A 54‐year‐old African American woman with diabetes mellitus, pulmonary sarcoidosis (diagnosed 6 months prior to presentation and maintained on prednisone and hydroxychloroquine), hypertension, and diastolic heart failure presented to the emergency room after a syncopal episode along with 1 month of dyspnea, othopnea, paroxysmal nocturnal dyspnea, and leg edema. Initial exam and workup revealed an acute decompensated congestive heart failure. Her initial cardiac enzymes were negative and EKG showed no acute ischemic changes. Her ejection fraction was 70% 2 weeks prior to presentation, which dropped to 42% on presentation with global abnormal LV myocardial strain. Right heart catheterization during this presentation demonstrated elevated filling pressures, restrictive hemodynamics, and low cardiac output: RA mean, 36; PA, 80/40; PA mean, 53.3; PCWP, 36; Fick CO, 2.99 L/min; Fick CI, 1.34 L/min/m2; PVR, 4; SVR, 1004, MVO2, 42.00%. She was transferred to the heart failure ICU and was started on swan‐guided therapy with nitroprusside, milrinone, dobutamine, and later vasodilators and diuretics as tolerated. In the meantime, endomyocardial biopsy demonstrated amyloidosis due to lambda light chain involvement. Based on endomyocardial and bone marrow biopsies in conjunction with negative skeletal survey, the diagnosis of cardiac amyloidosis was made. In addition, cardiac sarcoidosis was diagnosed based on history and findings of infiltrative process on the cardiac PET scan with focal myocardial inflammation. Because of multiple hospital complications, treatment for amyloidosis was deferred until the patient recovered from her cardiogenic shock. She improved with supportive management and methylprednisolone and her first dose of subcutaneous bortezomib was administered in the hospital. After discharge she was maintained on high‐dose prednisone and weekly bortezomib, and achieved complete remission by light‐chain criteria 5 weeks after treatment.
Infiltrative cardiac diseases are usually classified under restrictive cardiomyopathies. The 3 most common types are: sarcoidosis, hemochromatosis, and amyloidosis. Prognosis depends on the etiology and if left untreated can lead to high mortality. Therefore, it is important to distinguish these types to treat accordingly. This case illustrates a rare case of concomitant cardiac sarcoidosis and amyloidosis in a patient with decompensated heart failure.
In patients presenting with restrictive cardiomyopathy, one should not assume the diagnosis of cardiac sarcoidosis based on the patient's history alone, as further imaging (PET) and endomyocardial biopsy are required for definitive diagnosis. Recognition of concomitant cardiac amyloidosis and cardiac sarcoidosis is essential to preventing the morbidity and mortality associated with these restrictive cardiomyopathies.
To cite this abstract:Hamilton A, Al‐Bawardy R. New‐Onset Cardiomyopathy in a Patient with Sarcoidosis: A Rare Case of Concomitant Cardiac Sarcoidosis and Amyloidosis. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 337. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/newonset-cardiomyopathy-in-a-patient-with-sarcoidosis-a-rare-case-of-concomitant-cardiac-sarcoidosis-and-amyloidosis/. Accessed March 29, 2020.