Intramyocardial plasmid-encoding human vascular endothelial growth factor A165/basic fibroblast growth factor therapy using percutaneous transcatheter approach in patients with refractory coronary artery disease (VIF-CAD).

BACKGROUND: VIF-CAD randomized, placebo-controlled, double-blind trial was an attempt to induce therapeutic angiogenesis by percutaneous intramyocardial transfer of bicistronic (vascular endothelial growth factor/fibroblast growth factor [VEGF/FGF]) plasmid (pVIF) in patients with refractory heart ischemia. Myocardial perfusion, clinical symptoms, exercise tolerance, left ventricular function, and safety were assessed. METHODS: Fifty-two patients with refractory coronary artery disease were randomized to receive VEGF/FGF plasmid (n = 33) or placebo plasmid (n = 19) into myocardial region showing stress-induced perfusion defects. Repeat stress and rest technetium Tc 99m sestamibi single-photon emission computed tomography at 5 months was the primary efficacy measure. Secondary assessment included Canadian Cardiovascular Society class and exercise tolerance at 5 and 12 months. RESULTS: Rest- and stress-induced perfusion defects did not differ between groups. Canadian Cardiovascular Society functional class improved after 5 (P = .0210) and 12 months (P = .0607) in the treatment group. The exercise tolerance of treated patients improved: total exercise time increased marginally (P = .0541); maximum workload (P = .0419) and total test distance (P = .0473) increased significantly, compared to placebo. CONCLUSION: Bicistronic VEGF/FGF plasmid therapy did not improve myocardial perfusion measured by single-photon emission computed tomography. However, treated patients experienced improvement with respect to exercise tolerance and clinical symptoms. Intramyocardial VEGF/FGF bicistronic plasmid transfer seemed safe throughout the follow-up period of 1 year.

Sudden death in hypertrophic cardiomyopathy: old risk factors re-assessed in a new model of maximalized follow-up.

AIMS: in hypertrophic cardiomyopathy (HCM), the following five risk factors have a major role in the primary prevention of sudden death (SD): family history of SD (FHSD), syncope, massive wall thickness (MWTh) >30 mm, non-sustained ventricular tachycardia (nsVT) in Holter monitoring of electrocardiography, and abnormal blood pressure response to exercise (aBPRE). In HCM, as a genetic cardiac disease, the risk for SD may also exist from birth. The aim of the study was to compare the survival curves constructed for each of the five risk factors in a traditional follow-up model (started at the first presentation of a patient at the institution) and in a novel follow-up model (started at the date of birth). In an additional analysis, we compared the survival rate in three subgroups (without FHSD, with one SD, and with two or more SDs in a family). METHODS AND RESULTS: a total of 1306 consecutive HCM patients (705 males, 601 females, mean age of 47 years, and 193 patients were <18 years) evaluated at 15 referral centres in Poland were enrolled in the study. In a novel method of follow-up, all the five risk factors confirmed its prognostic power (FHSD: P = 0.0007; nsVT: P < 0.0001; aBPRE: P = 0.0081; syncope: P < 0.0001; MWTh P> 0.0001), whereas in a traditional method, only four factors predicted SD (except aBPRE). In a novel model of follow-up, FHSD in a single episode starts to influence the prognosis with a delay to the fifth decade of life (P = 0.0007). Multiple FHSD appears to be a very powerful risk factor (P < 0.0001), predicting frequent SDs in childhood and adolescence. CONCLUSION: the proposed concept of a lifelong calculated follow-up is a useful strategy in the risk stratification of SD. Multiple FHSD is a very ominous risk factor with strong impact, predicting frequent SD episodes in the early period of life.

Implantable cardioverter-defibrillator in patients with hypertrophic cardiomyopathy: efficacy and complications of the therapy in long-term follow-up.

INTRODUCTION: Although implantable cardioverter-defibrillators (ICDs) are used in sudden cardiac death (SCD) prevention in high-risk patients with hypertrophic cardiomyopathy (HCM), long-term results as well as precise risk stratification are discussed in a limited number of reports. The aim of the study was to assess the incidence of ICD intervention in HCM patients with relation to clinical risk profile. METHODS AND RESULTS: We studied 104 consecutive patients with HCM implanted in a single center. The mean age of study population was 35.6 (SD, 16.2) years with the average follow-up of 4.6 (SD, 2.6) years. ICD was implanted for secondary (n = 26) and primary (n = 78) prevention of SCD. In the secondary prevention group, 14 patients (53.8%) experienced at least 1 appropriate device intervention (7.9%/year). In the primary prevention (PP) group appropriate ICD discharges occurred in 13 patients (16.7%) and intervention rate was 4.0%/year. Nonsustained VT was the only predictive risk factor (RF) for an appropriate ICD intervention in the PP (positive predictive value 22%, negative predictive value 96%). No significant difference was observed in the incidence of appropriate ICD discharges between PP patients with 1, 2, or more RF. Complications of the treatment included: inappropriate shocks (33.7%), lead dysfunction (12.5%), and infections: 4.8% of patients. Four patients died during follow-up. CONCLUSION: ICD therapy is effective in SCD prevention in patients with HCM, although the complication rate is significant. Nonsustained ventricular tachycardia seems to be the most predictive RF for appropriate device discharges. Number of RF did not impact the incidence of appropriate ICD interventions.

Repeat percutaneous mitral balloon valvuloplasty for patients with mitral valve restenosis.

OBJECTIVES: To determine immediate and long-term clinical outcome, as well prognostic factors in patients who underwent repeat percutaneous mitral balloon valvuloplasty (PMBV). BACKGROUND: Repeat PMBV may be a method of treatment for symptomatic patients with restenosis after successful initial PMBV, but data regarding its long term safety and efficacy are scarce. METHODS: The study group consisted of 67 patients (mean age 52.1 ± 10.5 years). All PMBV procedures were performed using the Inoue balloon system. RESULTS: Repeat PMBV resulted in significant increase in MVA from 1.17 ± 0.16 cm(2) to 1.63 ± 0.22 cm(2) (P < 0.001). Good immediate result (MVA ≥1.5 cm(2) , mitral regurgitation ≤2) was obtained in 52 (77.6%) patients and was not predicted by any analyzed factors. During follow-up (mean time 4.9 ± 2.9 years) six patients died, nine underwent mitral valve replacement, four-third PMBV, and four developed heart failure. The 3-, 5-, and 8-year good functional results (survival free of mitral valve replacement, third PMBV or heart failure ≥ NYHA III) by Kaplan-Meier estimates were 89.3, 75.6, and 52.6%, respectively. These results were significantly superior in patients with good immediate results and echo score <7. In the entire population multivariate Cox regression analysis identified echo score <7 and absence of prior surgical commissurotomy as the independent predictors of event-free survival. CONCLUSIONS: Repeat PMBV is safe and provides good immediate results in patients with restenosis after successful first procedure. Long-term results of repeat PMBV are satisfactory and related mainly to the echo score and quality of the procedure.

Long-term follow-up of children and young adults treated with implantable cardioverter-defibrillator: the authors' own experience with optimal implantable cardioverter-defibrillator programming.

AIMS: Young implantable cardioverter-defibrillator (ICD) recipients present a high rate of inappropriate interventions. Some of them are caused by suboptimal pre-discharge programming of the device. There are conflicting data as regards antitachycardia pacing (ATP) effectiveness in children and young adults. We report our experience with ICD programming and a rate of complications during a 10 year follow-up. METHODS AND RESULTS: We analysed the use and effectiveness of ATP and complications rate in 63 patients aged 6-21 years. Antitachycardia pacing (burst or ramp) was programmed ON in 14 patients (22%), 49 patients (78%) had only ventricular fibrillation (VF) therapy when discharged after implantation. The incidence of effective vs. ineffective or harmful ATP therapy: 5% of patients vs. 19% of patients differed significantly (P < 0.05). Fourteen patients (22%) received > or =1 appropriate shock(s) for ventricular tachycardia/VF and 17 patients (27%) had one or multiple inappropriate therapy (IT). Inappropriate therapy resulted from T-wave over-sensing (nine patients), sinus tachycardia (three patients), fast atrial fibrillation (five patients), and lead insulation disruption (1%). Reprogramming of the device eliminated IT in all cases. There were 13 (21%) surgical complications. Serious psychological sequelae developed in 27 (43%) patients. There was one death during the follow-up period. CONCLUSION: Antitachycardia pacing therapy is rarely effective and often harmful in young ICD recipients. In most patients, programming ICD for only VF therapy is sufficient. Routine pre-discharge programming against inappropriate interventions (especially T-wave over-sensing) helps to reduce the incidence of discharges during the follow-up. The incidence of complications and inappropriate therapies is high in young ICD recipients and affects 50% of patients.

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.

Autophagy in transition from hypertrophic cardiomyopathy to heart failure.

Endomyocardial biopsy of a patient in transition stage from hypertrophic cardiomyopathy to heart failure was investigated. The tissue showed hypertrophy, atrophy of myocytes and an increased amount of fibrosis. In addition, numerous cardiomyocytes revealed ubiquitin positive inclusions. Ultrastructural analysis indicated that cardiomyocytes contained typical autophagic vacuoles including mitochondria, glycogen granules, degraded remnants and myelin structures. The most obvious ultrastructural finding was the presence of amorphous plaques and tubulofilamentous inclusions. Such ultrastructural abnormalities allow us to conclude that degeneration of myocardial cells by autophagy mechanisms leads to cardiomyocyte death, loss and heart failure.

Intracoronary infusion of bone marrow-derived selected CD34+CXCR4+ cells and non-selected mononuclear cells in patients with acute STEMI and reduced left ventricular ejection fraction: results of randomized, multicentre Myocardial Regeneration by Intracoronary Infusion of Selected Population of Stem Cells in Acute Myocardial Infarction (REGENT) Trial.

AIMS: Comparison of intracoronary infusion of bone marrow (BM)-derived unselected mononuclear cells (UNSEL) and selected CD34(+)CXCR4(+) cells (SEL) in patients with acute myocardial infarction (AMI) and reduced <40% left ventricular ejection fraction (LVEF). METHODS AND RESULTS: Two hundred patients were randomized to intracoronary infusion of UNSEL (n = 80) or SEL (n = 80) BM cells or to the control (CTRL) group without BM cell treatment. Primary endpoint: change of LVEF and volumes measured by magnetic resonance imaging before and 6 months after the procedure. After 6 months, LVEF increased by 3% (P = 0.01) in patients treated with UNSEL, 3% in patients receiving SEL (P = 0.04) and remained unchanged in CTRL group (P = 0.73). There were no significant differences in absolute changes of LVEF between the groups. Absolute changes of left ventricular end-systolic volume and left ventricular end-diastolic volume were not significantly different in all groups. Significant increase of LVEF was observed only in patients treated with BM cells who had baseline LVEF < median (37%). Baseline LVEF < median and time from the onset of symptoms to primary percutaneous coronary intervention > or = median were predictors of LVEF improvement in patients receiving BM cells. There were no differences in major cardiovascular event (death, re-infarction, stroke, target vessel revascularization) between groups. CONCLUSION: In patients with AMI and impaired LVEF, treatment with BM cells does not lead to a significant improvement of LVEF or volumes. There was however a trend in favour of cell therapy in patients with most severely impaired LVEF and longer delay between the symptoms and revascularization.

Comparison between maximal left ventricular wall thickness and left ventricular mass in patients with hypertrophic cardiomyopathy.

BACKGROUND: Cardiovascular magnetic resonance enables accurate and reproducible assessment of left ventricular (LV) dimensions and function, free of geometric assumptions and limitations related to an inadequate acoustic window. In patients with hypertrophic cardiomyopathy (HCM), LV mass (LVM) and maximal LV wall thickness (MLVWT) have prognostic significance. AIM: To compare MLVWT and LVM in patients with HCM. METHODS: The study population included 33 patients with HCM (17 males, mean age 48.5 +/- 16.5 years). Subjects after alcohol septal ablation or surgical myectomy were excluded from the study. The MLVWT and LVM were measured with the use of cardiac magnetic resonance. The MLVWT was determined with the use of the dedicated software in short axis slices after manual definition of endocardial and epicardial contours. The LVM was indexed for body surface area and expressed in g/m(2). Cut-off values for normal, mildly increased and markedly increased LVM were based on previously published studies. RESULTS: Mean LVM in the whole study group was 107.4 +/- 30.9 g/m(2) (range 57.0-163.4 g/m(2)) and was higher in males than females (120.2 +/- 30.8 g/m(2) vs 93.8 +/- 25.3 g/m(2), respectively; p = 0.01). Mean MLVWT was 23.4 +/- 4.8 mm (range 16-36 mm). There was only a weak trend toward higher MLVWT in men when compared to women (24.8 +/- 5.4 mm vs 21.9 +/- 3.7 mm, respectively; p = 0.09). There was no correlation between LVM and MLVWT (r = 0.24; p = 0.17). A significant variability in LVM was observed in subjects with similar MLVWT; a greater than two-fold difference was noted in extreme cases. In three patients (9%; one female, two male) LVM was within the normal range and in another one female (3%) patient LVM was mildly increased. In the remaining patients (n = 29; 88%) markedly increased LVM was observed. CONCLUSIONS: The MLVWT does not reflect the degree of LV hypertrophy in patients with HCM. Patients with similar MLVWT may have substantial differences in LVM. A substantial group of patients with HCM is characterised by normal, or only mildly increased LVM, despite significant LV wall hypertrophy measured as MLVWT.

Cardiac magnetic resonance imaging in patients with Fabry's disease.

Fabry's disease (FD) is a rare hereditary disorder caused by the loss of alpha galactosidase A activity leading to accumulation of glycosphingolipids in various organs including hypertrophy of the heart. Most reports on cardiac involvement in FD focus on the left ventricular hypertrophy (LVH) and its relation to diastolic function. However, recent studies demonstrated large subset of patients with FD and right ventricle (RV) hypertophy. The accurate depiction of RV volumes, function and mass is possible with cardiovascular magnetic resonance (CMR). The CMR study can be also used to identify typically localised regions of intramyocardial fibrosis (infero-lateral segments of the LV), which have been shown to be a marker of inefficacious response to enzyme replacement therapy. We present series of 8 patients with genetically confirmed FD who underwent CMR study. We demonstrated a typical concentric and diffuse pattern of LVH with RV involvement in patients with the most severe LVH without significant impact on RV function and volumes. We showed that myocardial fibrosis can be observed not only in LV but also in RV. In 2 patients FD coexisted with symptomatic coronary artery disease with evidence of subendocardial myocardial fibrosis typical for ischaemic origin in one patient. The CMR confirmation of the presence of FD in one patient at an early stage of the disease, before the onset of advanced hypertrophy or failure of other organs, supports the value of this imaging technique in differential diagnosis of concentric and diffuse LVH.

Paced ventricular electrogram fractionation predicts sudden cardiac death in hypertrophic cardiomyopathy.

AIMS: Paced electrogram fractionation analysis (PEFA) has been assessed for the prediction of sudden cardiac death (SCD) in a large-scale, prospective study of patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: We determined the positive predictive value (PPV) of PEFA in relation to other risk factors for SCD and outcomes in 179 patients with HCM and no prior history of cardiac arrest. Patients were followed over a mean 4.3 years (range: 1.1-6.3 years). Thirteen patients had SCD-equivalent events: four of these patients died suddenly, three were resuscitated from ventricular fibrillation (VF), and six had implantable cardioverter-defibrillator (ICD) discharges in response to VF. PEFA identified nine of these patients and another 14 non-VF patients yielding a censored PPV of between 0.19 and 0.59 that was greater than the PPV that was the formal stopping point of the trial (0.18). Eighty per cent of patients were followed for 4 years or more. The PPV for the identification of SCD in this group was 0.38 (0.17-0.59). The use of two or more conventional markers to predict SCD identified five patients with SCD-equivalent events in the 4-year follow-up group and 42 other patients without events yielding a PPV of 0.106 (confidence limits 0.02-0.15). CONCLUSION: PEFA identifies HCM patients at risk of SCD with greater accuracy than non-invasive techniques and may have an important role in determining indications for ICD prescription.

Left ventricular diastolic function assessed with cardiovascular magnetic resonance imaging and exercise capacity in patients with non-obstructive hypertrophic cardiomyopathy.

BACKGROUND: In patients with non-obstructive hypertrophic cardiomyopathy (HCM) and preserved left ventricular (LV) systolic function, diastolic dysfunction is one of the major factors contributing to limited exercise capacity. Cardiovascular magnetic resonance (CMR) imaging has become a useful tool in diagnosis, risk stratification and treatment monitoring in patients with HCM. AIM: To assess the relationship between simple CMR parameters of LV diastolic function at rest and exercise capacity measured by means of cardiopulmonary exercise testing on a treadmill in patients with non-obstructive HCM and preserved LV systolic function. METHODS: The study included 13 patients with non-obstructive HCM and preserved LV systolic function who underwent cardiopulmonary exercise testing on a treadmill and CMR within 1 month. Analysed parameters of diastolic function included: LV mass index (LVMI), peak filling rate normalised to LV stroke volume index (PFR/LVSVI) and time from the end-systole to PFR normalised to heart rhythm (TPFR). RESULTS: There was a significant correlation between PFR/LVSVI at rest and peak oxygen uptake (V02peak) (r=0.64, p=0.02). Patients with V02peak below median (<30 ml/kg/min) had a significantly lower PFR/LVSVI than patients with higher V02peak [5.12 m2/s, interquartile range (IQR) 4.16-6.82 vs. 7.93 m2/s, IQR 7.49-8.21 respectively, p=0.035]. LVMI, TPFR were not related to exercise capacity. There was also no correlation between V02peak and age (r=-0.38, p=0.19), LV ejection fraction (r=-0.36, p=0.22) or normalised LV volume indices: LVEDVI (r=0.09, p=0.76), LVESVI (r=0.34, p=0.26). CONCLUSIONS: Assessment of LV diastolic function by peak filling rate normalised to stroke volume index by means of CMR at rest in patients with non-obstructive HCM and preserved LV systolic function is a useful marker of exercise capacity.

[Typical magnetic resonance features of cardiac fibroma--a case report]. / Typowy obraz wlókniaka serca w badaniu rezonansu magnetycznego--opis przypadku.

Cardiac tumors are rare. Magnetic resonance allows non-invasive, detailed evaluation of myocardial tissue and differential diagnosis. We present a case of a young man with unexplained cardiac mass and typical magnetic resonance features of a left ventricular fibroma.

Long term exercise capacity in patients with hypertrophic cardiomyopathy treated with percutaneous transluminal septal myocardial ablation.

BACKGROUND: In hypertrophic obstructive cardiomyopathy, percutaneous transluminal septal myocardial ablation (PTSMA) improves functional capacity in the short term. However, long term functional capacity is unknown. AIM: To assess the long term exercise capacity of patients with hypertrophic obstructive cardiomyopathy undergoing PTSMA. METHODS: Twenty three patients (56.5% male, mean age 44.5+/-13.6 years) who underwent PTSMA were included. All patients had also undergone a symptom limited cardiopulmonary exercise treadmill test before the procedure, then after 3 months (early follow-up) and after a mean 7.2+/-1.0 years (long term follow-up). RESULTS: Before PTSMA, mean maximal pressure gradient in the left outflow tract (LVOTGmax) was 82+/-29 mmHg, 17 patients had NYHA functional class> or = III and peak oxygen uptake (pVO2) was 18+/-4 ml/kg/min. PTSMA led to a reduction in mean LVOTGmax (to 29+/-19 mmHg, p<.0001), improvement of heart failure symptoms (NYHA> or =III in 1 patient, p<.0001) and an increase of pVO2 (to 22+/-6 ml/kg/min, p=.0002) at short term. LVOTGmax, functional class and pVO2 did not change significantly during long term follow-up compared to early follow-up. However, there was a continuous improvement in percentage predicted pVO2 over time. CONCLUSIONS: In patients with hypertrophic obstructive cardiomyopathy and symptoms of heart failure, PTSMA leads to stable long term improvement of objectively measured exercise capacity.

Cardioverter-defibrillator lead-related thrombus treated with prolonged anticoagulation in patient with prothrombotic disorder.

Following an accidental finding of a large atrial lead-related thrombus in a 33-year-old woman with suspected prothrombotic disorder, a conservative treatment with low molecular weight heparin injections and close transthoracic and transesophageal echocardiographic monitoring was used. It led to a complete resolution of the thrombus in 4 weeks without the need for a high-risk operation in this patient.

[Angioplasty of the unprotected left main coronary artery stenosis with standby cardiopulmonary support--a case report]. / Angioplastyka pnia lewej tetnicy wiencowej w zabezpieczeniu krazeniem pozaustrojowym.

Stenosis of the unprotected left main coronary artery (LMCA) is a classical indication for coronary artery bypass graft surgery (CABG). Percutaneous coronary intervention (PCI) of LMCA may be an alternative to surgical treatment if atherosclerosis of distal segments is very advanced. The periprocedural risk is high, especially if comorbidities are present. However, long-term results remain unclear. The ongoing Syntax trial will clarify whether angioplasty of LMCA with drug-eluting stents can be equivalent to CABG. We present a case of a patient with occluded right coronary artery, severe stenoses of the LMCA, left anterior descending artery and left circumflex artery, and poor left ventricular ejection fraction in whom PCI for stenosis of unprotected LMCA with standby cardiopulmonary support was performed.

[Coronary venoplasty during the implantation of the cardiac resynchronisation device--a case report]. / Plastyka balonowa zyly wiencowej w trakcie wszczepiania ukladu resynchronizujacego. Opis przypadku.

We describe a case of a 76-year-old patient with advanced block in the His-Purkjnie system, who underwent implantation of a cardioverter-defibrillator with cardiac resynchronisation therapy. Complete heart block occurred during the insertion of the coronary sinus cathether. The implantation of the left ventricular (LV) lead was impossible due to difficult anatomy of the coronary venous system. The right ventricular pacing caused the further prolongation of the QRS duration and exacerbation of heart failure symptoms. Thus, the LV lead was successfully implanted during the second procedure after the balloon venoplasty of the lateral cardiac vein. The biventricular pacing was successful during 4 months follow-up.

Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy.

OBJECTIVES: In this study we assessed the long-term efficacy and safety of disopyramide for patients with obstructive hypertrophic cardiomyopathy (HCM). BACKGROUND: It has been reported that disopyramide may reduce left ventricular outflow gradient and improve symptoms in patients with HCM. However, long-term efficacy and safety of disopyramide has not been shown in a large cohort. METHODS: Clinical and echocardiographic data were evaluated in 118 obstructive HCM patients treated with disopyramide at 4 HCM treatment centers. Mortality in the disopyramide-treated patients was compared with 373 obstructive HCM patients not treated with disopyramide. RESULTS: Patients were followed with disopyramide for 3.1 +/- 2.6 years; dose 432 +/- 181 mg/day (97% also received beta-blockers). Seventy-eight patients (66%) were maintained with disopyramide without the necessity for major non-pharmacologic intervention with surgical myectomy, alcohol ablation, or pacing; outflow gradient at rest decreased from 75 +/- 33 to 40 +/- 32 mm Hg (p < 0.0001) and mean New York Heart Association functional class from 2.3 +/- 0.7 to 1.7 +/- 0.6 (p < 0.0001). Forty other patients (34%) could not be satisfactorily managed with disopyramide and required major invasive interventions because of inadequate symptom and gradient control or vagolytic side effects. All-cause annual cardiac death rate between disopyramide and non-disopyramide-treated patients did not differ significantly, 1.4% versus 2.6%/year (p = 0.07). There was also no difference in sudden death rate, 1.0%/year versus 1.8%/year (p = 0.08). CONCLUSIONS: Two-thirds of obstructed HCM patients treated with disopyramide could be managed medically with amelioration of symptoms and about 50% reduction in subaortic gradient over >/=3 years. Disopyramide therapy does not appear to be proarrhythmic in HCM and should be considered before proceeding to surgical myectomy or alternate strategies.

[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.

The value of cardiac magnetic resonance and distribution of late gadolinium enhancement for risk stratification of sudden cardiac death in patients with hypertrophic cardiomyopathy.

BACKGROUND: The presence of late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM) is associated with worse clinical outcome and the extent of LGE predicts the increased risk of sudden cardiac death (SCD). Limited data exist regarding the distribution of LGE. We attempted to verify whether the presence of LGE outside the interventricular insertion points carries additional risk for patients with HCM. METHODS: In this prospective study, 328 patients with HCM, who underwent cardiac magnetic resonance (CMR) were enrolled. Five major risk factors for SCD were assessed in all patients. The median follow-up was 37 months. RESULTS: LGE was detected in 226 (68.9%) patients. In 70 (21.3%) patients it was present only at the interventricular insertion points - LGE (+) group, while in 156 (47.6%) it was noted in other locations - LGE (++) group. Primary endpoint defined as SCD or appropriate implantable cardioverter-defibrillator intervention occurred in 14 (4.3%) patients, one in LGE (+) and 13 in LGE (++). In multivariable analysis including five traditional risk factors and left ventricular ejection fraction <50%, only the presence of LGE outside the insertion points was a significant predictor of SCD/aborted SCD (HR 10.01, 95% CI 1.21-83.86, p=0.033). The performance of the multivariable sudden cardiac death risk model was improved by the addition of LGE (++) to the traditional risk factors (likelihood ratio p=0.005). The Kaplan-Meier curves showed better event-free survival in the LGE (-) and LGE (+) patient groups compared to the LGE (++) group. CONCLUSIONS: In HCM patients, presence of LGE outside interventricular insertion points is associated with increased risk of sudden cardiac death or its equivalent as well as overall mortality. Cardiac fibrosis as a substrate for SCD in HCM may be identified on CMR and serve as an imaging biomarker of increased risk.