Critical Role of Extracellular Matrix Remodelling in Patients with Dilated Cardiomyopathy: Lessons from Connective Tissue Disorders.

Heritable connective tissue disorders are a large group of genetically determined multisystem disorders that primarily target the extracellular matrix (ECM). The ECM of the cardiovascular system consists of collagen and elastic fibres embedded in a composite of proteoglycans and matricellular proteins and regulated by matrix metalloproteinases, tissue inhibitors of matrix metalloproteinases, A disintegrin and metalloproteinases, and various signalling complexes. 1 Takawale A. Sakamuri S.S. Kassiri Z. Extracellular matrix communication and turnover in cardiac physiology and pathology. Compr Physiol. 2015; 5: 687-719 Google Scholar Loeys-Dietz Cardiomyopathy? Long-term Follow-up After Onset of Acute Decompensated Heart FailureCanadian Journal of CardiologyVol. 38Issue 3PreviewLoeys-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 therefore 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 (LV) systolic function was reduced at the onset of acute heart failure (HF) but markedly improved by pharmacologic therapy including an angiotensin-receptor blocker, with no recurrence of worsening HF during 7 years of follow-up. Full-Text PDF