European Journal of Echocardiography Advance Access published online on April 28, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen151
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org
An unusual case of angina pectoris: a patient with isolated non-compaction of the left ventricular myocardium
André L. Dabarian1,
Charles Mady1,
Carlos E. Rochitte2,
Afonso A. Shiozaki2,
Pedro A. Lemos3 and
Vera Maria Cury Salemi1,*
1 Cardiomyopathy Unit, Heart Institute (InCor), University of Sao Paulo Medical School
2 Magnetic Resonance Section, Heart Institute (InCor), University of Sao Paulo Medical School, São Paulo, Brazil
3 Service of Invasive Cardiology, University of Sao Paulo Medical School, São Paulo, Brazil
Received 15 January 2008; accepted after revision 21 March 2008.
* Corresponding author: Tel: +55 11 35 56 98 12; fax: +55 11 35 56 98 12. E-mail address: verasalemi{at}uol.com.br/ carvera{at}incor.usp.br
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Abstract
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A 29-year-old white woman with typical angina pectoris presented
diastolic dysfunction and was suggestive of isolated non-compaction
of the ventricular myocardium (INCM) by echocardiography. Cardiac
catheterization disclosed normal coronary arteries. Cardiovascular
magnetic resonance (CMR) depicted prominent left ventricular
INCM areas with non-compaction/compaction ratio of 3.7, and
dipiridamol CMR demonstrated global perfusion defect at stress
and normal perfusion at rest. Adenosine-induced vasodilation
showed subnormal coronary velocity flow reserve in the right,
left circumflex, and left anterior descending coronary arteries.
The evidence of our case indicates that patients with INCM may
present angina pectoris and, probably, relative chronic myocardial
ischaemia related to a impaired microvascular function is responsible
for this symptom as demonstrated invasively here. It is a possible
mechanism for progressive myocardial dysfunction seen in these
patients.
Keywords: Cardiomyopathy; Angina pectoris; Diastole; Microcirculation; Magnetic resonance imaging
A 29-year-old white woman was admitted to our inpatient clinic with a history of typical angina pectoris. The electrocardiogram revealed sinus rhythm with repolarization changes. The chest radiograph was normal. The echocardiogram showed normal cardiac chamber volumes, left ventricular (LV) ejection fraction of 0.56, and pseudonormal diastolic dysfunction (Figure 1). Decreased early-diastolic tissue-Doppler peak velocities were observed in the septal, lateral, inferior, and anterior border of the mitral annuli, as well as in the lateral border of the tricuspid annulus. Although the echocardiographic window was limited in visualizing the apex, the short-axis view showed multiple ventricular trabeculations with deep intertrabecular recesses. Colour Doppler revealed blood flow through the deep recesses in continuity with the ventricular cavity, suggestive of isolated non-compaction of the ventricular myocardium (INCM, Supplementary data online Video 1). Cardiac catheterization disclosed normal coronary arteries. Cardiovascular magnetic resonance (CMR) depicted prominent LV INCM areas with non-compaction/compaction ratio of 3.7 (Figure 2A and B and Supplementary data online Video 2). Long-axis view delayed enhancement CMR showed absent fibrosis areas (Figure 2D and E). Short-axis views, dipiridamol CMR stress and rest, respectively, demonstrated global perfusion defect at stress and normal perfusion at rest (Figure 2C and F). Adenosine-induced vasodilation showed subnormal coronary velocity flow reserve in the right coronary artery (Figure 3, left panel), left circumflex (Figure 3, middle panel), and left anterior descending (Figure 3, right panel).
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Figure 1 Mitral inflow (A) and pulsed-wave tissue-Doppler imaging demonstrating decreased early-peak velocity of the septal border of the mitral annuli (B), suggestive of pseudonormal pattern. The lateral border of the tricuspid annulus also showed decreased early-peak velocity (C).
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Figure 2 Long-axis view cardiovascular magnetic resonance (CMR) depicted prominent non-compaction area (A, B). Long-axis view delayed enhancement CMR showed absent fibrosis areas (D, E). Short-axis views stress and rest CMR, respectively, demonstrated global perfusion defect at stress and normal perfusion at rest (C, F). Black arrows indicate non-compaction regions. RA, right atrium; LA, left atrium; RV, right ventricle; LV, left ventricle.
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Figure 3 Adenosine-induced vasodilation showing subnormal coronary velocity flow reserve in the right coronary artery (left panel), left circumflex (middle panel), and left anterior descending (right panel). CFR indicates coronary flow reserve.
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Isolated non-compaction of the ventricular myocardium is a rare
unclassified cardiomyopathy, characterized by the presence of
multiple prominent ventricular trabeculations and deep intertrabecular
recesses, with no other congenital structural malformation.
The diagnosis of our patient fulfilled all three relevant echocardiographic
definitions for INCM.
1–3 Kohdi
et al.4 found that 23.6%
of 199 patients with LV systolic impairment fulfilled one or
more echocardiographic definitions, and suggested that the current
diagnostic criteria for INCM may be too sensitive. The aetiology
is unknown, and heterogeneous genetic background has been described.
The main clinical manifestations are heart failure, arrhythmias,
embolic events, and sudden cardiac death. Chest pain has been
reported in 26% of the patients with INCM.
5 Subendocardial or
transmural perfusion deficits and decreased flow reserve on
CMR or positron emission tomography may play roles in ventricular
diastolic and/or systolic dysfunction and possibly generating
arrhythmias, despite of normal epicardial coronary arteries.
3,6 The evidence of our case indicates that patients with INCM may
present angina pectoris and, probably, relative chronic myocardial
ischaemia related to an impaired microvascular function is responsible
for this symptom as demonstrated invasively here. It is a possible
mechanism for progressive myocardial dysfunction seen in these
patients.
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Supplementary data
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Supplementary data is available at
European Journal of Echocardiography online.
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References
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Abstract
Supplementary data