Daniela Blaschke

Atrial fibrillation-induced cardiac troponin I release

BACKGROUND Cardiac troponin I (cTnI) is highly specific for myocardial damage and for the diagnosis of acute coronary syndrome. We investigated cTnI utility and predictive value in patients with atrial fibrillation (AF) in the acute setting. METHOD We studied 354 consecutive patients with the primary diagnosis of AF and clinical symptoms suggestive of myocardial ischemia presenting to our emergency department. cTnI was obtained on presentation. Patients with ST-segment elevation myocardial infarction were excluded. Coronary angiography was performed in 100 patients. RESULTS cTnI was elevated (>0.09 μg/L) in 51 of 354 (15%) patients. The mean cTnI in these patients was 0.37 μg/L (0.09-3.14). In 23 of 100 patients undergoing coronary angiography, cTnI was elevated. Only 6 of these 23 patients (26%) had significant stenosis. In 77 of 100 patients undergoing coronary angiography, cTnI was normal, revealing significant stenosis in 25 patients (33%). The positive predictive value of elevated cTnI for a coronary intervention was 26% and the negative predictive value was 68%. Using multivariate logistic regression, we found that heart rate on presentation, the presence of angina pectoris, left ventricular ejection fraction, serum creatinine, and hemoglobin independently predicted elevated cTnI level. CONCLUSION These data are the first to show that AF in the acute setting is frequently associated with cTnI elevations. AF patients with high heart rate and/or angina pectoris often show false elevated cTnI levels. These findings are relevant for clinicians evaluating patients with acute AF and myocardial ischemia symptoms. Appropriate clinical guidelines must be established that also consider AF-related elevations in cTnI.

Patients with Fabry Disease after Enzyme Replacement Therapy Dose Reduction and Switch–2-Year Follow-Up

Because of the shortage of agalsidase-β supply between 2009 and 2012, patients with Fabry disease either were treated with reduced doses or were switched to agalsidase-α. In this observational study, we assessed end organ damage and clinical symptoms with special focus on renal outcome after 2 years of dose-reduction and/or switch to agalsidase-α. A total of 89 adult patients with Fabry disease who had received agalsidase-β (1.0 mg/kg body wt) for >1 year were nonrandomly assigned to continue this treatment regimen (regular-dose group, n=24), to receive a reduced dose of 0.3-0.5 mg/kg and a subsequent switch to 0.2 mg/kg agalsidase-α (dose-reduction-switch group, n=28), or to directly switch to 0.2 mg/kg agalsidase-α (switch group, n=37) and were followed-up for 2 years. We assessed clinical events (death, myocardial infarction, severe arrhythmia, stroke, progression to ESRD), changes in cardiac and renal function, Fabry-related symptoms (pain, hypohidrosis, diarrhea), and disease severity scores. Determination of renal function by creatinine and cystatin C-based eGFR revealed decreasing eGFRs in the dose-reduction-switch group and the switch group. The Mainz Severity Score Index increased significantly in these two groups (P=0.02 and P<0.001, respectively), and higher frequencies of gastrointestinal pain occurred during follow-up. In conclusion, after 2 years of observation, all groups showed a stable clinical disease course with respect to serious clinical events. However, patients under agalsidase-β dose-reduction and switch or a direct switch to agalsidase-α showed a decline of renal function independent of the eGFR formula used.

Polymorphic ventricular tachycardia in a patient with herpes encephalitis

We present a patient with polymorphic ventricular tachycardia and subsequent ventricular fibrillation with acquired long QT syndrome secondary to herpes encephalitis.

Findings and outcome of fluoroscopic visualization of the oesophageal course during catheter ablation of atrial fibrillation

AIMS The close topographic relationship between the left atrial posterior wall (LAPW) and the oesophagus creates a potential hazard of thermal lesions to the oesophagus during radiofrequency (RF) catheter ablation of atrial fibrillation (AF). The purpose of the study was to describe topographic relation of the oesophagus behind the left atrium in the ablation situation, and to evaluate the clinical outcome of subsequent modifications to the strategy using continuous real-time fluoroscopic visualization of the oesophageal course. METHODS AND RESULTS In 214 consecutive patients, a gastric tube (GT) was inserted before circumferential pulmonary vein isolation (CPVI) for the treatment of paroxysmal (n= 160) or persistent (n= 54) AF. In the real-time mapping situation at the LAPW, the tissue interface between catheter tip and oesophagus lumen measured only 2.9 ± 1.9 mm, and 2.5 ± 1.2 mm at the level of the upper and lower pulmonary vein (PV) ostia, respectively. Modifications of the intended antral CPVI approach due to an oesophageal course close to the left or right PV ostia (in 76.6% of patients) were associated with reduced success rate (sustained sinus rhythm) after one (54.9 vs. 72.0%, P = 0.03), or 1-3 ablation procedures (85.4 vs. 96.0%, P = 0.04) during a mean follow-up of 13 ± 10 months. CONCLUSION Continuous real-time fluoroscopic visualization using a GT emphasizes the very small distance of the catheter tip and oesophageal lumen that may be present in the real-ablation situation and may help to avoid RF lesion application in close proximity to the oesophagus. However, accordant modification of AF ablation strategy may reduce efficacy of the procedure.

Endomyocardial biopsy in Anderson–Fabry disease: The key in uncertain cases

a Dept. of Cardiology, Charite, Campus Virchow Klinikum (CVK), Berlin, Germany b Dept. of Cardiology, Hospital Universitario Puerta de Hierro, Madrid, Spain c Dept. of Cardiology, Deutsches Herzzentrum Berlin (DHZB), Berlin, Germany d Dept. of Nephrology, Charite, Campus Virchow Klinikum (CVK), Berlin, Germany e Berliner Zentrum fur Regenerative Therapien (BCRT), Campus Virchow Klinikum (CVK), Berlin, Germany f Deutsches Zentrum fur Herz Kreislaufforschung (DZHK) — Standort Berlin/Charite, Germany

Fabry disease under enzyme replacement therapy—new insights in efficacy of different dosages

Background Fabry patients on reduced dose of agalsidase-beta or after switch to agalsidase-alfa show a decline in estimated glomerular filtration rate (eGFR) and an increase of the Mainz Severity Score Index. Methods In this prospective observational study, we assessed end-organ damage and clinical symptoms in 112 patients who had received agalsidase-beta (1.0 mg/kg) for >1 year, who were (i) non-randomly assigned to continue this treatment regime (regular-dose group, n = 37); (ii) received a reduced dose of agalsidase-beta and subsequent switch to agalsidase-alfa (0.2 mg/kg) or a direct switch to 0.2 mg/kg agalsidase-alfa (switch group, n = 38); or (iii) were re-switched to agalsidase-beta after receiving agalsidase-alfa for at least 12 months (re-switch group, n = 37) with a median follow-up of 53 (38-57) months. Results eGFR of patients in the regular-dose group remained stable. Patients in the switch group showed an annual eGFR loss of - 4.6  ±  9.1 mL/min/1.73 m2 (P < 0.05). Patients in the re-switch group also had an eGFR loss of - 2.2  ±  4.4 mL/min/1.73 m2 after re-switch to agalsidase-beta, but to a lower degree compared with the switch group (P < 0.05). Patients in the re-switch group suffered less frequently from diarrhoea (relative risk 0.42; 95% confidence interval 0.19-0.93; P = 0.02). Lyso-Gb3 remained stable in the switch (P = 0.97) and the regular-dose (P = 0.48) groups, but decreased in the re-switch group after change of the therapy regimen (P < 0.05). Conclusions After switch to agalsidase-alfa, Fabry patients experienced a continuous decline in eGFR, while this decline was attenuated in patients who were re-switched to agalsidase-beta. Decreasing lyso-Gb3 levels may indicate a better treatment response in the latter group.

Third-degree AV block sensitive to prednisolone 72 hours post AVNRT ablation

A patient developed a transient first‐degree AV block during a radiofrequency ablation of an atrioventricular nodal reentrant tachycardia. Three days later the patient presented with a third‐degree AV block. It resolved within 24 h under antiphlogistic therapy. Patient was asymptomatic without necessity for pacemaker implantation at 12 months follow‐up.

Segment-by-segment assessment of left ventricular myocardial affection in Anderson-Fabry disease by non-enhanced T1-mapping

Background Anderson-Fabry disease (AFD) is an X-linked lysosomal enzyme disorder associated with an intracellular accumulation of sphingolipids, which shorten myocardial T1 relaxation times. Myocardial affection, however, varies between different segments. Purpose To evaluate the specific segmental distribution and degree of segmental affection in AFD patients. Material and Methods Twenty-five patients with AFD, 14 patients with hypertrophic cardiomyopathy (HCM), and 21 controls were included. A Modified Look-Locker Inversion Recovery sequence (MOLLI) was used for non-enhanced T1 mapping at 1.5 T in addition to standard cardiac imaging in 10–12 short axis views. T1 values were evaluated with a mixed model ANOVA and regression analysis to determine the best diagnostic cutoff values for T1 for each myocardial segment. Results Regression analysis showed the best diagnostic cutoff compared to controls in cardiac segments 1–4, 8–9, and 14. Mean differences between T1 for AFD versus HCM were greatest in segment 3, 4, and 9 (99 ms, 103 ms, 86 ms, respectively). Overall T1 times were 888 ± 70 ms and 903 ± 14 ms (AFD with and without LVH); 1014 ± 17 ms and 1001 ± 22 ms (HCM and controls, P < 0.05). Conclusion Myocardial segments are affected by a varying degree of T1 shortening in AFD patients. Segment-specific cutoff values allow the most specific detection and quantification of the extent of myocardial affection.