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Re: Noncompaction is associated with neuromuscular disorders even in the absence of systematic neurologi

Dear Editor,

With interest we read the paper by Lofiego et al. on a long-term follow-up of 65 patients with left ventricular hypertrabeculation/noncompaction (LVHT).[1] We want to add some points and raise some concerns: The prevalence of neuromuscular disorders (NMDs) among patients with LVHT has been reported to be up to 82%.[2] The low rate of 9% in the presented study may be attributed to the fact that the patients were not systematically screened for NMDs. Under these aspects the rate of NMDs in

Lofiego’s study is high and most surprising. Why were the included patients not systematically referred to a neurologist in light of these findings and previous recommendations to examine all LVHT patients neurologically?[3] Which type of muscular dystrophy was diagnosed in the 4 patients mentioned in the results? In the discussion, but not in the results, a patient with Duchenne-muscular-dystrophy is mentioned. The authors mention a patient with limb-girdle-muscular-dystrophy with LVHT, a NMD in which LVHT has not been observed thus far.[3] Which type of limb- girdle-muscular-dystrophy did the patient suffer from and was there a correlation between the cardiac abnormalities and the severity of the muscular abnormalities?

There are conflicting results concerning the need to generally anticoagulate patients with LVHT [4]. The idea that the rate of thromboembolism is increased in LVHT is intriguing, but it is only substantiated by case reports or case studies. In a study on 62 LVHT patients, no increased rate of stroke/embolism was found in comparison with control patients, matched for age, sex, and systolic function.[5] Thus, it is not understandable why all patients with an EF <30% (in the discussion <35%) were anticoagulated, irrespective if they had atrial fibrillation or not.

Were sources of thromboembolism other than LVHT excluded in patients with LVHT and a history of thromboembolism? The rate of patients receiving oral anticoagulation was 62% in the symptomatic group but only one had atrial fibrillation at inclusion, and 5 developed it obviously during follow-up. How many of the remaining patients had a history of thromboembolism or reduced systolic function? Which was the indication for oral anticoagulation in the 4 non-symptomatic patients? Detection of only 65 LVHT patients within 14 years (1991 respectively 1995 to 2004) appears to be lower than previously reported.[6] How to explain this low prevalence of LVHT in the two centres?

How to explain that among non-symptomatic LVHT familiarity was found in 71% as compared to 17% in the symptomatic LVHT cases? If this is attributable to the non-systematic search for familial cases, such figures are misleading and should be considered with caution. How to explain the discrepancy between the follow-up duration of 6 to 193 months and an observational period of 14y (168m)? Concerning the follow-up character of the study it would be interesting to know how many of the non-symptomatic patients became symptomatic and how many developed heart failure during the observational period?