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.

Catheter ablation of atrial fibrillation in patients with hypertrophic cardiomyopathy: atrial fibrillation type determines the success rate.

BACKGROUND: Atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HCM) is generally associated with deterioration of the clinical status, functional capacity, and quality of life. It is also an independent risk factor for stroke and death. Studies evaluating the effectiveness of AF ablation in this cohort are relatively scant, have included relatively few patients, and their results are somewhat conflicting. Thus, the aim of this study was to assess the safety and efficacy of catheter ablation of AF in patients with HCM. METHODS: Thirty patients (10 females; mean age 48.7 ± 11 years) with drug-refractory paroxysmal (n = 14), persistent (n = 7), or long-persistent (> 1 year; n = 9) AF were prospectively recruited into the study. Eleven patients were in New York Heart Association (NYHA) class I, 13 patients were in NYHA class II, and 6 patients were in NYHA class III. Mean atrial volume was 180 ± 47 mL, interventricular septum thickness was 20.5 ± 6.3 mm, and left atrial area was 29.8 ± 6.2 cm2. Ablation protocol was adjusted to the clinical and electrophysiological status of the patients. Pulmonary vein isolation and bidirectional cavo-tricuspid isthmus block were performed in all patients. In addition, left atrial linear lesions were created and complex fragmented atrial potentials were ablated in patients with persistent and long-persistent AF, as well as during repeated procedures. RESULTS: At 12 months, stable sinus rhythm (SR) was present in 16 (53%) patients, significantly more frequently in patients with paroxysmal AF (71% in SR) compared to those with persistent (57.1% in SR) or long-persistent (22% in SR) AF. A significant reduction of AF burden was observed in 85.7% of patients with paroxysmal AF, 71.4% of patients with persistent AF, and 55.5% of patients with long-persistent AF. Single procedure success rate was 33% (10 patients), and repeat ablation procedures were performed in 13 patients. No periprocedural complications occurred. Thromboembolic events were noted in 2 patients with arrhythmia recurrence during the follow-up, including stroke in 1 patient and peripheral embolism in the other patient. In both these patients, heart failure worsening was observed during these events, and anticoagulation was inadequate in one of them. Five of 16 patients in whom stable SR was observed during the follow-up were off antiarrhythmic drug therapy at final evaluation. In the other 6 patients, antiarrhythmic drug therapy was continued due to ventricular arrhythmias. Successfully treated patients more often had paroxysmal AF (successful ablation: paroxysmal AF in 10 of 16 patients; unsuccessful ablation: paroxysmal AF in 4 of 14 patients; p = 0.009) and were younger (45 ± 11.5 years vs. 52.6 ± 9.2 years; p = 0.046). In addition, a trend toward a reduced need for cardioversion at the end of the procedure was also observed in these patients (3 patients in the successful ablation group vs. 8 patients in the unsuccessful ablation group; p = 0.056). In multivariate regression analysis, paroxysmal AF was the only independent predictor of a successful outcome. CONCLUSIONS: Catheter ablation of AF in patients with HCM is an effective and safe therapeutic option, particularly in patients with paroxysmal AF. Effectiveness of ablation is significantly smaller in patients with persistent AF and even more so in those with long-persistent AF. Repeated procedures were often necessary. Continued antiarrhythmic drug therapy is often required due to a significant degree of atrial remodelling.

An assessment of regression of left ventricular hypertrophy following alcohol ablation of the interventricular septum in patients with hypertrophic cardiomyopathy with left ventricular outflow tract obstruction.

BACKGROUND: Hypertrophic obstructive cardiomyopathy (HOCM) is characterised by asymmetric myocardial hypertrophy, which is most pronounced in the interventricular septum (IVS) and is responsible for the dynamic obstruction of the left ventricular outflow tract (LVOT). Successful alcohol septal ablation (ASA) of the IVS allows to reduce the thickness of the parabasal part of the IVS myocardium and, in most cases, to permanently reduce the gradient in the LVOT. AIM: To assess, using cardiac magnetic resonance imaging (MRI) and transthoracic echocardiography (TTE), the impact of gradient reduction in the LVOT on the type and severity of left ventricular (LV) remodelling. METHODS: The study included 30 patients (aged 56.9 ± 11.9 years) with HOCM and the mean peak gradient (PG) in the LVOT of 123 ± 33 mm Hg who underwent ASA. MRI measurements were performed before and at 6 months after ASA and TTE measurements were performed before, at 3 months and at 6 months after ASA. RESULTS: PG in the LVOT decreased to an average of 52 ± 37 mm Hg (p 〈 0.0001) at 3 months after ASA and to 37 ± 28 mm Hg (p 〈 0.0001) at 6 months after ASA. TTE revealed a decrease in IVS thickness outside the scar following ASA from 23.6 ± 3.5 mm to 19.3 ± 4.0 mm (p 〈 0.0001) and 19.4 ± 0.4 mm (p 〈 0.0001) at 3 and 6 months, respectively. There was also a decrease in lateral wall (PW) thickness from 15.9 ± 3.2 mm to 14.9 ± 2.9 mm (p = 0.046) and 14.16 ± 2.00 (p = 0.0065) at 3 and 6 months, respectively. MRI revealed a decrease in IVS thickness from 23.7 ± 2.8 mm to 18.04 ± 4.00 mm (p = 0.0001) at 6 months following ASA. We observed a regression of the PW hypertrophy from 13.2 ± 3.35 mm to 12.18 ± 2.4 mm (p = 0.0225). There was a decrease in IVS mass from 108.9 ± 20 g to 91.5 ± 29 g (p = 0.0006). There was a trend towards a decreased LV mass and LV mass excluding IVS mass at 6 months. CONCLUSIONS: A significant decrease in PG in the LVOT is associated with a decrease in LV mass and with regression of LV hypertrophy outside the scar after ASA.

Coronary computed tomographic angiography for prediction of procedural and intermediate outcome of bypass grafting to left anterior descending artery occlusion with failed visualization on conventional angiography.

Conventional coronary angiography (CCA) has considerable limitations regarding visualization of distal vessel segments in chronic total occlusion. We assessed the ability of coronary computed tomographic angiography (CCTA) to predict the success of coronary artery bypass grafting (CABG) to the chronically occluded left anterior descending coronary artery (LAD) incompletely visualized on CCA. Thirty symptomatic patients rejected for CABG on the basis of the CCA findings underwent preoperative CCTA before intended transmyocardial laser revascularization. The LAD was explored operatively in all patients, and CABG to the LAD was attempted if the distal vessel was suitable for anastomosis. The procedural outcome of CABG and the 6-month patency of the left internal mammary artery graft at follow-up CCTA were defined as the primary and secondary end point, respectively. The primary and secondary end points were achieved in 80% and 77% of patients, respectively. We found a significant correlation between the intraoperative and computed tomographic measurement of distal LAD diameter (R = 0.428, p = 0.037). On multivariate analysis, the maximum diameter of the distal LAD by CCTA (odds ratio 8.16, p = 0.043) was the only independent correlate of procedural success of CABG. A cutoff value of 1.5 mm for the mean distal LAD diameter predicted left internal mammary artery graft patency with 100% specificity and 83% sensitivity. Successful CABG resulted in significant improvements in angina class and left ventricular function in LAD segments at 6 months of follow-up. In conclusion, CCTA predicted both the procedural and the intermediate outcome of CABG to chronic LAD occlusion with failed visualization on CCA.

Are cardiomyocytes able to generate pre-amyloid peptides?

We performed ultrastructural testing of a cardiac biopsy taken from a heart with amyloidosis in which transthyretin mutation and light chain A amyloidosis were excluded. Cardiomyocytes of the affected heart showed accumulation of endosomal-like structures in which soluble amyloid oligomeric conformation was deposited. Intracellular accumulation of ß -amyloid as well as phosphorylated tau protein seen in the immunohistochemical study suggest that the heart tissue may generate an amyloidogenic peptide leading to cardiomyocyte destruction and heart dysfunction.

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.

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.

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.

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.

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.

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.

Late gadolinium enhancement gray zone in patients with hypertrophic cardiomyopathy. Comparison of different gray zone definitions.

To quantify heterogeneous tissue at the periphery of areas of fibrosis (gray zone) in patients with hypertrophic cardiomyopathy (HCM) with the use of two different techniques. Cardiac magnetic resonance with late gadolinium enhancement analysis was performed in 33 patients with HCM. Gray zone was evaluated with the use of two different techniques previously described in patients after myocardial infarction. LGE was present in 25 (78%) patients. There was no significant difference in total LGE mass at two different cut-off values [53.8 g (interquartile range, IQR 43.5-77.8 g) vs. 53.8 g (IQR 37.8-64.5 g), respectively, P = 0.49]. Significant difference in gray zone mass assessed with the use of two techniques was demonstrated (19.1 +/- 7.3 g vs. 50.8 +/- 47.8 g; P = 0.003). There was a strong correlation between total LGE and gray zone mass (r = 0.789, P = 0.0001 for first method and r = 0.951, P < 0.0001 for the second one, respectively). However, significant variability of gray zone mass (and extent expressed as % of left ventricular mass) in patients with similar LGE size/extent was observed. Moreover, LGE mass varied greatly in patients with similar gray zone size. Neither left ventricular mass, nor with maximal wall thickness correlated with extent of gray zone assessed with both methods. The studied techniques provided similar results with regard to total LGE but significant differences were observed in gray zone mass. Two patients may have similar extent (or absolute mass) of LGE, but strikingly discrepant gray zone size.

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.

The risk of non-sustained ventricular tachycardia after percutaneous alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy.

BACKGROUND: Percutaneous alcohol septal ablation (ASA) becomes an alternative option of treatment for symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM). The procedure relieves left ventricular outflow tract obstruction, but produces a myocardial scar in patients who already have a substrate for life-threatening ventricular arrhythmia. OBJECTIVES: To examine the effect of ASA on the occurrence of non-sustained ventricular tachycardia (nsVT) on 24 h ambulatory Holter monitoring in HOCM patients. METHODS: Sixty-one consecutive patients (34 males, mean age 48 years), who underwent ASA between 1997 and 2003 were analyzed. Holter recordings were performed in each patient before and after ablation. RESULTS: Follow-up ranged from 60 to 125 months (median 116 months). The mean number of Holter recordings per patient was 2.7 (range 1-11) before and 8.3 (range 2-23) after ASA (p < 0.001). Non-sustained ventricular tachycardia occurred in 14 patients before and 27 patients after ASA (23 vs. 44%, p = 0.01). The percentage of Holter recordings with nsVT before and after ablation was similar (14.5 vs. 15.7%, p = 0.56, respectively). No difference was observed between the number of nsVT per Holter recording before and after ablation (0.21 vs. 0.24%, p = 0.65, respectively). The percentage of patients with nsVT after ASA was comparable to the proportion of patients with nsVT in a control group consisting of 705 patients with hypertrophic cardiomyopathy under follow-up at our institution (44.3 vs. 43.2%, p = 0.91). There was no significant difference in percentage of Holter recordings with nsVT with respect to sex, amount of alcohol used during ASA, peak creatine phosphokinase level, and gradient reduction at rest. CONCLUSION: Alcohol septal ablation affected neither the percentage of Holter recordings with nsVT nor the number of nsVT episodes per Holter recording among HOCM patients.

A new model of home-based telemonitored cardiac rehabilitation in patients with heart failure: effectiveness, quality of life, and adherence.

AIMS: Despite proven benefits of cardiac rehabilitation (CR), currently proposed CR models are not acceptable for many heart failure (HF) patients. The purpose of this study was to evaluate a new model of home-based telemonitored cardiac rehabilitation (HTCR) using walking training compared with an outpatient-based standard cardiac rehabilitation (SCR) using interval training on a cycle ergometer. METHODS AND RESULTS: The study included 152 HF patients (aged 58.1 + or - 10.2 years, NYHA class II and III, ejection fraction < or = 40%) who were randomized to HTCR (n = 77) or SCR (n = 75). All patients underwent 8 weeks of CR. Both groups were comparable in terms of demographic and clinical characteristics and medical therapy. The effectiveness of CR was assessed by changes in NYHA class, peak oxygen consumption, 6-min walking test distance, and SF-36 score. Cardiac rehabilitation resulted in a significant improvement of all parameters in both groups. All patients in the HTCR group completed the 8 weeks of CR, whereas 15 patients in the SCR group (20%) discontinued CR. CONCLUSION: In patients with HF, HTCR is equally as effective as SCR and provides a similar improvement in quality of life. Adherence to CR seems to be better for HTCR. Home-based telemonitored cardiac rehabilitation may be a useful alternative form of CR in patients with HF.

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.