He had also experienced a left shoulder and bilateral knee pain over the last 6 months. The patient was found to have a massive pericardial effusion associated with ECD. While pericardial effusions can have various causes, including infection, cancer, and autoimmune disorders, ECD is one potential cause of this condition. Therefore, it is important for clinicians to consider ECD in the differential diagnosis of patients presenting with unexplained pericardial effusions, particularly in the context of other systemic symptoms suggestive of ECD. We discuss about this specific aetiology and the clinical management of this uncommon condition.DiscussionErdheim-Chester disease, a non-Langerhans cell histiocytosis, is a rare multisystem disorder. Diagnosis is challenging and should be suspected in the presence of a pericardial effusion with conduction abnormalities with indicators of a multisystem disease.

Project description:Background: Primary cardiac angiosarcomas, especially those originating in the pericardium, are extremely rare and aggressive tumors with poor prognosis. These types of malignant tumors have diverse clinical presentations and are often masked by other comorbidities. Case Summary: Our hospital reported a 59-year-old woman who initially presented with pulmonary thromboembolism (PTE) and was subsequently treated with low-molecular-weight heparin. However, she experienced acute pericardial tamponade after anticoagulation therapy, where no obvious mass was primarily identified upon imaging, both in the pericardium or within the heart. Emergency pericardiocentesis and drainage were performed, where a total of 210 mL of bloody effusion was drained. Four months later, she was hospitalized with progressive hemoptysis and dyspnea. A large mixed mass occupying the right pericardium was later identified by coronary computed tomography angiography (CCTA). The mass was consistent with the right atrium, with heterogeneous thickened pericardium and localized moderate pericardial effusion. CCTA and positron emission tomography scans later showed metastases in both lungs and bilateral pleura. Nodules in hilar and mediastinal lymph nodes were also significant. Ultrasound-guided biopsy was performed, and the patient was ultimately diagnosed with an angiosarcoma based on final positive results for both CD31 and CD34 markers. The patient refused chemotherapy and passed away while waiting for her pathology results. The patient survived for 6 months since the first reported episode of PTE. Conclusions: Our case indicates that patients presenting with both embolism and hemorrhage should urgently be channeled to a clinical specialist to confirm any malignant etiology. This would be beneficial to confirm an early diagnosis and lengthen the duration of patient survival. However, the diagnosis of primary cardiac angiosarcoma is still challenging and requires multiple imaging modalities and biopsies in order to assist the accurate diagnosis of disease and achieve effective patient management.

Dataset Information

A case report of cardiac amyloidosis presenting with chronic pericardial effusion and conduction block.

Publications

A case report of cardiac amyloidosis presenting with chronic pericardial effusion and conduction block.

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