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• W2000661930 abstract "To our recently published manuscript ‘Non-compaction cardiomyopathy in an adult with hereditary spherocytosis’ 1, which describes for the first time left ventricular (LV) non-compaction in an adult with hereditary spherocytosis, Finsterer and Stöllberger have raised a number of questions, to which we are happy to respond. Since our article was published as a ‘Short Communication’ its length was limited and thus, our description was restricted to essential and noteworthy findings and obviously, all of the normal data on the patient could not be included. Finsterer and Stöllberger had questions on the episodes and thromboembolic consequences of episodes of atrial fibrillation. As described in the original contribution, prior episodes of atrial fibrillation had been reported and splenectomy had been performed 6 years ago. In several ECG recordings over recent years the patient was in sinus rhythm as documented also by Holter monitoring, and no embolic events were observed. There is no evidence from prospective studies that LV non-compaction per se is associated with an increased risk of thromboembolic events. Remarkably, Finsterer and Stöllberger themselves concluded from their own retrospective analysis that LV non-compaction does not increase the risk of embolism or stroke and, thus, does not require oral anticoagulation 2. Also, the case report on an 18-month-old infant with LV non-compaction and thromboembolism 3, which they quoted in their letter, does not support their argument for an increased risk of thromboembolism with non-compaction, since the patient suffered from impaired thrombostasis. In accordance with guidelines 4,5, we did not recommend that the young patient, who was in documented sinus rhythm during the last year, initiate oral anticoagulation therapy, as this would have inherently increased the risk of bleeding substantially. In their Letter to the Editor, Finsterer and Stöllberger summarize various case reports with potential genetic disorders in single cases of LV-non compaction, and an overview of the previous literature 6. We are well aware of the fact that several very rare neuromuscular disorders can be associated with LV non-compaction. This was not the point that we wanted to make with our contribution, which focuses on the association between non-compaction, myopathy and spherocytosis. This is the first report describing this combination. Since the association between LV non-compaction and muscular disorders has been extensively described previously and documented to have no therapeutic consequences, we did not recommend that our patient undergo specific neuromuscular examinations, since there is no evidence for any treatment benefit. With respect to genetic analysis, we have suggested that our patient is included in the multi-centre EUROGENE study of hereditary cardiomyopathies. We await the results of this large registry analysis in the near future and will then be happy to comment upon this aspect." @default.
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• W2000661930 date "2007-01-01" @default.
• W2000661930 modified "2023-09-27" @default.
• W2000661930 title "Reply to letter from Josef Finsterer and Claudia Stollberger" @default.
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• W2000661930 doi "https://doi.org/10.1016/j.ejheart.2006.09.005" @default.
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