Abstract 2007: A 67-year-old Man with Waldenstrom’s Macroglobulinemia, Heart Failure, and a Pericardial Effusion
A 67-year-old man with Waldenstrom’s macroglobulinemia and renal failure was admitted for fatigue. On exam, he had signs of congestive heart failure. His electrocardiogram revealed low voltages. A transthoracic echocardiogram showed symmetrically increased left ventricular thickness, sparkling myocardium consistent with an infiltrative process, diastolic dysfunction, thickened pericardium, and a large pericardial effusion. With impending tamponade, a pericardial window was performed due to the posterior location of the effusion. Postoperatively, the patient was hypotensive with worsening right ventricular failure, and he was treated with inotropes and plasmapheresis for possible hyperviscosity syndrome secondary to elevated IgM lambda light chains. Initially, the patient’s course was felt to be caused by the pericardial effusion and amyloid restrictive cardiomyopathy, because the patient had evidence of primary amyloidosis by renal biopsy and clinical findings indicative of amyloid cardiomyopathy. However, his pericardial biopsy also revealed a thickened, noninflamed pericardium heavily infiltrated with amyloid and raised suspicions for concurrent constrictive pericardial disease. Simultaneous right and left heart catheterization revealed equalization of right atrial pressure, right ventricular end diastolic pressure (RVEDP), pulmonary capillary wedge pressure, and left ventricular end diastolic pressure (LVEDP). There was right and left ventricular concordance, although this did not rule out constriction. Other findings, including similar RVEDP and LVEDP pressures and a RVEDP greater than one third of the RV systolic pressure, were more consistent with constriction. This case highlights a recurring and difficult theme in clinical cardiology, which is to distinguish constrictive pericardial disease from restrictive cardiomyopathy. Diagnostic criteria are reviewed here, and there is evidence to support both processes occurring in this patient, as amyloid appeared to involve both the myocardium, as seen by echocardiography, and the pericardium, as shown by pathology. Pericardial amyloid leading to constrictive pericarditis is extremely rare, with only one previously reported case in the literature.