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Marfan Syndrome May Be the Vulnerable Factor for Rapidly Progressive Cardiac Decompensation in Patients with Dilated Cardiomyopathy



This is the first case to report coexisting Marfan syndrome and hyperthyroidism, two recognized contributory factors of dilated cardiomyopathy, in a patient who presents with rapidly progressive severe congestive cardiac failure. Dilated cardiomyopathy was the first presenting feature in this young tall man with Marfanoid phenotype who had only a few vague symptoms of hyperthyroidism. Initial cardiac failure treatment with optimised medical therapy did not show improvement and even led to rapid deterioration in the presence of undiagnosed hyperthyroidism which rapidly progressed to thyroid storm. Full screening for all possible underlying causes of dilated cardiomyopathy should be recommended even the patient has full-blown phenotypic pictures of Marfan syndrome, a recognized cause of dilated cardiomyopathy. The weakness in connective tissue integrity in Marfan syndrome can be a vulnerable factor for severe and rapidly progressive cardiomyopathy if the patient has coexisting dilated cardiomyopathy secondary to hyperthyroidism. 

18 Dec, 2017
e-Published: 20 Dec, 2017


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