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DOI:10.2214/AJR.07.3481
AJR 2008; 190:W274
© American Roentgen Ray Society

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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.


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References
 

  1. 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]
  2. 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]
  3. 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]
  4. 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]
  5. 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]
  6. 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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This Article
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