J wave and ST segment elevation in the inferior leads: a latent type of variant Brugada syndrome?

In a patient referred for the evaluation of non-sustained monomorphic ventricular tachycardia on Holter recordings, ventricular fibrillation was electrically induced during electrophysiologic study. Despite the absence of structural heart diseases, his ECG revealed J wave and ST segment elevation in the inferior leads, which showed circadian variation and were augmented by the sodium channel blocker, pilsicainide. This case might lead us to notice a new concept, a 'latent' type of variant Brugada syndrome, and these ECG findings and changes might serve as its diagnostic sign.     

ST-segment elevation in precordial leads: anterior or right ventricular myocardial infarction?

Isolated acute right ventricular (RV) infarction is rare, and ECG diagnosis may be difficult. We report two cases of acute myocardial infarction with ST-segment elevation in anterior precordial leads caused by such an RV involvement. Potential mechanisms for the relationship are given.     

Total occlusion of inferior vena cava in a patient with antiphospholipid antibody syndrome associated with behçet's disease

Beh?et's disease frequently involves the venous system, usually affecting small vessels, but sometimes large vessels such as the vena cava. Antiphospholipid antibody syndrome is associated with an increased incidence of arterial and venous thrombosis. A 29-year-old male with Beh?et's disease developed bilateral leg edema secondary to thrombotic occlusion of the inferior vena cava. Laboratory tests revealed positive antiphospholipid antibodies and lupus anticoagulant. Treatment with steroid and warfarin subsequent to intravenous administration of uro-kinase resulted in improvement of symptoms. The association of antiphospholipid antibody syndrome and Beh?et's disease may have caused the total thrombotic occlusion of the vena cava in this case.     

Assessment of coronary morphology and flow in a patient with Guillain-Barré syndrome and ST-segment elevation

Patients with Guillain-Barré syndrome often have cardiac disturbances as a manifestation of autonomic dysfunction. Such abnormalities consist of arrhythmias and disturbances of heart rate and blood pressure. We report a case of a patient with Guillain-Barré syndrome who developed ST-segment elevation in the inferolateral leads, suggestive of an acute coronary syndrome. Cardiac catheterization revealed angiographically normal coronary arteries. Intracoronary ultrasound was also normal. Intracoronary Doppler flow measurements revealed an elevated baseline coronary flow velocity of up to 41 cm/s and decreased coronary flow reserve, particularly in the left circumflex artery. Myopericarditis as cause of the electrocardiographic changes could be ruled out by echocardiography and endomyocardial biopsy. We postulate that the intracoronary Doppler findings are caused by autonomic dysfunction with decrease of coronary resistance and redistribution of the transmural myocardial blood flow.     

Impressive ST-segment elevation during dobutamine stress echocardiography in a patient with normal coronary arteries: dobutamine-induced vasospasm?

ST-segment elevation during dobutamine stress echocardiography is a serious complication usually related to severe coronary artery disease. However, it can occur in absence of significant coronary artery disease supposedly as a consequence of an occlusive dobutamine-induced coronary artery spasm. We report the case of a 56-year-old man without cardiovascular history who presented during a dobutamine stress echocardiography an intense precordial pain along with an impressive 10-mm ST elevation and in whom urgent catheterization documented the absence of significant coronary artery stenoses.     

15-year-old patient with decompensated heart failure and tachycardia with negative P-waves in inferior ECG recordings. An indication for heart transplantation?

A 15-year-old girl was admitted with signs of severe cardiac failure. There were no symptoms of cardiac insufficiency 4 weeks before hospital admission. She presented with permanent supraventricular tachycardia with negative P-waves in leads II, III and aVF, the heart rate was 150 beats per minute. The electrophysiological examination showed a permanent junctional reentry tachycardia. A postero-septal accessory pathway could be eliminated successfully by radiofrequency catheter ablation. Immediately after the procedure cardiac function deteriorated with slight decrease of the strongly reduced cardiac output. Intensive care and application of dobutamine led to clinical stability. During a follow-up of two years the young patient showed permanent sinus rhythm and an age related physical strain. This case report documents the rapid and severe manifestation of cardiac failure owing to permanent junctional reentry tachycardia in a 15-year-old girl. She was referred for consideration of heart transplantation. Invasive electrophysiological treatment led to permanent sinus rhythm with improvement of left ventricular function.     

Long-term effect of β-blocker in ST-segment elevation myocardial infarction in patients with preserved left ventricular systolic function: a propensity analysis

The current guidelines for acute myocardial infarction (AMI) recommended that β-blocker should be used in patients with decreased left ventricular (LV) systolic function for long-term period. However, the effect of β-blocker in AMI patients with preserved LV systolic function is uncertain. We sought to assess the long-term effect of β-blocker in AMI patients with preserved LV systolic function. During the follow-up period (1997–2011), total 3508 patients were performed percutaneous coronary intervention (PCI). Of these patients, 424 AMI patients with preserved LV systolic function [ejection fraction (EF) > 40 %] were analyzed. Median follow-up period was 4.7 years. Then, patients were divided into two groups (β-blocker group 197 patients and no-β-blocker group 227 patients). However, there are substantial differences in baseline characteristics between two groups. Therefore, we calculated propensity score to match the patients in β-blocker and no-β-blocker groups. After post-match patients (N = 206, 103 matched pair), β-blocker therapy significantly reduced cardiac death compared with no-β-blocker [hazard ratio (HR) 0.40, p = 0.04], whereas β-blocker therapy was not associated with major adverse cardiac events (MACE) and all-cause death. β-Blocker is an effective treatment for AMI patients who underwent PCI with preserved LV systolic function.     

Severe thrombocytopenia caused by digitoxin intoxication in a patient with heart failure associated with Sjögren's syndrome

Congestive heart failure (CHF) related to Sj?gren's syndrome is extremely rare. This report concerns a patient who presented with CHF and severe thrombocytopenia (5,000/microl). Serum concentrations of K, Mg and digitoxin were 3.2mmol/L, 1.4mg/L and 57.2ng/ml, respectively. Digitoxin intoxication was evident, seemingly evoked by hypokalemia, hypomagnesemia, hepatorenal dysfunction and hypothyroidism. The severe thrombocytopenia was considered to have been caused by this intoxication, as it disappeared soon after the digitoxin was discontinued and potassium was supplemented.     

A case report of a right ventricular mass in a patient with Beh?et’s disease: Myxoma or thrombus?

IntroductionBehçet’s disease (BD) is a multi-system, chronic and relapsing disorder classified as “vasculitic syndrome”. It typically affects young adult females between 20 and 40 years of age. There are some typical clinical manifestations associated with this disease, however, at times; rare sign and symptoms pose a challenge to the treating physician and making a definitive diagnosis. Presentations with cardiac symptoms are one of the extremely rare manifestations of the Behçet’s disease.MethodsThe authors present clinical, laboratory and imaging findings of a patient who presented with a cardiac mass which was the first presenting feature or manifestation of Behçet’s disease.ResultsA 19-year-old boy was admitted to our hospital for the investigation of “fever of unknown origin”, weight loss, shortness of breath and a scrotal ulcer of recent on-set. X-ray chest and electrocardiograms were inconclusive. Transthoracic echocardiography revealed a right ventricular (RV) mass attached to the interventricular septum measuring 1.5 × 1.5 cms (Panel A). Cardiac MRI identified it as a RV Myxoma. In addition, on CT scan of the chest pulmonary embolism was noted. The patient underwent excision biopsy of the tumor under cardiopulmonary bypass via right atriotomy (Panel B). Histopathology of the mass described it as “an organizing thrombus with a few groups of interrupted myocardial fibers and some infiltration of lymphocytes and plasma cells”. Moreover his HLA typing was found positive for HLA-B51 (5). In view of the above findings and associated lesions, the patient was diagnosed as a case of Behçet’s disease. The medical management included immunosuppressant and anticoagulation.ConclusionBehçet’s disease, even in the absence of the typical clinical features, should be considered in the differential diagnosis of right ventricular mass, especially when dealing with young adults from the Mediterranean basin and the Middle-East.     

Left ventricular noncompaction in a patient with fabry disease: overdiagnosis, morphological manifestation of fabry disease or two unrelated rare conditions in the same patient?

We report a clinical case of a young female with Fabry disease but without left ventricular hypertrophy, which fulfills the diagnostic criteria of left ventricular noncompaction (LVNC). To our knowledge, this is the first report of LVNC in a patient with Fabry disease. The possibility of an overdiagnosis of LVNC is discussed based on the limitations of the current diagnostic criteria. This case was further investigated by genetic analysis, which came to demonstrate the limited usefulness of genetic testing in the diagnosis of LVNC. Assuming a true trabecular pattern of LVNC, the hypothesis that the same patient has two unrelated and rare conditions, although possible, is unlikely. The genetic and clinical heterogeneity of LVNC is discussed and supports, along with this clinical case, the hypothesis that LVNC is a morphological expression of different diseases rather than a distinct cardiomyopathy. Accordingly, LVNC could be a rare cardiac manifestation of Fabry disease.     

Functional asplenia in a patient with Rothmann-Makai syndrome: a pathogenetic relationship?

SIR, A 27-yr-old woman was referred for evaluation of mild anaemia,leucocytosis and thrombocytosis. History revealed a diagnosisof Rothmann–Makai syndrome (lipogranulomatosis subcutanea),a rare variant of Weber–Christian disease, at the ageof 13 yr. At that time the patient had presented with a minorfracture of the left ankle, and when the plaster was removedshe was found to