Ventricular Non-compaction
Rolf Gebker, Ingo Paetsch, Andreas Wahl, Rudolf Meyer and Eike Nagel
German Heart Institute Berlin, Berlin, Germany
A 37-year-old man presented to our outpatient clinic with progressive dyspnoea he had had for two months. He reported to have been diagnosed with hypertrophic non-obstructive cardiomyopathy at the age of 15 and had been treated with a calcium-antagonist ever since. Physical examination was unremarkable except for a third heart sound. The resting ECG demonstrated sinus rhythm, extreme right axis deviation, broad QRS complexes and signs of right and left ventricular hypertrophy. During a 24-hour Holter ECG a non-sustained ventricular tachycardia was registered (Panel A).
Echocardiography and cardiac magnetic resonance imaging (CMRI) showed hyper-trabecularisation with a compacted epicardial and a non-compacted endocardial layer of the left ventricle. Systolic left and right ventricular function was globally impaired (24% and 30%, respectively, Panel B).
Delayed enhancement MRI showed extensive diffuse involvement of the left ventricle and the right-sided inter-ventricular septum (Panel C); the areas of hyper-enhancement were mainly found in areas of non-compacted myocardium.
Left ventricular x-ray angiography revealed the presence of deep recesses (Panel D) and the coronary angiographic status was found to be normal.
Histologically, a right ventricular biopsy, which was taken from an area of delayed enhancement, showed disarray of the myocardial architecture together with infiltration of fibrotic tissue (Panel E).
As a result, ventricular non-compaction was diagnosed. Additionally we demonstrated the potential of CMRI in the non-invasive evaluation of this rare cardiomyopathy: an exact morphological and functional characterisation of the heart, together with delayed enhancement MRI was valuable to assess fibrotic transformation of the myocardium. Such areas may represent the morphological substrate for an electrical re-entry, leading to ventricular arrhythmia.
Figure legend
Panel A: Holter ECG with non-sustained ventricular tachycardia.
Panel B: Echocardiography at end-diastole of a mid ventricular short axis view showing deep inter-trabecular recesses of LV myocardium.
Panel C: Delayed enhancement MRI acquired with diffuse myocardial hyper-enhancement occurring mainly in non-compacted areas.
Panel D: Left ventricular angiography, showing deep inter-trabecular recesses.
Panel E: Histological specimen of a right ventricular biopsy which was taken from an area of hyper-enhancement (haematoxylin and eosin stain); the disarray of myocardial architecture and infiltration of fibrotic tissue can be appreciated.
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