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 Editor: Asher Kimchi, MD

Alleles for Long –QT Syndrome are More Often Transmitted to Daughters Than to Sons 
Congenital long-QT syndrome is a rare cardiac disorder in which patients present with a prolongation of the QT interval of the heart rate. The patients are at risk for ventricular arrhythmias, which could lead to syncope and death. Inheritance of long-QT syndrome is autosomal dominant but can also be recessive. Mutations in the potassium-channel genes, KCNQ1 and KCNH2, cause type 1 and type 2 forms of the disease, respectively.  A female predominance has often been observed and has been attributed to an increased susceptibility to cardiac arrhythmias. Pascale Guicheney, PhD et al from the INSERM in Paris, France investigated the possibility of an unbalanced transmission of the deleterious trait to women. Their results showed a skewed segregation of the mutations from mothers to their daughters and explained how it contributes to the female predominance in long-QT syndrome. The study was published in the December 28, 2006 issue of The New England Journal of Medicine. MOREĽ
Balloon Mitral Valvuloplasty Effectively Relieves Severe Congenital Mitral Stenosis 
Congenital mitral stenosis (MS) is a rare condition that is often associated with left heart obstructions. Treatments for MS include balloon mitral valvuloplasty (BMVP), surgical mitral valvuloplasty (SMVP) and mitral valve replacement (MVR). A study by Dr. Doff McElhinney et al from Harvard Medical School compared the outcomes of patients with severe congenital MS who underwent BMVP or surgical intervention. The study, which was published in the August 2, 2005 issue of Circulation found BMVP effectively relieves left ventricular inflow obstruction in the majority of cases and is the preferred mode of treatment in most infants with severe congenital MS. MORE…

Percutaneous Closure of Patent Foramen Ovale May Prove Superior to Drug Therapy in Prevention of Stroke






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