[Cardiac abnormalities in patients with myotonic dystrophy--preliminary results]. / Zaburzenia kardiologiczne u pacjentów z dystrofia miotoniczna--doniesienie wstepne.

INTRODUCTION: Myotonic dystrophy (DM) is an inherited multisystem disorder associated with myotonia, progressive skeletal muscle weakness and atrophy, involvement of peripheral and central nervous system and sudden death likely due to atrioventricular block and/or ventricular arrhythmia. AIM OF THE STUDY: to assess the type and degree of cardiac and neurological involvement in patients (pts) with DM. MATERIALS AND METHODS: 10 pts (6 male), in mean age of 35 +/- 13 years, treated for DM type I (DM1)--7 pts and type II (DM2)--3 pts. All pts underwent a neurological examination including muscle strength assessment as well as cardiac diagnostics including: standard and 48-hour ambulatory electrocardiogram, echocardiographic examination, magnetic resonance imaging (MRI) of the heart and late potentials assessment. RESULTS: Muscle strength was moderately diminished (46-48 points in MRC sub score) in 3 pts with DM1 and mildly diminished (56-58 points in MRC sub score) in 2 pts with DM2. These patients showed clinical symptoms of myopathy. Cardiovascular examinations revealed: QRS duration above 110 ms in 5 pts, clinically significant supraventricular arrhythmia or atrioventricular block in 3 pts, focal myocardial fibrosis in 3 pts, asymmetric hypertrophy of inter-ventricular septum in 1 patient, presence of late potentials in 5 pts. We have not observed correlation between impaired muscle strength and cardiac abnormalities. However, most pronounced cardiac abnormalities were observed in 2 male DM1 patients with clinical symptoms of myopathy and lowest MRC score. At a mean follow up of 3.2 +/- 1.4 years none of the pts died. CONCLUSIONS: Cardiac involvement in pts with myotonic dystrophy is frequent and is characterized by phenotypic heterogeneity. Detection of cardiac abnormalities may require extensive diagnostics. The most important is the assessment of ECG. Cardiac and neurological abnormalities vary in intensity between patients without close relationship to each other.

Ventricular fibrillation risk factors in over one thousand patients with accessory pathways.

BACKGROUND: Published data concerning risk factors of VF in WPW patients are inconsistent or contradictory. METHODS AND RESULTS: We included 1007 patient (pts) (mean age 35 years; 45% female) with an accessory pathway (AP) referred for non pharmacological treatment. Group 1 consisted of 56 pts (42M, aged 34 ± 15 yrs) with an AP and documented VF and Group 2-951 pts (513M, aged 35 ± 15 yrs) with an AP and without VF. Univariate predictors of VF were: overt pre-excitation, male gender, multiple AP, large AP. Multivariate predictors were: overt pre-excitation, male gender and MAP. The mean shortest pre-excited RR interval during AF was significantly shorter in Group 1: 205 ± 27 vs. 243 ± 64, P=0.019. VF as an end point of the first arrhythmia episode (AVRT or AF) was observed in 20 pts (15M, 5F). Primary VF (no documented arrhythmia prior to aborted SCD) occurred in 16 pts (13M, 3F). The mean age of primary VF pts was significantly lower than of pts with history of AVRT or AVRT and/or AF (24.5 vs. 36.5 vs. 38 yrs., P<0.005 and P=0.002, respectively). Age at VF occurrence shows a bi-modal distribution with peak occurrences in the 2-nd/3-rd and 5-th decades. CONCLUSION: In patients with an accessory pathway, overt pre-excitation, male gender and multiple AP constitute independent risk factors of VF episodes. Young patients in the 2-nd/3-rd and older patients in the 5-th decade might be at higher risk of VF occurrence.

Relationship between cycle length of typical atrial flutter and double potential interval after achievement of complete isthmus block.

BACKGROUND: There is some disagreement concerning the minimal value of the interval between components of double potentials (DPs interval) that allows distinguishing complete and incomplete block in the cavotricuspid isthmus (CTI). OBJECTIVES: To assess clinical utility of the relationship between atrial flutter cycle length (AFL CL) and the DPs interval. METHODS: Ablation of the CTI was performed in 87 patients during AFL (245 ± 40 ms). Subsequently, DPs were recorded during proximal coronary sinus pacing at sites close to a gap in the ablation line and after achievement of complete isthmus block. RESULTS: We noted strong correlation between AFL CL and the DPs interval after achievement of isthmus block (r = 0.73). The mean DPs interval was 95.3 ± 18.3 ms (range 60-136 ms) and 123.3 ± 24.3 ms (range 87-211 ms) during incomplete and complete isthmus block, respectively (P < 0.001). When expressed as a percentage of AFL CL, this interval was 35.7 ± 3.5% AFL CL (range 28-40.2%) and 50.4 ± 6.9% AFL CL (range 39-72%) during incomplete and complete isthmus block, respectively (P < 0.001). A cutoff value of 40% of AFL CL identified CTI block with 96.7% sensitivity and 100% specificity. CONCLUSIONS: The interval between DPs after achievement of block in the CTI correlates with AFL CL. The DPs interval expressed as a percentage of AFL CL allows better distinguishing between complete and incomplete isthmus block compared to standard method based on milliseconds. The DPs interval below 40% of AFL CL indicates sites close to a gap in the ablation line.

Predictors of the atrial fibrillation occurrence in patients with Wolff-Parkinson-White syndrome.

BACKGROUND: Atrial fibrillation (AF) in WPW syndrome occurs earlier and is more common than in the general population. AIM: To evaluate the predisposing factors for the first episode of AF in patients with WPW. METHODS: We analysed data on 930 patients (510 males, 420 females) with WPW treated in our centre during 1988-2007. AF was diagnosed in 236 patients (25% - 161 males, 75 females, aged 36 +/- 15 years). The AF group was divided into two subgroups - patients with AF and atrio-ventricular reentrant tachycardia (AVRT), and patients with AF only. The analysis included subjects' age and gender, the presence of AVRT, the number and properties of accessory pathways, left ventricular ejection fraction (LVEF) and concomitant cardiovascular diseases. RESULTS: The groups did not differ in terms of concomitant diseases and LVEF. In the whole group of patients with AF, arrhythmia occurred earlier in men than in women (34 +/- 14 vs. 40 +/- 15 years of age, p = 0.013). In the subgroup with AF and AVRT, AF was documented earlier compared to patients with AF only (34 +/- 15 vs. 41 +/- 15 years of age, p = 0.0072). AVRT was more common in patients with AF compared to those without AF (69 vs. 53%, p < 0.001). In the whole group of 930 patients, AF was observed more often in patients with overt pre-excitation compared to concealed WPW (29 vs. 12%, p < 0.001). CONCLUSIONS: In patients with WPW syndrome, AF occurs earlier in patients with AVRT compared to patients with AF and without documented AVRT, earlier in men compared to women, and is more common in patients with overt WPW.

[Pseudo atypical atrial flutter or atrial tachycardia dependent on complex substrate in a patient with univentricular heart after palliative operations - mapping and ablation]. / Rzekomo atypowe trzepotanie przedsionków czy czestoskurcz przedsionkowy zalezny od zlozonego podloza u chorej z sercem jednokomorowym po operacjach paliatywnych - opis ablacji.

We describe a case of persistent atrial tachycardia/flutter in a 19-year old female with corrected transposition of great arteries (ccTGA) and dual inlet left ventricle (DILV), treated with surgical palliative operations. The arrhythmia became persistent and symptomatic with dyspnea and severe cyanosis. During the EP study, the right atrial isthmus-dependent reentry was identified. In electroanatomical maps large areas of low voltage and electrical silence were localised. Due to these areas of slow conduction the isthmus dependent arrhythmia had long CL. Linear RF applications closed the isthmus, resulting in flutter termination. During 3 months of follow-up the patient remained free of arrhythmia.

['Weak' and 'strong' palpitations in a girl with atrioventricular nodal reentrant tachycardia--a case report]. / Napady "slabego" lub "mocnego" kolatania serca u chorej z nawrotnym czestoskurczem wezlowym.

We present a case of a 16-year-old girl with AVNRT with 1:1 or 2:1 conduction from the atria to ventricles which was perceived by the patient as two different types of palpitations.

The clinical course and risk in patients with pseudo-Mahaim fibers.

BACKGROUND: Pseudo-Mahaim (AP-M) fibers are a rare variant of atrioventricular (AV) accessory pathways. Atriofascicular and atrioventricular accessory connections are characterized by slow conduction and decremental properties. Dual physiological AV node pathways, slow and fast, are observed in a large number of patients with AP-M. Therefore, there is substrate for AV nodal reentrant tachycardia (AVNRT) in addition to antidromic AV reentrant tachycardia (AVRT) with left bundle branch block (LBBB)-like morphology. Other arrhythmia such as atrial fibrillation (AF) or atrial flutter (AFL) and ventricular fibrillation (VF) are also observed. We analysed the occurrence of arrhythmias in a group of patients with AP-M treated in our department. METHODS: We evaluated 27 patients (12 women) aged 14-53 years (mean age 25.6 years) with AP-M. The clinical course in these patients, in particular with regard to the occurrence of arrhythmias, was analysed. Patients with dual AV node properties were compared to patients without such findings. RESULTS: We found dual AV node properties in 18 patients (Group 1), while 9 patients had fast pathway only (Group 2). Twenty-six patients presented with AVRT, 2 patients with AVNRT, 3 patients with AF, 1 patient with AT, 2 patients with AFL, and 3 patients with VF. In 2 patients, AP-M were seen in an atypical area. In one patient, the pathway connected the right atrium with the left ventricle (septal region), and in the other patient it connected the left atrium with the left ventricle (left anterior region). CONCLUSIONS: The majority of AP-M was right-sided. Two thirds of patients with AP-M had anatomical substrate for AVNRT (fast/slow pathway AV node). VF or asystole occurred in 10% of patients.

[Right atrial flutter or tachycardia?]. / Trzepotanie czy czestoskurcz prawoprzedsionkowy?

We present a case of 67-year-old patient with recurrent persistent atrial macroreentry after surgical removal of left atrial myxoma. The macroreentry was cavo-tricuspid isthmus dependent with a pseudo-atypical atrial flutter morphology and variable cycle lengths between 290 and 340 ms.

Evaluation of the QRS-T angle using the high-resolution 64-lead electrocardiography.

OBJECTIVE: The aim of the study was to evaluate the influence of the number of electrocardiogram (ECG) leads on the diagnostic value of TCRT (spatial QRS-T angle) parameter (12 standard ECG leads and 61 surface ECG leads were used). The TCRT parameter, which describes the spatial QRS-T angle, is a useful indicator of the risk of ventricular tachycardia (VT) and sudden cardiac death (SCD). It is usually calculated from standard 12 leads ECG. METHODS: The TCRT parameter was calculated from the three virtual orthogonal leads obtained by singular value decomposition of the averaged ECG signals. Sensitivity and specificity of TCRT parameter in identifying VT patients were tested on two groups of patients after myocardial infarction: 13 non - VT patients and 30 VT patients. Additionally 17 healthy volunteers were studied as a control group. RESULTS: Mean value (+/-SD) of TCRT parameter calculated for 61 leads was -0.80+/-0.27 for VT patients and 0.27+/-0.46 for non VT patients. For 12 standard leads TCRT mean value was -0.80+/-0.22 for VT patients and 0.27+/-0.49 for non VT patients. Sensitivity for VT patients was 87% (61 leads) and 83% (12 leads). Specificity in non-VT group was 100% for both lead sets. CONCLUSIONS: Results of the study show distinct differences in the TCRT parameter values between VT patients and non VT patients for both lead sets. The sensitivities of the TCRT parameter obtained for 61 leads and for 12 standard leads were comparable.

[The vein of Marshall as a source of various types of atrial arrhythmias, including adrenergic paroxysmal atrial fibrillation--successful catheter ablation]. / Zyla Marshalla--ograniczone zródlo adrenergicznego migotania przedsionków--skuteczna ablacja podloza.

We describe a case of a 44-year old man with recurrent atrial arrhythmias (atrial ectopy, atrial tachycardia and fibrillation) originating from the vein of Marshall (VoM). Stress, exertion and isoproterenol increased frequency and duration of highly symptomatic arrhythmia paroxysms. In 2002 he underwent two sessions of RF ablation. In the area of the left inferior pulmonary vein ostium (LIPV) and along the course the VoM, spiky, firing potentials were recorded. Ablation of the VoM region induced high frequency focal firing (CL approximately 200 ms) with 1: 1 VoM-->LA conduction. Before sinus rhythm return, a gradual increase of conduction of VoM-->LA block was observed. During the second session residual breakthroughs were recorded. During 5 years of follow-up the patient remained free from arrhythmia without antiarrhythmic drugs.

[Wide QRS complex tachycardia in a patient after inferior myocardial infarction and CABG -- the role of electrophysiological study and RF ablation]. / Czestoskurcz z szerokimi zespolami QRS u pacjenta po zawale serca i pomostowaniu aortalno-wiencowym - rola badania elektrofizjologicznego i ablacji RF.

We present a case of 53-year-old patient who experienced paroxysms of wide QRS complex tachycardia at a rate of 150 bpm and LBBB morphology 6 years after inferior myocardial infarction and CABG. Anamnesis and morphology of tachycardia could suggest ventricular tachycardia. However during electrophysiological study we observed AVRT with LBBB and "regularly irregular" AVRT with normal QRS complex and changing entrance to the AV node (through slow and fast pathway). In this paper we present our approach and try to explain why the tachycardia using concealed bypass tract appeared so late in life.

[Spectrum of atrial arrhythmias from the right upper pulmonary vein--successful isolation of the arrhythmogenic focus by RF ablation of atriovenous breakthroughs--a case report]. / Zespól uporczywych arytmii z zyly plucnej górnej prawej w postaci licznych pobudzen przedwczesnych, rytmów imitujacych rytm zatokowy, patologicznej bradykardii oraz napadów czestoskurczu--rola ablacji wybiórczej.

We present a case of a 19-year-old patient with incessant arrhythmias originating from the right upper pulmonary vein (RUPV) presenting as frequent premature beats, automatic rhythms mimicking sinus rhythm (SR) and pulmonary vein tachycardias. Morphology of P' wave resembled sinus P wave due to relatively short distance of the ectopic focus from the sinus node. Occasionally, when discharges from the focus were relatively slow (800-500 ms) and regular it was mimicking sinus rhythm. Activation preceding P' wave during arrhythmia was recorded in RUPV as well as in superior vena cava. In this paper we discuss our approach that allowed localising the arrhythmogenic focus in the RUPV. After isolation of the RUPV sinus rhythm was restored with tachycardia at a cycle length of 320 ms continuing in the isolated vein.

[Symptomatic recurrent bradycardia--always an indication for pacemaker implantation? The role of RF ablation for incessant focal tachycardia from AV node region and advanced AV nodal block]. / Objawowa bradykardia--czy zawsze jest wskazaniem do wszczepienia ukladu stymulujacego? Rola ablacji.

We present a case of a 17-year-old patient in whom recurrent bradycardia and syncope episodes were present. During the pathological bradycardia incessant runs of irregular tachycardia from the region of atrial extension of AV node were observed. The bradycardia runs were due to advanced functional AV nodal block. The patient was qualified for pacemaker implantation and antiarrhythmic treatment by his GP. Successful ablation of atrial tachycardia revealed that SA and AV node properties were in the normal range; therefore no pacemaker implantation was needed.

[Application of spectral frequency analysis in atrial fibrillation mechanism assessment]. / Zastosowanie spektralnej analizy czestotliwosciowej do oceny podloza napadowego migotania przedsionków.

Recent studies have demonstrated spatio-temporal organisation in atrial fibrillation (AF), with a highest dominant frequencies (DF) at pulmonary veins ostia (PVo). We present a case of 58-year-old woman with AF evaluated by spectral frequency analysis. Simultaneous recordings at each veno-atrial junction and coronary sinus were obtained. Sequential fast Fourier transforms (FFT) of digitalized signals were performed. FFT profiles were analysed to determine DF. Low DF recorded at right inferior PVo suggested no contribution to AF process. Ablation of high DF PVo and low DF SVC with nonuniform anisotropic conduction resulted in AF termination and good clinical outcome in 1-year follow-up.

[Eleven-years long follow-up in a patient after myocardial infarction, with low ejection fraction and recurrent ventricular tachycardia. The role of implantable cardioverter defibrillator and selective ablation]. / Jedenastoletnia obserwacja pacjenta z przebytym rozleglym zawalem serca, niska frakcja wyrzucania oraz nawracajacymi czestoskurczami komorowymi. Rola kardiowertera-defibrylatora i wybiórczej ablacji.

The selective ablation of the recurrent ventricular tachycardia (VT) in a 75-year old patient after extensive inferior myocardial infarction (24 years ago), with low ejection fraction was performed. In 1995 the cardioverter-defibrillator was implanted due to recurrent, symptomatic VT. The coronary angiography in 1995 and in 2006 revealed the occlusion of the right coronary and the circumflex arteries. One year after implantation, he had electrical storm caused by proarrhythmic effect of amiodarone with prolongation of QT/QTc interval. During follow up episodes of VT (approximately 5/year) were successfully terminated by ATP and rarely by cardioversion. Recently, the patient was admitted to the hospital because of the very frequent (25/day) episodes of slow (500-560 ms), sustained ventricular tachycardia. The pharmacological treatment was unsuccessful. CARTO mapping and entrainment pacing revealed VT circuit around mitral annulus. A few applications at the paraseptal part of the mitral isthmus terminated VT, which was no longer inducible. During following days there were no VTs requiring ICD interventions.

[Ventricular fibrillation in a patient with three accessory pathways, Ebstein anomaly and intermittent long QT interval. RF ablation and electrophysiologic considerations]. / Migotanie komór u chorego z trzema dodatkowymi szlakami przewodzenia, poronna anomalia Ebsteina i okresowo dlugim odstepem QT. Ablacja RF i refleksje elektrofizjologiczne.

We present a case of a 19-year old man with minor Ebstein's anomaly, intermittent long QT interval and WPW syndrome in whom atrial fibrillation, degenerating into ventricular fibrillation was the first symptom. QRS complex morphologies during atrial fibrillation revealed the presence of three accessory pathways (septal, right inferior paraseptal and antero-inferior). Immediately after resuscitation the patient was treated with amiodarone, which resulted in a significant prolongation of QT interval to 700 ms. After RF ablation of accessory pathways patient remains asymptomatic during 6-month follow up, however QTc interval is about 500 ms.

Selective ablation or isolation of all pulmonary veins in atrial fibrillation -- when and for whom?

INTRODUCTION: Targeted treatment of atrial fibrillation (AF) involves circumferential isolation of all pulmonary veins (PV) or isolation of electrical connections within their ostia. Only in some cases are the real localisation and number of triggering foci, the anatomy of venous ostia as well as the form of AF (paroxysmal, persistent, chronic, primary or secondary) taken into consideration. AIM: To compare the results of selective electrical isolation (1-3 PV ostia or ablation of a single focus in other veins or atrium) versus isolation of all pulmonary veins. METHODS: RF ablation was performed in eighty patients (51 men, 29 women) with symptomatic, drug-refractory AF. Fifty-nine patients had paroxysmal AF (PAF), 16 persistent (AFpers), and 5 chronic AF (AFchro). Selective ablation was carried out in those patients who had detectable AF triggers during sinus rhythm -- supraventricular extrasystolic beats (SVEB) of 1 to 3 morphologies (group I). Extended ablation -- isolation of all 4-5 PV -- was performed in patients with multiple SVEB morphologies and heterogeneous electrical connections within all PV (group II). Group I consisted of 60 patients (22 females) aged 46+/-14 years, whereas group II comprised 20 patients (7 females) aged 52+/-13 years. In 24 patients (18 from group I and 6 from group II) with concomitant typical atrial flutter, an ablation line in the cavo-tricuspid isthmus was also performed. Long-term results were assessed 17+/-15.6 (4-105) months after the procedure based on routine ECG, ambulatory 24-hour ECG monitoring, clinical evaluation and regular phone calls. In patients with PAF, left atrial diameter <4.2 cm and evidence of successful ablation, antiarrhythmic agents were withheld. In patients with AFpers and AFchro, antiarrhythmic drugs were discontinued 3 to 6 months after successful ablation. RESULTS: Complete procedural success was achieved in 61 (76%) patients, and significant clinical improvement was observed in another 9 (11%) patients. Effective ablation significantly improved quality of life. In group I the procedure was entirely successful or a marked improvement was reported (single, transient palpitation episodes and/or atrial tachyarrhythmias lasting up to 30 seconds) in 54 (90%) patients. Among 48 (80%) patients with complete success, 25 (42%) did not receive any antiarrhythmic drugs during follow-up, 12 (20%) with arterial hypertension received beta-blockers, and 11 (18%) continued beta-blocker + class I antiarrhythmic drug. In another 6 (10%) patients a significant clinical improvement in arrhythmia control was observed. In Group II the procedure was fully effective or a significant improvement was observed in 16 (80%) patients. Among 13 (65%) patients with complete success, 5 (25%) did not require any antiarrhythmic drugs, 4 (20%) who had hypertension continued beta-blockers, and another 4 (20%) continued beta-blocker + I class antiarrhythmic drug. A significant clinical improvement of arrhythmia control was observed in another 3 (15%) patients. CONCLUSIONS: In patients with a limited number of triggering foci and limited AF substrate, selective ablation effectively eliminates AF with a low risk of complications. Detailed electrophysiological assessment (standard ECG, 12-lead Holter ECG monitoring and endocardial mapping) allows precise identification of this group of patients. In patients with chronic and persistent AF benefits occur with some delay which is associated with a delayed reversal of atrial remodelling.

[Tachycardia with rSr' <110 ms in lead V1 -- is it always an atrio-ventricular nodal tachycardia?]. / Czestoskurcz z zespolem rSr' <110 ms w odprowadzeniu V1 -- czy zawsze wezlowy?

We present a case of a 15-year old girl in whom supraventricular tachycardia (SVT) with rSr' pattern in lead V1 and the QRS width of less than 110 ms, suggesting typical slow-fast AVNRT, was the presenting arrhythmia. During sinus rhythm no preexcitation was observed. Considerable variability of the tachycardia cycle length (240-370 ms), attributable to the presence of the fast and slow nodal pathways, was also observed. However, during the electrophysiological study only orthodromic atrio-ventricular reciprocating tachycardia with the left-sided superior accessory pathway as the retrograde limb was documented. Thus, rSr' pattern was due to incomplete right bundle branch block. We describe how the correct diagnosis and the site of the atrial insertion of the accessory pathway could be inferred from the careful analysis of the P' wave morphology during the tachycardia.

[Removal of thrombus associated with ablation of atrial fibrillation -- the role of intracardiac echocardiography]. / Usuniecie skrzepliny powstalej podczas ablacji podloza migotania przedsionków z zastosowaniem echokardiografii endokawitarnej.

Lef atrial thrombus formation during ablation of atrial fibrillation carries the risk of thrombo-embolic complications. We describe a case of a 55 year old male in whom thrombus formation was detected by intracardiac echocardiography. Thrombus was safely removed by aspiration into transseptal sheath.