Heart 2009;95:173-175
FEATURED EDITORIAL
Marfan syndrome: 30 years of research equals 30 years of additional life expectancy
Departments of Medicine and Genetics, Center for the Integration of Genetic Healthcare Technologies, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
Dr R E Pyeritz, Maloney 538, Hospital of the University of Pennsylvania, 3400 Spruce St., Philadelphia, PA 19104, USA; reed.pyeritz@uphs.upenn.edu
| The first 150 words of the full text of this article appear below. |
NATURAL HISTORY VERSUS CLINICAL HISTORY
Clinicians should focus on two characteristics of any disorder: natural history and clinical history. The former is defined by manifestations and outcomes that typically occur in the absence of management. The latter is the course of the disease when health professionals and even patients themselves intervene. The concepts are clearly related in a number of ways. One challenging aspect of this inter-relationship results from improved clinical history: as patients live longer, "new", age-dependent features of the disease emerge.
These concepts are well illustrated by Marfan syndrome (MFS). This autosomal dominant heritable disorder of connective tissue is not rare—with a prevalence of one in a few thousand—and has major cardiovascular involvement.1 2 Myxomatous deterioration of the atrioventricular valves leads to prolapse in the majority and moderate regurgitation or worse in some. Dysrrhythmia is common.3 A dilated cardiomyopathy occurs in some. The most characteristic and troublesome feature is dilatation of the aortic root,
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