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INTRODUCTION
The development of transgenic and gene-targeting
technologies that enable experimental manipulation
of the mouse genome have yielded important new
insights into the molecular mechanisms and patho-
physiology of numerous human diseases. Mouse
models of hypertrophic and dilated cardiomyopathy
offer unique advantages in the study of the patho-
physiology of heart failure compared with larger ani-
mal models.The abilities to express transgenes in a
tissue-specific, temporally regulated manner and to
target genes for inactivation allow detailed studies of
the influence of single genes on the progression and
severity of cardiac disease.Transgenic and knockout
mice have a short gestational period of 3 weeks and
reach adulthood in 8 weeks, which allows assess-
ment of therapeutic interventions on the natural his-
tory of heart failure over a relatively short period of
time. Furthermore, recent advances in mouse genet-
ics allow the possibility of screening randomly muta-
genized populations in search of novel genes in-
volved in cardiac function and development.
We have recently used a transgenic approach to
develop a mouse model of idiopathic dilated car-
diomyopathy that resembles many of the anatomic,
physiologic, and clinical features of human dilated
cardiomyopathy.
1
These mice express a dominant
negative mutant form of the CREB transcription fac-
tor (CREB
A133
) under the control of the cardiac spe-
cific α–major histocompatibility complex promoter.
Between 4 and 20 weeks of age, 4-chamber cardiac
dilatation develops in the CREB
A133
mice and, as
noted in preliminary observations, a significant re-
duction is demonstrated in both systolic and diastolic
left ventricular (LV) performance.
Until recently, the usefulness of mouse models of
heart failure has been limited by the inability to accu-
rately and precisely assess cardiovascular physiology
in vivo.This situation has changed in the last 5 years
with the development of high-frequency, high-reso-
lution echocardiographic transducers and catheter-
The Left Ventricular Stress-Velocity Relation
in Transgenic Mice Expressing a Dominant
Negative CREB Transgene in the Heart
Richard C. Fentzke, BS, Claudia E. Korcarz, DVM, Sanjeev G. Shroff, PhD, Hua Lin, MD,
Jeffrey M. Leiden, MD, PhD, and Roberto M. Lang, MD, Chicago, Illinois;
Pittsburgh, Pennsylvania; and Boston, Massachusetts
From the Department of Medicine, University of Chicago (R.C.F.,
C.E.K., R.M.L.); the Department of Bioengineering, University
of Pittsburgh (S.G.S.); and the Harvard School of Public Health/
Harvard Medical School, Brigham and Women’s Hospital, Boston,
Mass (H.L., J.M.L.).
This work was supported in part by a grant (HL54592) from the
NHLBI to J.M.L.
Reprint requests: Roberto M. Lang, MD, University of Chicago
Medical Center, 5841 S. Maryland Avenue, MC 5084, Chicago,
IL 60637 (E-mail: rlang@medicine.bsd.uchicago.edu).
Copyright © 2001 by the American Society of Echocardiography.
0894-7317/2001/$35.00 + 0 27/1/111473
doi:10.1067/mje.2001.111473
Objective: CREB
A133
transgenic mice that express a
dominant negative CREB transcription factor in car-
diomyocytes develop a dilated cardiomyopathy that
is anatomically, physiologically, and clinically simi-
lar to human idiopathic dilated cardiomyopathy. The
goals of this study were to quantitate left ventricular
(LV) contractility and measure cardiac reserve in
CREB
A133
mice by using the relation of end-systolic
wall stress to the velocity of fiber shortening.
Methods: A total of 37 adult CD-1 mice (including both
nontransgenic and CREB
A133
transgenic mice) were
studied with simultaneously acquired high-fidelity
instantaneous aortic pressures and 2-dimensionally
targeted M-mode echocardiograms.
Results: CREB
A133
mice displayed significantly lower
values of LV fiber shortening velocities over a wide
range of afterloads, and they displayed smaller do-
butamine-induced shifts from baseline contractility
relations. Counterbalancing effects of differences in
LV geometry and aortic pressures resulted in com-
parable levels of LV wall stress during ejection in
both groups.
Conclusion: These results demonstrate directly that
CREB
A133
mice display reduced LV contractility at
baseline and decreased cardiac reserve. (J Am Soc
Echocardiogr 2001;14:209-18.)