DOI:10.2214/AJR.07.3481
AJR 2008; 190:W274
© American Roentgen Ray Society
Reply
Jonathan D. Dodd and
Ricardo C. Cury
St. Vincent's University Hospital Dublin, Ireland
Massachusetts General Hospital Harvard Medical School Boston,
MA
WEB—This is a Web exclusive article.
We thank Dr. Fazio and colleagues
[1] for their comments
regarding our study [2]. It is
interesting to read that not only adults (as in our study) but also pediatric
patients with left ventricular noncompaction may exhibit trabecular delayed
hyperenhancement. It is also interesting to note that in their pediatric
population, trabecular delayed hyperenhancement is associated with a reduction
in ejection fraction (EF) and a poorer prognosis. Although the letter by Dr.
Fazio comments that in our study delayed hyperenhancement was seen in the
middle myocardium, we presume this to be a typographic error, and that they
meant to indicate trabecular delayed hyperenhancement at the mid ventricular
level.
In our adult study, patients with a mild stage of clinical disease (minimal
symptoms and normal left ventricular EF) showed no trabecular hyperenhancement
compared with those with moderate or severe clinical stages of disease.
Patients with the most severe clinical stage of disease showed the most
extensive trabecular delayed hyperenhancement. Thus, noninvasive detection of
trabecular delayed hyperenhancement is both technically feasible and appears
associated with clinical severity of disease. The clinical relevance of such
findings lies in the mixed reports regarding long-term outcome in this
disorder [3].
Some studies suggest many patients may have a relatively good prognosis
[4], whereas others have found
complications associated with left ventricular noncompaction to be relatively
common [5]. Contributors to
endomyocardial embryogenesis, such as genetic factors, appear to have little
influence on the long-term prevalence of arrhythmias or thromboembolic risk
[5]. Our pilot study suggests
the addition of cardiac MRI with delayed contrast-enhanced sequences may help
identify those patients with more severe ventricular dysfunction.
Our study design did not allow us to correlate extent or severity of
trabecular delayed hyperenhancement with long-term outcome. Future
longitudinal studies should include cardiac MRI delayed contrast-enhanced
sequences to assess severity and extent of trabecular delayed hyperenhancement
in addition to assessing the morphologic abnormalities of left ventricular
noncompaction [6]. Such
investigations may help to advance accurate risk stratification of patients
with this disorder.
References
- Fazio G, Visconti C, Grassedonio E, et al. Delayed MRI
hyperenhancement in noncompaction: sign of fibrosis correlated with clinical
severity. (letter) AJR 2008;190
:[web] W273[Free Full Text]
- Dodd JD, Holmvang G, Hoffmann U, et al. Quantification of left
ventricular noncompaction and trabecular delayed hyperenhancement with cardiac
MRI: correlation with clinical severity. AJR2007; 189:974
-980[Abstract/Free Full Text]
- Fazio G, Sutera L, Corrado G, Novo S. The chronic heart failure is
not so frequent in noncompaction. (reply to letter) Eur Heart
J 2007; 28:1269[Free Full Text]
- Murphy RT, Thaman R, Blanes JG, et al. Natural history and familial
characteristics of isolated left ventricular non-compaction. Eur
Heart J 2005; 26:187
-192[Abstract/Free Full Text]
- Lilje C, Razek V, Joyce JJ, et al. Complications of non-compaction
of the left ventricular myocardium in a paediatric population: a prospective
study. Eur Heart J 2006;27
: 1855-1860[Abstract/Free Full Text]
- Petersen SE, Selvanayagam JB, Wiesmann F, et al. Left ventricular
non-compaction: insights from cardiovascular magnetic resonance imaging.
J Am Coll Cardiol 2005;46
: 101-105[Abstract/Free Full Text]

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