From the Department of Internal Medicine III, University of Heidelberg,
Germany.
Correspondence to C. Haller, MD, Medizinische Klinik III, Universität Heidelberg, Bergheimer Str 58, 69115 Heidelberg, Germany. E-mail challer{at}krzmail.krz.uni-heidelberg.de
Case Report
A 24-year-old woman was evaluated for exertional dyspnea and chest
pain. Echocardiography showed marked apical, septal
(14 mm), and anterolateral (21 mm) hypertrophy
with normal inferior and posterior wall thickness (Figure 1
Comment
The vascular ectasia was most pronounced in the vessels supplying
the hypertrophic myocardium, suggesting that the vascular
dilation was causally related to functional and/or structural changes
of the left ventricular wall. The coronary ectasia
associated with hypertrophic cardiomyopathy appears
to be morphologically and etiologically distinct from coronary
aneurysms in vasculitis and coronary artery disease. In
hypertrophic cardiomyopathy, the phasic
characteristics of coronary arterial blood flow may
be exaggerated to the extent that even systolic flow reversal
has been described.1 However, it is unusual to
see these phasic variations in luminal diameter during coronary
angiography. The systolic obliteration of the distal LAD system
in the present patient suggests that the abnormally high wall
tension of the hypertrophic left ventricular apex and
septum may act functionally as a giant "muscle bridge," causing
systolic blood flow cessation.2 High
intraluminal pressure during ventricular systole caused by
peripheral flow obstruction may have contributed to the
ectatic morphology of the LAD.
The clinical symptom of angina in the presence of massive
coronary ectasia could be explained by the mismatch between the
metabolic demand of the hypertrophic myocardium
and the regional blood supply3 compromised by
intermittent coronary flow obstruction and possibly
coexistent small-vessel
disease.4 5
Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1–267, Houston, TX 77030.
References
1.
Akasaka T, Yoshikawa J, Yoshida K, Maeda
K, Takagi T, Miyake S. Phasic coronary flow characteristics in
patients with hypertrophic cardiomyopathy: a study
by coronary Doppler catheter. J Am Soc
Echocardiogr. 1994;7:9–19.[Medline]
[Order article via Infotrieve]
2.
Brugada P, Bar FW, de Zwaan C, Roy D, Green M, Wellens
HJ. "Sawfish" systolic narrowing of the left anterior
descending coronary artery: an angiographic sign of
hypertrophic cardiomyopathy.
Circulation. 1982;66:800–803.
3.
Nienaber CA, Gambhir SS, Mody FV, Ratib O, Huang SC,
Phelps ME, Schelbert HR. Regional myocardial blood flow and glucose
utilization in symptomatic patients with hypertrophic
cardiomyopathy. Circulation. 1993;87:1580–1590.
4.
Dissmann R, Schultheiss H. Ischemia in
patients with hypertrophic cardiomyopathy: various
causes and symptoms and the difficulties of ischemia screening
tests. Eur Heart J. 1996;17:982–984.
5.
Maron BJ, Wolfson JK, Epstein SE, Roberts WC.
Intramural ("small vessel") coronary artery disease in
hypertrophic cardiomyopathy. J Am Coll
Cardiol. 1986;8:545–557.[Abstract]
© 1998 American Heart Association, Inc.
Images in Cardiovascular Medicine
Coronary Artery Ectasia and Systolic Flow Cessation in Hypertrophic Cardiomyopathy
). The patient underwent right and left
heart catheterization, with coronary and
biventricular angiography. The cardiac index was 2.2 L
· min-1 · m-2.
The left ventricular pressure was 100/0 to 10 mm Hg,
without evidence of an intracavitary gradient. Right anterior oblique
ventriculography demonstrated a subtotal obliteration of the left
ventricular cavity during systole (Figure 2). Simultaneous right and
left ventricular angiography revealed a massively thickened
interventricular septum (Figure 3). Coronary angiography showed
no hemodynamically relevant fixed stenosis. The
striking finding was the dilation and pronounced tortuosity of the
coronary arteries, particularly the left anterior descending
arterial (LAD) system (Figure 4, bottom), without signs of a coronary
artery-to-left ventricular fistula. The coronary perfusion
pattern of the LAD showed marked dynamic changes: the dye propagation
occurred only during ventricular diastole;
during systole, radiocontrast flow practically ceased, with apparent
obliteration of the vascular lumen. Figure 4 shows the left
coronary arterial system during the same cardiac
cycle: during systole, the LAD system is "squeezed empty" (Figure 4, top), while the same vessels are rapidly filled during the
subsequent diastole (Figure 4, bottom). The dynamic changes
of luminal diameter are illustrated quantitatively in Figure 5. The caliber of a compressed and
noncompressed branch of the LAD (Figure 4, top and bottom, arrows) was
measured frame by frame (12.5 frames per second) during three
consecutive cardiac cycles by digital image analysis (CASS II,
CVA4v5.2).
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Figure 1. Echocardiographic apical
four-chamber view showing normal left ventricular
dimensions with septal and apical hypertrophy.
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Figure 2. Left ventriculogram (right anterior oblique
projection) during systole (top) and during diastole
(bottom). During systole, there is almost complete obliteration of
apical left ventricular cavity.
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[in a new window]
Figure 3. Simultaneous left and right
ventricular angiography (left anterior oblique
projection) showing marked septal hypertrophy.
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Figure 4. Left coronary arterial system
(right anterior oblique projection) during systole (top) and during
diastole (bottom) of same cardiac cycle. During systole,
there is complete cessation of radiocontrast flow in LAD, with
subsequent filling during diastole. Note that left
circumflex artery and epicardial LAD are completely filled during both
systole and diastole. Arrows indicate vessels in which
consecutive measurements of luminal diameter were made.
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[in a new window]
Figure 5. Changes in vascular diameter of same LAD segments
during three cardiac cycles measured with CASS II image
analysis program. A, Comparative analysis of epicardial LAD
segment during same cardiac cycles at some distance from hypertrophic
myocardium. There are only minor changes in luminal diameter. B, Distal
intramural branch in region of hypertrophic left ventricular septum.
Note marked diminution of luminal diameter during each ventricular
systole.
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