Failure of Femoral Access to Electrophysiological Evaluation Due to Aplasia of the Inferior Vena Cava.

We report an interesting case of a man with a persistent left superior vena cava (PLSVC) with left azygos vein who underwent electrophysiological evaluation. Further evaluation revealed congenital dilated azygos vein, while a segment connecting the inferior vena cava (IVC) to the hepatic vein and right atrium was missing. The azygos vein drained into the superior vena cava, and the hepatic veins drained directly into the right atrium. The patient did not have congenital anomalies of the remaining thoracoabdominal vasculature.

Continuous cardiac troponin I release in Fabry disease.

BACKGROUND: Fabry disease (FD) is a rare lysosomal storage disorder also affecting the heart. The aims of this study were to determine the frequency of cardiac troponin I (cTNI) elevation, a sensitive parameter reflecting myocardial damage, in a smaller cohort of FD-patients, and to analyze whether persistent cTNI can be a suitable biomarker to assess cardiac dysfunction in FD. METHODS: cTNI values were determined at least twice per year in 14 FD-patients (6 males and 8 females) regularly followed-up in our centre. The data were related to other parameters of heart function including cardiac magnetic resonance imaging (cMRI). RESULTS: Three patients (21%) without specific vascular risk factors other than FD had persistent cTNI-elevations (range 0.05-0.71 ng/ml, normal: <0.01). cMRI disclosed late gadolinium enhancement (LGE) in all three individuals with cTNI values ≥0.01, while none of the 11 patients with cTNI <0.01 showed a pathological enhancement (p<0.01). Two subjects with increased cTNI-values underwent coronary angiography, excluding relevant stenoses. A myocardial biopsy performed in one during this procedure demonstrated substantial accumulation of globotriaosylceramide (Gb3) in cardiomyocytes. CONCLUSION: Continuous cTNI elevation seems to occur in a substantial proportion of patients with FD. The high accordance with LGE, reflecting cardiac dysfunction, suggests that cTNI-elevation can be a useful laboratory parameter for assessing myocardial damage in FD.

Cardiac Troponin I elevation after epileptic seizure.

BACKGROUND: Cardiac troponin-I (cTNI) is highly specific biomarker to prove myocardial damage, e.g. in acute coronary syndrome (ACS). However, it occurs in other conditions as well. We therefore analysed cTNI increase in patients after generalized convulsive seizure. METHODS: Consecutive patients admitted with acute generalized convulsive seizure were included in case of cTNI measurement on admission. Among 898 selected cases, 53 patients were referred secondary to our department; in 845 cases cTNI measurements on admission were available. In case of multiple admissions (81 cases), only the first admission entered our analysis. In 17 patients elevated cTNI was determined due to ACS; in one patient a myocarditis was found. 5 patients suffered of relevant renal insufficiency. Finally 741 patients were included in the analysis. A cTNI cut-off level of ≥ 0.1 ng/ml was considered. Factors associated with a cTNI increase were analysed subsequently. RESULTS: The mean age of the study population (n = 741) was 47.8 years (SD ± 18.6), 40.9% were female. In 50 patients (6.7%) a cTNI elevation of unknown origin was found; no obvious cardiac involvement could be detected in these patients who all remained asymptomatic. A vascular risk profile (including at least hypertension, hypercholesterolemia or diabetes) (OR = 3.62; CI: 1.59 to 8.21; p = 0.001) and elevated creatine kinase on admission (OR = 2.36; CI: 1.26 to 4.39; p = 0.002) were independent factors associated with cTNI release. CONCLUSION: cTNI release occurs in patients with generalized convulsive seizure with predominance in patients with vascular risk profile.

Cerebral hemodynamic failure presenting as limb-shaking transient ischemic attacks.

Limb-shaking transient ischemic attacks (TIA) may occur in patients with insufficient brain perfusion due to an underlying occlusive disease. We present the case of a 64-year-old patient who suffered from repetitive TIA presenting with shaking movements of the right-sided extremities and accompanying speech arrest. Symptoms are documented in the online supplementary video (www.karger.com/doi/10.1159/000327683). These episodes were frequently triggered in orthostatic situations. The diagnosis of limb-shaking TIA was established. The diagnostic workup revealed pseudo-occlusion of the left internal carotid artery, a poor intracranial collateral status and, as a consequence, an exhausted vasomotor reserve capacity. At ultrasound examination, symptoms were provoked by a change of the patient's position from supine to sitting. During evolvement of symptoms, a dramatic decrease of flow velocities in the left middle cerebral artery was observed. This case thus documents the magnitude and dynamics of perfusion failure in a rare manifestation of cerebral ischemic disease.

Persistent increase in cardiac troponin I in Fabry disease: a case report.

BACKGROUND: Hypertrophic cardiomyopathy is a frequent manifestation in Fabry disease (FD) - an X-linked lysosomal storage disorder caused by reduced activity of the enzyme α-galactosidase A. In FD an elevation of specific cardiac biomarkers, such as cardiac troponin I (cTNI) has been reported in case of clinical manifestation suggestive of myocardial ischemia. In diagnosing acute myocardial infarction cTNI is considered the most reliable parameter. CASE PRESENTATION: In the referred case we present a 59 years old female patient with the diagnosis of FD presenting with persistently increased cTNI level (lowest value 0.46 ng/ml, highest value 0.69 ng/ml; normal range <0.05 ng/ml) over a period of 5 months lacking cardiac clinical signs. Since renal insufficiency did not explain the degree of cTNI elevation, this was interpreted as a result of cardiac involvement in FD. Cardiac MRI showed marked left ventricular hypertrophy and focal late Gadolinium enhancement. CONCLUSIONS: Our case report demonstrates a persistent cTNI release in FD with cardiac involvement. Proving the persistence in a symptom free interval, it might be related to a direct damage of myocytes. In FD cTNI could serve as a beneficial long term parameter providing new perspectives for screening strategies.

Randomized comparison of multipolar, duty-cycled, bipolar-unipolar radiofrequency versus conventional catheter ablation for treatment of common atrial flutter.

INTRODUCTION: Radiofrequency (RF) catheter ablation has been established as an effective and curative treatment for atrial flutter (AFL). Approved methods include a drag-and-drop method, as well as a point-by-point ablation technique. The aim of this study was to compare the acute efficacy and procedural efficiency of a multipolar linear ablation catheter with simultaneous energy delivery to multiple catheter electrodes against conventional RF for treatment of AFL. METHODS: Patients presenting to our department with symptomatic, typical AFL were enrolled consecutively and randomized to conventional RF ablation with an 8-mm tip catheter (ConvRF) or a duty-cycled, bipolar-unipolar RF generator delivering power to a hexapolar tip-versatile ablation catheter (T-VAC) group. For both groups, the procedural endpoint was bidirectional cavotricuspid isthmus block. RESULTS: Sixty patients were enrolled, 30 patients each assigned to ConvRF and T-VAC groups. Total procedure time (40.2 ± 15.8 min vs 60.5 ± 12.7 min), energy delivery time (8.5 ± 3.7 min vs 14.7 ± 5.2 min), radiation dose (14.5 ± 3.5 cGy/cm² vs 31.7 ± 12.1 cGy/cm²), and the minimum number of RF applications needed to achieve block (4.2 ± 2.4 vs 8.9 ± 7.2) were significantly lower in the T-VAC group. In 7 patients treated with the T-VAC catheter, bidirectional block was achieved with less than 3 RF applications, versus no patients with conventional RF energy delivery. CONCLUSION: The treatment of typical AFL using a hexapolar catheter with a multipolar, duty-cycled, bipolar-unipolar RF generator offers comparable effectiveness relative to conventional RF while providing improved procedural efficiency.