Constrictive Pericarditis Revealing Rare Case of ALH Amyloidosis With Underlying Lymphoplasmacytic Lymphoma (Waldenstrom Macroglobulinemia)

Ho, Vivian; O’Sullivan, Jack W; Collins, William J.; Ozdalga, Errol; Bell, Caitlin; Shah, Neil; Krishnam, Mayil S.; Ozawa, Michael G.; Witteles, Ronald

doi:10.1016/j.jaccas.2022.01.007

Cited by 3 publications

(1 citation statement)

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“…Other, less common, causes of constrictive pericarditis include connective tissue diseases such as rheumatoid arthritis, systemic lupus erythematosus, Behçet's disease, scleroderma, and Sjögren syndrome [7,[32][33][34]. Amyloidosis and sarcoidosis can result in fibrous adherence between pericardial layers, as can metabolic pathologies such as uremia [35][36][37][38][39][40]. Inflammation secondary to acute coronary syndromes have also been shown to precipitate constrictive pericarditis, a condition known as Dressler's syndrome [41,42].…”

Section: Etiology Of Constrictive Pericarditismentioning

confidence: 99%

Constrictive Pericarditis: Surgical Management

Cobb¹,

Pahwa²

2023

Pericarditis - Diagnosis and Management Challenges

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Constrictive pericarditis represents an uncommon sequela of multiple pathologic processes. It involves the pericardium, a tri-layered sac that encases the heart within the mediastinum. Inflammation of the pericardium can lead to formation of fibrous adhesions between the outer wall of this sac and the surface of the heart. Due to the stiff, inflexible structure of the pericardium, its adherence to the heart negatively impacts normal diastolic filling and hemodynamics. Over time, this can lead to reduced cardiac output and severe heart failure. This condition is typically refractory to medical treatment. The definitive treatment of constrictive pericarditis involves surgical decortication and removal of the pericardium to alleviate the constriction and restore normal diastolic filling capacity. This procedure has evolved since its inception and is now the gold standard in curing constrictive pericarditis. However, despite its necessity in the treatment of constrictive pericarditis, this procedure carries considerable risk of intra- and post-operative complications and poor outcomes. The poor prognosis is often related to the patient’s pre-surgical status, which must be considered when identifying candidates for surgery. When successful, though, pericardiectomy can produce immediate and progressive improvements in hemodynamic parameters.

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