Current Pharmaceutical Design - Volume 21, Issue 4, 2015

Cardiomyopathies are a heterogeneous group of primary diseases of the myocardium usually of genetic origin and with familial presentation. The identification of multiple genetic causes for these diseases has opened a new window for early diagnosis, understanding of their natural history and improvement in risk stratification and management. However, in the past years, the clinical application of genetics has been limited by t Read More

Improved treatments for heart failure patients will require the development of novel therapeutic strategies that target basal disease mechanisms. Disrupted cardiomyocyte Ca2+ homeostasis is recognized as a major contributor to the heart failure phenotype, as it plays a key role in systolic and diastolic dysfunction, arrhythmogenesis, and hypertrophy and apoptosis signaling. In this review, we outline existing knowledge of the in Read More

The pharmacological treatment of dilated cardiomyopathy overlaps with the treatment of heart failure. The primary objective of this treatment is to slow the progression of disease and improve quality and length of life. All patients, including those with asymptomatic dysfunction of the left ventricle, ought to receive angiotensin converting enzyme inhibitors, (in the case of intolerance, angiotensin receptor blockers), and bet Read More

Inflammatory cardiomyopathy is a term used for left ventricular systolic dysfunction associated with myocarditis. In order to establish definitely the diagnosis, an endomyocardial biopsy (EMB) must be performed. Based on the results of EMB analysis, specific treatment may be added to conventional heart failure therapy. Immunosuppressive therapy in patients with non-viral inflammatory cardiomyopathy has been shown Read More

Hypertrophic cardiomyopathy (HCM) is an inherited disorder of cardiac sarcomeric proteins characterised by the presence of myocardial hypertrophy in the absence of abnormal loading conditions. Individuals with HCM can remain asymptomatic throughout their lives but many experience chest pain, symptoms of heart failure, syncope and sudden ventricular arrhythmias. Cardiopulmonary exercise testing is an i Read More

Anderson-Fabry disease is an X-linked lysosomal storage disorder caused by alpha-galactosidase A deficiency. The intracellular storage of globotriaosylceramides in different tissues and organs leads to a multisystemic disease affecting kidneys, nervous system and the heart. Fabry cardiomyopathy is frequent and leads to concentric left-ventricular hypertrophy. Typical pattern in advanced stages is myocardial replac Read More

Friedreich’s ataxia (FA) is associated with progressive cardiac hypertrophy resulting from a genetic abnormality in the frataxin gene. Cardiac involvement is the most common cause of death (59%) in FA patients. Cardiac related death occurs at a significantly younger age than non-cardiac related death. Idebenone is a short-chain quinone analogue with a potent free-radical scavenger action. This drug has the potential to p Read More

We have gained considerable insight and understanding about the etiology, embryogenesis of the myocardium, genetic background, diagnosis and outcome of left ventricular non-compaction (LVNC) over the last 2 decades. LVNC has a distinct morphological appearance with a thickened, two-layered myocardium consisting of an epicardial compacted and a thicker endocardial non-compacted layer. These features make the re Read More

The amyloidoses represent a group of diseases characterized by extracellular deposition of abnormal protein, amyloid, which is formed by insoluble extracellular fibrils in β-pleated sheets. Although cardiac involvement may occur in all types of amyloidoses, clinically relevant amyloid cardiomyopathy is a typical feature of AL amyloidosis and transthyretin-related amyloidoses. Congestive heart failure represents the co Read More

Peripartum cardiomyopathy (PPCM) is a relatively rare disease characterized by systolic heart failure occuring towards the end of pregnancy or during the months following birth. It is most often seen in women of African descent, and its incidence seems to be slightly increasing in recent years. Other etiologies of heart failure should be excluded to determine the diagnosis of PPCM. The clinical picture corresponds to systol Read More

Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiac muscle disease associated with sudden cardiac death, ventricular arrhythmias and cardiac failure. It is primarily a disease of the desmosome, with mutations in desmosomal protein genes identified in approximately 50% of affected individuals. Mutations result in fibrofatty replacement of cardiomyocytes, aneurysm formation and dilata Read More

(117 words) Acute and recurrent pericarditis, isolated pericardial effusion, and constrictive pericarditis represent the main pericardial syndromes and therapy of these conditions should be targeted as much as possible at the specific aetiology, but unfortunately many cases still remain idiopathic with current diagnostic methods and should be empirically treated according to best available evidence. Unfortunately, treat Read More