Arrhythmogen jobb kamrai dysplasia – a terheléses vizsgálat prognosztikai jelentősége panaszmentes egyénben = Arrhytmogenic right ventricle – prognostic significance of exercise test

A szerzők áttekintik az arrhythmogen jobb kamrai cardiomyopathia/dysplasia témakörével kapcsolatos legfontosabb ismereteket. Igazolt arrhythmogen jobb kamrai cardiomyopathia/dysplasia esetén – tünetmentes egyénekben – a terheléses vizsgálat során észlelt kamrai tachycardia prognosztikus jelentőségéről nem rendelkezünk adatokkal. A szerzők közleményükben egy 25 éves nőbeteg esetét ismertetik, akinél bokasérülés ellátása kapcsán észleltek kóros EKG-t és emiatt küldték kardiológiai vizsgálatra. A fizikális vizsgálat kóros eltérést nem mutatott, az EKG-felvételen normális sinusrhythmus és bal tengelyállás mellett a II, III, aVF- és V2-V6 elvezetésekben negatív T-hullám látszott. A mellkasröntgen és a laboratóriumi vizsgálatok kóros eltérést nem igazoltak. Az echokardiográfia normális bal kamrai ejekciós frakció mellett inferior akinesist és tágabb jobb kamrát igazolt. A terheléses vizsgálat során a beteg 9 MET-et teljesített, a terhelés során előbb sporadikus kamrai extrasystolia, majd 3 percig tartó kamrai tachycardia jelentkezett, amely a terhelés megszakítása és hasprés alkalmazására megszűnt. A szív-MR-vizsgálat normális balkamra-méreteket, falmozgást és ejekciós frakciót igazolt. Az emelkedett jobb kamrai végszisztolés és végdiasztolés volumen mellett a jobb kamrai ejekciós frakció jelentősen csökkent (31,6%), és a jobb kamrának megfelelően akinetikus területek ábrázolódtak. Késői típusú kontraszthalmozódás a bal kamrában nem igazolódott, a vékony falú jobb kamra fala e tekintetben pontosan nem volt megítélhető. Az EKG- és a CMR-eltérések alapján arrhythmogen jobb kamrai cardiomyopathia/dysplasia diagnózisát állították fel. Tekintettel arra, hogy a beteg panaszmentes volt és a családban hirtelen halál nem fordult elő, preventív célzattal gyógyszeres kezelés és/vagy ICD-implantáció nem történt. Három hónappal később a beteg hirtelen meghalt. A sectio jelentősen tág jobb kamrát igazolt, a jobb kamra fala elvékonyodott, amelyben jellegzetes lokalizációban (beáramlási és kiáramlási traktus, valamint a csúcs) az izomszövet helyett felszaporodott zsírszövet volt látható. Az érintett jobb kamra falában a Mallory-féle foszfor-volfrám savas hematoxilinfestéssel fibrosist is igazolni lehetett. A szerzők azt a következtetést vonják le, hogy tünetmentes betegben, igazolt arrhythmogen jobb kamrai cardiomyopathia/dysplasia esetén a terheléses EKG-vizsgálat során provokált kamrai tachycardia rossz prognosztikai jel, és ilyen esetben ICD-implantáció indokolt lehet. Orv. Hetil., 2010, 151, 2145–2149. | The authors summarize the present knowledge on arrhythmogenic right ventricular cardiomyopathy/dysplasia. Limited data are available about natural history of asymptomatic patients with arrhythmogenic right ventricle cardiomyopathy/dysplasia, who have a ventricular tachycardia during exercise test. A 25-year old female patient was treated with osteosynthesis because of ankle injury. Cardiology consultation was performed because of an abnormal ECG. Physical examination was normal. ECG showed a normal sinus rhythm, left axis deviation, negative T waves in leads II, III, aVF and V2-V6. Chest X-ray and laboratory findings were normal. Echocardiography showed normal left ventricular ejection fraction along with inferior akinesis and dilated right ventricle. Bicycle exercise test revealed a good exercise tolerability (9 MET), and after sporadic ventricular extra systoles ventricular tachycardia developed lasting for 3 minutes, which spontaneously stopped after aborting the test and performed abdominal strain. MRI was performed which has shown normal left ventricular size, wall motion and ejection fraction and depressed right ventricle function (ejection fraction 31.6%) enlarged right ventricular end-systolic and diastolic volumes, hypo-akinetic regions without aneurysm and bulging. No contrast enhancement was seen in the thin right ventricular wall. According to abnormal ECG and MRI findings arrhythmogenic right ventricle cardiomyopathy/dysplasia was diagnosed. No ICD implantation was indicated because the patient was asymptomatic, and no sudden cardiac death occurred in the family. Three month later the patient was found dead. At autopsy the right ventricular chamber was markedly enlarged, with multiple translucent areas of fatty accumulation accompanied with extended myocytes loss. There was a characteristic triangle dysplasia: the inflow, outflow tracts and apical areas. The coronaries were free of atherosclerosis. Mallory’s phosphotungstic acid-hematoxilin stain demonstrated the presence of fibrosis within the scattered myocardium. Conclusion: malignant ventricular arrhythmia provoked by exercise test in an asymptomatic arrhythmogenic right ventricle cardiomyopathy/dysplasia patient with negative family history should be an indication for ICD implantation. Orv. Hetil., 2010, 151, 2145–2149

Automated T1 and T2 mapping segmentation on cardiovascular magnetic resonance imaging using deep learning

IntroductionStructural and functional heart abnormalities can be examined non-invasively with cardiac magnetic resonance imaging (CMR). Thanks to the development of MR devices, diagnostic scans can capture more and more relevant information about possible heart diseases. T1 and T2 mapping are such novel technology, providing tissue specific information even without the administration of contrast material. Artificial intelligence solutions based on deep learning have demonstrated state-of-the-art results in many application areas, including medical imaging. More specifically, automated tools applied at cine sequences have revolutionized volumetric CMR reporting in the past five years. Applying deep learning models to T1 and T2 mapping images can similarly improve the efficiency of post-processing pipelines and consequently facilitate diagnostic processes.MethodsIn this paper, we introduce a deep learning model for myocardium segmentation trained on over 7,000 raw CMR images from 262 subjects of heterogeneous disease etiology. The data were labeled by three experts. As part of the evaluation, Dice score and Hausdorff distance among experts is calculated, and the expert consensus is compared with the model’s predictions.ResultsOur deep learning method achieves 86% mean Dice score, while contours provided by three experts on the same data show 90% mean Dice score. The method’s accuracy is consistent across epicardial and endocardial contours, and on basal, midventricular slices, with only 5% lower results on apical slices, which are often challenging even for experts.ConclusionsWe trained and evaluated a deep learning based segmentation model on 262 heterogeneous CMR cases. Applying deep neural networks to T1 and T2 mapping could similarly improve diagnostic practices. Using the fine details of T1 and T2 mapping images and high-quality labels, the objective of this research is to approach human segmentation accuracy with deep learning

Impact of Clinical and Morphological Factors on Long-Term Mortality in Patients with Myocardial Bridge

Although myocardial bridging (MB) has been intensively investigated using different methods, the effect of bridge morphology on long-term outcome is still doubtful. We aimed at describing the anatomical differences in coronary angiography between symptomatic and non-symptomatic LAD myocardial bridges and to investigate the influence of clinical and morphological factors on long-term mortality. In our retrospective, long-term, single center study we found relevant MB on the left anterior descendent (LAD) coronary artery in 146 cases during a two-year period, when 11,385 patients underwent coronary angiography due to angina pectoris. Patients were divided into two groups: those with myocardial bridge only (LAD-MBneg, n = 78) and those with associated obstructive coronary artery disease (LAD-MBpos, n = 68). Clinical factors, morphology of bridge by quantitative coronary analysis and ten-year long mortality data were collected. The LAD-MBneg group was associated with younger age and decreased incidence of diabetes mellitus, as well as with increased minimal diameter to reference diameter ratio (LAD-MBneg 54.5 (13.1)% vs. LAD-MBpos 46.5 (16.4)%, p = 0.016), while there was a tendency towards longer lesions and higher vessel diameter values compared to the LAD-MBpos group. The LAD-MBpos group was associated with increased mortality compared to the LAD-MBneg group. The analysis of our data showed that morphological parameters of LAD bridge did not influence long-term mortality, either in the overall population or in the LAD-MBneg patients. Morphological parameters of LAD bridge did not influence long-term mortality outcomes; therefore, it suggests that anatomical differences might not predict long-term outcomes and should not influence therapy

ARVC vagy myocarditis?

L

Impact of Clinical and Morphological Factors on Long-Term Mortality in Patients with Myocardial Bridge

Although myocardial bridging (MB) has been intensively investigated using different methods, the effect of bridge morphology on long-term outcome is still doubtful. We aimed at describing the anatomical differences in coronary angiography between symptomatic and non- symptomatic LAD myocardial bridges and to investigate the influence of clinical and morphological factors on long-term mortality. In our retrospective, long-term, single center study we found relevant MB on the left anterior descendent (LAD) coronary artery in 146 cases during a two-year period, when 11,385 patients underwent coronary angiography due to angina pectoris. Patients were divided into two groups: those with myocardial bridge only (LAD-MBneg, n = 78) and those with associated obstructive coronary artery disease (LAD-MBpos, n = 68). Clinical factors, morphology of bridge by quantitative coronary analysis and ten-year long mortality data were collected. The LAD-MBneg group was associated with younger age and decreased incidence of diabetes mellitus, as well as with increased minimal diameter to reference diameter ratio (LAD-MBneg 54.5 (13.1)% vs. LAD-MBpos 46.5 (16.4)%, p = 0.016), while there was a tendency towards longer lesions and higher vessel diameter values compared to the LAD-MBpos group. The LAD-MBpos group was associated with increased mortality compared to the LAD-MBneg group. The analysis of our data showed that morphological parameters of LAD bridge did not influence long-term mortality, either in the overall population or in the LAD-MBneg patients. Morphological parameters of LAD bridge did not influence long-term mortality outcomes; therefore, it suggests that anatomical differences might not predict long-term outcomes and should not influence therapy

Impact of respiration gating on image integration guided atrial fibrillation ablation

BACKGROUND: Radiofrequency (RF) catheter ablation guided by electroanatomic mapping systems is an effective therapy for atrial fibrillation. However, it may be affected by respiration movements. The aim of this study was to determine the impact of respiratory gating on procedural parameters in patients undergoing catheter ablation of atrial fibrillation (AF). METHODS AND RESULTS: One-hundred forty consecutive patients undergoing pulmonary vein isolation were admitted to study. Respiratory gating module (AccuResp algorithm, Carto3, Biosense Webster) was enabled in 70 patients and disabled in 70 patients during procedures. Successful pulmonary vein isolation and sinus rhythm were obtained in all patients. A significant reduction in total procedure times [median 77, interquartile range (IQR 66-95) min vs median 82 (IQR 72-104) min, p < 0.05] and fluoroscopy times [median 14 (IQR 9-17) min vs median 16 (IQR 12-22) min, p < 0.05] were observed in the respiratory gated group. Although ablation times (duration between the first and last ablation) were significantly shorter in respiratory gated group [median 37 (IQR 32-53) min vs median 48 (IQR 39-65) min, p < 0.05], total RF application durations were not different between two groups [median 1,554 (IQR 1,213-2,196) s vs median 1,802 (IQR 1,344-2,448) s, p = 0.11]. Difference in electroanatomical map reconstruction times was not significant [median 14 (IQR 12-16) min in gated group vs median 13 (IQR 10-18) min in nongated group, p = 0.19]. CONCLUSION: Respiratory gating significantly improves fluoroscopy and ablation times during electroanatomic mapping guided AF ablation. Respiratory gated maps may provide uninterrupted continuous ablation applications. Furthermore, using automatic respiratory gating module does not prolong mapping times

Survival of Myocardial Infarction Patients with Diabetes Mellitus at the Invasive Era (Results from the Városmajor Myocardial Infarction Registry)

Introduction. Due to the lifelong nature of diabetes mellitus (DM), it has been demonstrated to have significant effects on patients’ morbidity and mortality. The present study aimed to assess the effects of DM on the clinical outcome and survival in patients who underwent percutaneous coronary intervention (PCI) due to myocardial infarction (MI) and to examine the relationship of DM to the type of the MI and to left ventricular (LV) and renal functions. Methods. A total of 12,270 patients with ST-elevation MI (STEMI) or non-ST-elevation MI (NSTEMI) were revascularized at our Institution between 2005 and 2013. In this pool of patients, 4388 subjects had DM, while 7018 cases had no DM. Results. In both STEMI and NSTEMI, the 30-day and 1-year survival were worse in diabetic patients as compared to non-diabetic cases. In the patients with DM, NSTEMI showed worse prognosis within 1-year than STEMI similarly to non-diabetic subjects. Regarding survival, the presence of DM seemed to be more important than the type of MI. Regardless of the presence of DM, reduced LV function was a maleficent prognostic sign and DM significantly reduced the prognosis both in case of reduced and normal LV function. Survival is primarily affected by LV function, rather than DM. Worse renal function is associated with worse 30-day and 1-year survival in both cases with and without DM. Considering different renal functions, the presence of DM worsens both short- and long-term survival. Survival is primarily affected by renal function, rather than DM. Conclusions. The results from a high-volume PCI center confirm significant the negative prognostic impact of DM on survival in MI patients. DM is a more important prognostic factor than the type of the MI. However, survival is primarily affected by LV and renal functions, rather than DM. These results could highlight our attention on the importance of recent DM treatment with new drugs including SGLT-2 inhibitors and GLP-1 antagonists with beneficial effects on survival

A cerebralis paresis előfordulása és a betegdokumentáció minőségi vizsgálata a Semmelweis Egyetem klinikáin

Bevezetés és célkitűzés: A Semmelweis Egyetem klinikáin cerebralis paresis (CP) miatt gondozott gyerekek betegdoku- mentációjának minőségi ellenőrzését végeztük el. Módszer: Retrospektív audit vizsgálatunkban a 2005 és 2015 között született, CP-gyanús gyerekek elektronikus kór- házi rendszerben rögzített adatait revideáltuk, s ennek során 673, igazoltan CP-vel diagnosztizált esetet regisztrál- tunk. A fellelhető adatok alapján elvégeztük az esetek klinikai és etiológiai besorolását, valamint ellenőriztük ezen adatok elérhetőségét a betegdokumentációban. Eredmények: A gyerekek 86%-a rendelkezett a klinikai besorolásra alkalmas dokumentációval. A vizsgálható esetek 90,5%-ában spasticus, 7,8%-ában hypoton, 1,2%-ában dyskineticus és 0,5%-ában ataxiás CP-t írtak le. Az osztályoz- ható spasticus állapotok (az összes spasticus eset 98%-a) között 51% tetraparesis/tetraplegia, 26% diparesis/diplegia és 23% hemiparesis/hemiplegia volt; a maradék 2% topológiai besorolására nem volt elegendő információ a doku- mentációban. Az esetek 82%-ában volt meghatározható a súlyosság a Gross Motor Function Classification System skála szerint, 43%-ban I–II., 28%-ban III., 29%-ban IV–V. fokú mozgáskorlátozottságot véleményeztek. A születési anamnézisre vonatkozó adatokat az esetek 89%-ában találtunk. Koraszülöttség az esetek 55%-ában, perinatalis asphyxia vagy hypoxiás-ischaemiás encephalopathia 31%-ban, agykamrai vagy agyállományi vérzés 27%-ban, ikerter- hesség 19%-ban, méhen belüli sorvadás 18%-ban, intrauterin/perinatalis/csecsemőkori infekció 15%-ban, fejlődési rendellenesség 12%-ban, in vitro fertilisatio 5%-ban, stroke 3%-ban, CP-vel asszociálható genetikai mutáció pedig 3%-ban szerepelt az anamnézisben. A CP rizikótényezőinek szempontjából negatív anamnézist 16%-ban írtak le. Következtetés: Audit vizsgálatunk alapján a CP klinikai leírása egységes kritériumok szerint történt, hiányosságokat a klinikai besorolás és az anamnesztikus adatok rögzítése terén észleltünk. Az adatgyűjtés egységesítése céljából javas- latot teszünk az érintett betegek gondozása során regisztrálandó dokumentációs standardra, mely az egységes beteg- dokumentáció és egy jövőbeli hazai CP-regiszter alapfeltétele