Key result
CRT-D and corticosteroids stabilize progressive heart failure in a Marfan patient with isolated cardiac sarcoidosis.
Case Report (n=1)
No
This case highlights the importance of considering inflammatory cardiomyopathy, such as isolated cardiac sarcoidosis, for postoperative heart failure in patients with Marfan syndrome.
May prompt sarcoidosis consideration in Marfan patients with post-surgical LV dysfunction; hypothesis-generating and requires prospective validation.
A 55-year-old male patient with Marfan syndrome presented with exertional dyspnea. The patient had undergone aortic valve and ascending aortic replacement and experienced two admissions for congestive heart failure. His thoracic echocardiography revealed severe left ventricular systolic dysfunction with regional wall motion abnormalities and an apical aneurysm. Meanwhile, coronary angiography showed no significant stenosis. Cardiac magnetic resonance imaging demonstrated wall thinning and late gadolinium enhancement (LGE) in the apical, inferior, and inferolateral segments, corresponding to focal-on-diffuse myocardial uptake on 18F-fluorodeoxyglucose positron emission tomography/computed tomography. No extracardiac sarcoid lesions were identified. Moreover, right ventricular endomyocardial biopsy was nondiagnostic. Based on the multimodality imaging and laboratory findings, a clinical diagnosis of isolated cardiac sarcoidosis was established according to current guidelines. The patient underwent cardiac resynchronization therapy with defibrillator implantation followed by corticosteroid therapy, with no subsequent heart failure events. This case highlights the importance of considering inflammatory cardiomyopathy for postoperative heart failure in patients with Marfan syndrome.
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Tasaka et al. (2026) conducted a case report in Isolated Cardiac Sarcoidosis and Marfan Syndrome (n=1). CRT-D implantation and corticosteroid therapy was evaluated on Clinical stability. A 55-year-old male with Marfan syndrome and progressive postoperative heart failure was diagnosed with isolated cardiac sarcoidosis and remained clinically stable after CRT-D and corticosteroids.
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