Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2005 Apr 27:3:11.
doi: 10.1186/1476-7120-3-11.

Reduced coronary flow reserve in Anderson-Fabry disease measured by transthoracic Doppler echocardiography

Paweł Petkow Dimitrow  1 , Marek Krzanowski, Anetta Undas
Affiliations
Case Reports

Reduced coronary flow reserve in Anderson-Fabry disease measured by transthoracic Doppler echocardiography

Paweł Petkow Dimitrow et al. Cardiovasc Ultrasound. .

Abstract

Coronary flow reserve was assessed in a patient with Anderson-Fabry disease complicated by symmetric left ventricular hypertrophy. Coronary flow reserve was measurable in all three major coronary arteries providing an opportunity to compare regional coronary flow reserve from different vascular beds. In this patient all the three vascular beds supplied diffusely hypertrophied myocardium. Coronary flow disturbances in small intramyocardial perforating arteries were visible. The coronary flow reserve was reduced to a similar level (around to 2.0) in all three major arteries. In our patient with Anderson-Fabry disease, the coronary vasodilatation was blunted in a diffuse pattern corresponding to the myocardial hypertrophy distribution. In small intramyocardial arteries coronary flow was also disturbed. Accordingly, retrograde systolic flow and accelerated anterograde diastolic flow were documented.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Parasternal short axis view, color Doppler examination at the heart base level. Left main, left anterior descending (LAD) and origin of left circumflex (Cx) coronary arteries are seen. Blood flow within the left main coronary artery and proximal LAD is depicted in red, while in the Cx – in blue.
Figure 2
Figure 2
Modified apical four-chamber view, color Doppler examination. Flow detected in long segment of the right posterior descending (RPD) coronary artery is depicted in red.
Figure 3
Figure 3
Modified apical 2-chamber view, apical area, color Doppler examination. The LAD and the penetrating intramyocardial arteries (vertical perforators branching of the LAD) are seen.
Figure 4
Figure 4
Modified apical 2-chamber view, middle segment of the inferior wall on color Doppler examination. Small (arch-shaped) arteries branching of the right posterior descending (RPD) coronary artery are seen.
Figure 5
Figure 5
The orientation of Doppler gate within the LAD perforator.
Figure 6
Figure 6
The flow velocity spectrum in the LAD perforator visualized in figure 5. In diastole the flow velocity is negative (towards ventricular chamber) and during systole the retrograde flow (towards epicardium) results in positive value of velocity.
Figure 7
Figure 7
Diastolic flow velocity spectrum in distal portion of LAD.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Linhart A, Palecek T, Bultas J, Ferguson JJ, Hrudova J, Karetova D, Zeman J, Ledvinova J, Poupetova H, Elleder M, Aschermann M. New insights in cardiac structural changes in patients with Fabry's disease. Am Heart J. 2000;139:1101–1108. doi: 10.1067/mhj.2000.105105. - DOI - PubMed
    1. Kampmann C, Wiethoff CM, Perrot A, Beck M, Dietz R, Osterziel KJ. The heart in Anderson Fabry disease. Z Kardiol. 2002;91:786–795. doi: 10.1007/s00392-002-0848-5. - DOI - PubMed
    1. Doi Y, Toda G, Yano K. Sisters with atypical Fabry's disease with complete atrioventricular block. Heart. 2003;89:e2. doi: 10.1136/heart.89.1.e2. - DOI - PMC - PubMed
    1. Dimitrow PP. Transthoracic Doppler echocardiography- noninvasive diagnostic window for coronary flow reserve assessment. Cardiovasc Ultrasound. 2003;1:4. doi: 10.1186/1476-7120-1-4. - DOI - PMC - PubMed
    1. Krzanowski M, Bodzon W, Dimitrow PP. Imaging of all three coronary arteries by transthoracic echocardiography: an illustrated guide. Cardiovasc Ultrasound. 2003;1:16. doi: 10.1186/1476-7120-1-16. - DOI - PMC - PubMed

Publication types