SummaryBackground: The extracellular matrix tissue of the myocardium importantly contributes to left ventricular (LV) performance. Inherited connective tissue disorders related to the FBN1 gene could involve cardiac interstitium resulting in functional abnormalities.Hypothesis: To disclose a primary involvement of myocardium, LV function was studied in 28 patients affected by Marfan syndrome or Marfan-related disorders: 20 Marfan and 8 MASS (Mitral valve prolapse, Myopia, Aortic dilatation, Skeletal involvement, Skin striae) and in 28 healthy, age and gender-matched controls. No valvular regurgitation or any other cardiac alterations were present.Methods: Echocardiographic study was performed to investigate LV systolic and diastolic function.Results: No statistically significant differences were observed between patients and the control group in LV dimensions, systolic function parameters (ejection and shortening fraction), and some diastolic function parameters (E peak, A peak, E/A), while statistically significant differences were
The values of cerebral circulation time were determined by means of rapid serial angiography in the chloralosed dog under control conditions, after administration of strychnine and after injection of ergotamine. These results were compared with those obtained by the simultaneous registration of the intracranial venous pressure. Confirming previous observations made by means of this latter technique, the angiographic results showed that strychnine reduces the cerebral circulation time by provoking an increase in cerebral blood flow, whereas ergotamine causes a cerebral vasoconstriction, thereby increasing intracranial circulation time. Moreover, a statistically significant inverse correlation exists between the values of the intracranial pressure and the cerebral circulation time. New evidence of the relationship between the extra- and the intracranial circulation was obtained by the angiographic observations; these latter data are discussed in relation with the problem of the lability of cerebral vasoconstrictor reactions.
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