We thank Professors Stöllberger and Finsterer for their interest in our review. It is with sadness that we must respond without the support of Robert Freedom. As the readers of “Cardiology in the Young” are well aware, the encyclopaedic review of left ventricular non-compaction was one of Robert's final projects prior to his untimely death.1 The topics that are raised by Stöllberger and Finsterer, in fact, were all discussed at length in the original review.2 Could it be that, as in Shakespeare's Hamlet, “The lady doth protest too much, methinks”? We received a letter couched in similar vein subsequent to the publication of the report concerning the use of magnetic resonance imaging in the diagnosis of non-compaction prepared by Petersen et al.3 This exchange of letters has now been published.4, 5 We predict, nonetheless, that like Canute, our Viennese colleagues will find it hard to turn back the ever-increasing tide of studies showing non-compaction to be a morphological trait rather than a specific cardiomyopathy, be the cardiomyopathy itself congenital or acquired. But Stöllberger and Finsterer are correct when they stress that the evidence for the trait being congenital has still to be established with certainty, and we fully accept that some patients may develop the manifestation of the trait during life. We wonder, however, whether the evidence is equally compelling for disappearance of the non-compacted layer during life?
It would be non-productive to answer each of their comments in turn, since it remains our belief that our opinions were stated with clarity by Robert Freedom in our initial review.2 We would emphasise that our opinion that the lesion represented failure of compaction of the embryonic myocardium was based more on examination of developing human hearts than on inferences made from the study of Petersen et al.3 We accept, nonetheless, that our embryological evidence to date is far from complete. We doubt very much that, despite their protestations, the term “hypertrabeculation” will replace the much more popular alternative of ventricular non-compaction. There is no question in our own minds that the arrangement seen in postnatal life is remarkably reminiscent of the non-compacted arrangement of the left ventricular wall as illustrated in our review in the early stages of development of the human heart, but we accept fully that further investigation is needed finally to prove the hypothesis that most cases represent a congenital malformation. Disputation remains the lifeblood of science, and it is good that we continue to debate the origins of left ventricular non-compaction, since the one fact on which all agree is that the entity is now recognised with ever-increasing frequency.
