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The follow electronic only articles are published in conjunction with this issue of Heart.
Altered dystrophin expression in the right atrium of a patient after fontan procedure with atrial flutter
C J McMahon, M Vatta, C D Fraser Jr, J A Towbin, A C Chang
Underlying mechanisms in the development of atrial flutter or intra-atrial re-entry tachycardia in patients with structural cardiac abnormalities remain poorly defined. The right atrial myocardium from two patients with congenital heart disease was evaluated, of whom one presented with severe right atrial dilation and arrhythmia and the other with a normal right atrium, to assess whether increased right atrial pressure and volume overload give rise to sarcolemmal alteration. N-terminus dystrophin staining in the atrium from the patient who had undergone a Fontan procedure showed a normally distributed but significantly reduced staining signal compared with the second patient. This is the first report that patients with severe right atrial dilation and atrial flutter have marked reduction in atrial dystrophin expression.
Lipomatous hypertrophy of the interatrial septum: a commonly misdiagnosed mass often leading to unnecessary cardiac surgery
I Nadra, D Dawson, S A Schmitz, P P Punjabi, P Nihoyannopoulos
Lipomatous hypertrophy of the interatrial septum is a benign cardiac mass that should be considered as part of the differential diagnosis for any atrial cardiac tumour. In the reported case, this lesion was initially suspected to be malignant and the patient was thus referred directly to cardiac surgeons for surgical removal. Unnecessary surgical intervention was swiftly averted because the cardiac surgeon promptly referred the patient for an expert echocardiogram that confirmed the diagnosis of lipomatous hypertrophy. The authors discuss the characteristic features of this lesion and how the diagnosis may be made based on several non-invasive imaging modalities without the need for a tissue biopsy. This condition is more common than initially thought and remains under-recognised by most clinicians. In such cases an increased awareness of this lesion along with the opinion of a specialist echocardiologist would help to avoid a misdiagnosis and unnecessary intervention.
Apical ballooning in relatives
L Pison, P De Vusser, W Mullens
Apical ballooning of the left ventricle was first introduced as takotsubo-like left ventricular dysfunction in 1990 by Satoh and colleagues. The syndrome is characterised by reversible extensive akinesia of the apical and mid-portions of the left ventricle with hypercontraction of the basal segment. For the first time two sisters with this syndrome are reported, suggesting a possible genetic aetiology.
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