Loeys-Dietz Cardiomyopathy? Long-Term Follow-Up After Onset of Acute Decompensated Heart Failure.

Loeys-Dietz syndrome (LDS) is an inherited connective tissue disorder the phenotype of which resembles Marfan syndrome (MFS). LDS frequently affects the cardiovascular system leading to aortic aneurysm or dissection, but unlike MFS, primary cardiomyopathy is very rare in LDS, and thus the detailed clinical course of LDS-associated cardiomyopathy is unknown. We report the first case of a patient with LDS-associated cardiomyopathy whose left ventricular systolic function was reduced at the onset of acute heart failure (HF) but markedly improved by pharmacological therapy including an angiotensin-receptor blocker with no recurrence of worsening HF during 7 years of follow-up.