Fabry’s disease is an X-linked lysosomal storage disease caused by a deficiency of a-galactosidase-A that results in an accumulation of intracellular glycolipid in the heart and other organs. l Several electrocardiographic changes have been described in affected patients, including varying degrees of atrioventricular block, ST and T-wave changes, and a short PR interval.24 These changes are thought to be due to glycosphingolipid deposition involving the myocardial fibers and conduction system.24 However, the etiology of a short PR interval in Fabry’s disease is not completely understood. We report a patient with heterozygous Fabry’s disease who presented with palpitations and a short PR interval, and underwent detailed electrophysiologic testing. A 43-year-old white woman with a well-documented history of a carrier state for Fabry’s disease (based on decreased levels of a-galactosidase)presented with palpitations and dyspnea. On examination, her pulse was irregular, with a rate of 150 beats1 min. An initial electrocardiogram demonstrated atrialBbrillation, with an average ventricular response of approximately 150 beatslmin and shortest RR interval of 350 ms. A total of 0.75 mg of digoxin was administered in sequential doses, followed by conversion to normal sinus rhythm. Further digoxin dosing was held. An electrocardiogram recorded during sinus rhythm (Figure 1) showed a short PR interval of 110 ms, with a normal QRS duration and no evidence of preexcitation. The echocardiogram was normal. To assess the etiology of the short PR interval, an electrophysiologic

Lipomatous hypertrophy of the atrial septum (LHAS) is an infrequently observed entity characterized by an unencapsulated proliferation of mature and multivacuolated adipose tissue within the interatrial septum.1–3 It has been suggested that LHAS is associated with an increased incidence of supraventricular dysrhythmias, sudden death, obesity, abnormal P waves on electrocardiography, and advanced age.1–5 Whereas initial descriptions of this entity were from autopsy studies,1–3,5 in vivo diagnosis of LHAS has been described using transthoracic echocardiography (TTE).4,6–8 Recently, it was found that transesophageal echocardiography (TEE) may be superior to TTE in the detection of abnormalities associated with the atrial septum.9 Thus, we hypothesized that TEE would be a superior technique to detect LHAS in a referral population and would enable the characterization of a group of patients with this entity.

The aortic homograft has become the replacement valve of choice in the treatment of complicated endocarditis involving native and prosthetic aortic valves. Complications are rare, typically involving chronic leaflet degeneration causing valvular insufficiency or rarely chronic calcific stenosis. We present a case in which functional stenosis of the homograft valve was caused by compression and distortion by blood transmitted directly from the left ventricle into a space between the homograft and an external cavity formed by a Dacron wrap. The latter had been placed to help control suture-line bleeding. This case presentation demonstrates an unusual cause of homograft failure and suggests that wrapping of a homograft conduit by native aorta or an external Dacron wrap is not a substitute for meticulous surgical technique to assure a hemostatic suture line.

Left ventricular hypertrophy (LVH) is frequently present in patients referred for radionuclide ventriculography (RVG) for evaluation of left ventricular function. During interpretation of these studies, the nuclear medicine physician may have the subjective impression that increased septal thickening is present because of the abnormally prominent separation of the right and left ventricular blood pools. To examine the diagnostic reliability of this finding, we retrospectively reviewed the RVG studies of 43 consecutive patients and correlated the finding of subjectively increased septal thickness with established echocardiographic (ECHO) criteria and commonly used electrocardiographic (ECG) indices of LVH. Using standard ECHO measurements of septal thickness as a gold standard, RVG interpretation of septal thickening demonstrated a sensitivity of 0.69, specificity of 0.70, and accuracy of 0.70. When compared with standard ECG criteria for LVH, RVG performed quite favorably in the diagnosis of LVH confirmed by ECHO left ventricular mass index. We conclude that scintigraphic evidence of LVH should be reported when RVG studies are interpreted.

Right aortic arch is a rare congenital anomaly associated with abnormal development of the paired embryological aortic arches. While various abnormalities of the great vessels have been described using both first-pass and multigated radionuclide ventriculographic studies, diagnosis of a right-sided aortic arch has typically required a radiographic contrast technique. We present a case of a patient with a suspected right-sided aortic arch diagnosed by radionuclide methods.

Le myxome est une tumeur cardiaque primitive bénigne rare, qui peut être à l’origine de complications vasculaires. Des symptômes neurologiques peuvent précéder ou accompagner le diagnostic de myxome par embolisation systémique survenant le plus souvent dans la circulation cérébrale.Nous rapportons un cas de myxome cardiaque compliqué d’anévrismes cérébraux et révélé par un accident ischémique cérébral chez une patiente de 21 ans. Dans le cadre du bilan étiologique, l’échocardiographie transthoracique a objectivé le myxome cardiaque. Devant le haut potentiel emboligène, la masse a été excisée et la valve mitrale réparée chirurgicalement. L’évolution postopératoire fût favorable.Bien que le myxome soit une tumeur histologiquement bénigne, son évolution peut être marquée par plusieurs complications graves comme les embolies systémiques et la mort subite.Myxoma is a rare benign primary cardiac tumor. It may cause vascular complications. Neurological symptoms may precede or accompany the diagnosis of myxoma with systemic embolization occurring most often in the cerebral circulation.We report a case of cardiac myxoma complicated with cerebral aneurysms and revealed by an ischemic stroke in a 21-year-old patient. The cardiac myxoma was discovered during the transthoracic echocardiography performed as part of the etiological work-up. Because of the high risk of embolism, the mass was resected and mitral valve surgically repaired. The postoperative outcome was uneventful.Although myxoma is a benign tumor histologically, it can lead to serious complications such as systemic embolism and sudden death.

False-positive findings suggestive of metastatic functioning thyroid carcinoma have been well documented in I-131 whole-body imaging. These artifacts are often associated with contamination from radioiodine-containing body secretions. Recently, a contaminated handkerchief on an iodine-131 whole-body scan was reported. In the following study, two additional cases involving the sequestration of I-131 contaminated handkerchiefs in patients' pockets are presented, and the literature regarding these false-positive findings is reviewed. Although rare, this "radioactive handkerchief sign" may cause serious misinterpretation of a focal radioiodine accumulation.

A case of idiopathic pulmonary fibrosis with multiple areas of mismatch on ventilation-perfusion lung imaging in the absence of pulmonary embolism is presented. Idiopathic pulmonary fibrosis is one of the few nonembolic diseases producing a pulmonary ventilation-perfusion mismatch. In this condition, chest radiographs may not detect the full extent of disease, and xenon-133 ventilation imaging may be relatively insensitive to morbid changes in small airways. Thus, when examining patients with idiopathic pulmonary fibrosis, one should be aware that abnormal perfusion imaging patterns without matching ventilation abnormalities are not always due to embolism. In this setting, contrast pulmonary angiog-raphy is often needed for accurate differential diagnosis.