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Arterial wall properties and Womersley flow in Fabry disease.

Moore DF, Altarescu G, Pursley R, Campia U, Panza JA, Dimitriadis E, Schiffmann R - BMC Cardiovasc Disord (2002)

Bottom Line: No significant difference was found between Fabry patients and controls for internal or external arterial diameters, the incremental Young's modulus, the arterial wall thickness, the pulse wave speed and the basal radial artery blood flow.Further, no significant difference was found for the radial artery blood flow in response to intra-arterial acetylcholine or sodium nitroprusside.Both drugs however, elevated the mean arterial flow.

View Article: PubMed Central - HTML - PubMed

Affiliation: Developmental and Metabolic Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA. dfm1@helix.nih.gov

ABSTRACT

Background: Fabry disease is an X-linked recessive lysosomal storage disease resulting in the cellular accumulation of globotriaosylceramide particularly globotriaosylceramide. The disease is characterized by a dilated vasculopathy with arterial ectasia in muscular arteries and arterioles. Previous venous plethysomographic studies suggest enhanced endothelium-dependent vasodilation in Fabry disease indicating a functional abnormality of resistance vessels.

Methods: We examined the mechanical properties of the radial artery in Fabry disease, a typical fibro-muscular artery. Eight control subjects and seven patients with Fabry disease had a right brachial arterial line placed allowing real time recording of intra-arterial blood pressure. Real time B-mode ultrasound recordings of the right radial artery were obtained simultaneously allowing calculation of the vessel wall internal and external diameter, the incremental Young's modulus and arterial wall thickness. By simultaneously measurement of the distal index finger-pulse oximetry the pulse wave speed was calculated. From the wave speed and the internal radial artery diameter the volume flow was calculated by Womersley analysis following truncation of the late diastolic phase.

Results: No significant difference was found between Fabry patients and controls for internal or external arterial diameters, the incremental Young's modulus, the arterial wall thickness, the pulse wave speed and the basal radial artery blood flow. Further, no significant difference was found for the radial artery blood flow in response to intra-arterial acetylcholine or sodium nitroprusside. Both drugs however, elevated the mean arterial flow.

Conclusions: The current study suggests that no structural or mechanical abnormality exists in the vessel wall of fibro-muscular arteries in Fabry disease. This may indicate that a functional abnormality downstream to the conductance vessels is the dominant feature in development Fabry vasculopathy.

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Related in: MedlinePlus

B-mode ultrasound image of the radial artery. The arterial lumen is indicated by  while the skin surface is indicated by . Image consists 256 × 239 pixels (isotropic scale 0.1 mm/pixel).
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Figure 1: B-mode ultrasound image of the radial artery. The arterial lumen is indicated by while the skin surface is indicated by . Image consists 256 × 239 pixels (isotropic scale 0.1 mm/pixel).

Mentions: The pulse pressure for each subject was derived from the ensemble pressure waveform while estimates of the vessel radii were obtained directly from the TIFF images using NIH Image 1.62 in a blinded fashion. The peak systolic and peak diastolic images were measured separately but in an identical fashion by defining the perimeter of the arterial lumen and the external vessel wall perimeter defined by the medial-adventitial plane with a piece-wise polygon. Using NIH Image the respective internal and external areas were calculated. The caliper line placement along the intimal-medial border and the medial-adventitial interface was judged by review of the video records, and by calculation of a moving edge image by the logical .AND. combination of gradient images, and sequential time difference images. The individual internal and external vessel lengths were also measured and the internal and external vessel radii calculated by dividing the internal and external vessel areas by their respective lengths. This method allowed use of the whole vessel image in estimation of the arterial radii (Figure 1). Subtraction of the internal and external vessel diameters allowed estimation of the vessel wall thickness. The Womersley solution is the only analytical solution for time varying flow in a rigid infinitely long pipe. Validation of the equation for physiological flows has been performed with a high correlation between the calculated mean and observed mean flow [4]. The harmonic volume flow (Q) was calculated by using the following


Arterial wall properties and Womersley flow in Fabry disease.

Moore DF, Altarescu G, Pursley R, Campia U, Panza JA, Dimitriadis E, Schiffmann R - BMC Cardiovasc Disord (2002)

B-mode ultrasound image of the radial artery. The arterial lumen is indicated by  while the skin surface is indicated by . Image consists 256 × 239 pixels (isotropic scale 0.1 mm/pixel).
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC80154&req=5

Figure 1: B-mode ultrasound image of the radial artery. The arterial lumen is indicated by while the skin surface is indicated by . Image consists 256 × 239 pixels (isotropic scale 0.1 mm/pixel).
Mentions: The pulse pressure for each subject was derived from the ensemble pressure waveform while estimates of the vessel radii were obtained directly from the TIFF images using NIH Image 1.62 in a blinded fashion. The peak systolic and peak diastolic images were measured separately but in an identical fashion by defining the perimeter of the arterial lumen and the external vessel wall perimeter defined by the medial-adventitial plane with a piece-wise polygon. Using NIH Image the respective internal and external areas were calculated. The caliper line placement along the intimal-medial border and the medial-adventitial interface was judged by review of the video records, and by calculation of a moving edge image by the logical .AND. combination of gradient images, and sequential time difference images. The individual internal and external vessel lengths were also measured and the internal and external vessel radii calculated by dividing the internal and external vessel areas by their respective lengths. This method allowed use of the whole vessel image in estimation of the arterial radii (Figure 1). Subtraction of the internal and external vessel diameters allowed estimation of the vessel wall thickness. The Womersley solution is the only analytical solution for time varying flow in a rigid infinitely long pipe. Validation of the equation for physiological flows has been performed with a high correlation between the calculated mean and observed mean flow [4]. The harmonic volume flow (Q) was calculated by using the following

Bottom Line: No significant difference was found between Fabry patients and controls for internal or external arterial diameters, the incremental Young's modulus, the arterial wall thickness, the pulse wave speed and the basal radial artery blood flow.Further, no significant difference was found for the radial artery blood flow in response to intra-arterial acetylcholine or sodium nitroprusside.Both drugs however, elevated the mean arterial flow.

View Article: PubMed Central - HTML - PubMed

Affiliation: Developmental and Metabolic Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA. dfm1@helix.nih.gov

ABSTRACT

Background: Fabry disease is an X-linked recessive lysosomal storage disease resulting in the cellular accumulation of globotriaosylceramide particularly globotriaosylceramide. The disease is characterized by a dilated vasculopathy with arterial ectasia in muscular arteries and arterioles. Previous venous plethysomographic studies suggest enhanced endothelium-dependent vasodilation in Fabry disease indicating a functional abnormality of resistance vessels.

Methods: We examined the mechanical properties of the radial artery in Fabry disease, a typical fibro-muscular artery. Eight control subjects and seven patients with Fabry disease had a right brachial arterial line placed allowing real time recording of intra-arterial blood pressure. Real time B-mode ultrasound recordings of the right radial artery were obtained simultaneously allowing calculation of the vessel wall internal and external diameter, the incremental Young's modulus and arterial wall thickness. By simultaneously measurement of the distal index finger-pulse oximetry the pulse wave speed was calculated. From the wave speed and the internal radial artery diameter the volume flow was calculated by Womersley analysis following truncation of the late diastolic phase.

Results: No significant difference was found between Fabry patients and controls for internal or external arterial diameters, the incremental Young's modulus, the arterial wall thickness, the pulse wave speed and the basal radial artery blood flow. Further, no significant difference was found for the radial artery blood flow in response to intra-arterial acetylcholine or sodium nitroprusside. Both drugs however, elevated the mean arterial flow.

Conclusions: The current study suggests that no structural or mechanical abnormality exists in the vessel wall of fibro-muscular arteries in Fabry disease. This may indicate that a functional abnormality downstream to the conductance vessels is the dominant feature in development Fabry vasculopathy.

Show MeSH
Related in: MedlinePlus