Abstract Background Recent studies have shown the promise of new approaches to the management of hypertrophic cardiomyopathy (HCM) symptoms. However, as HCM is a chronic disorder that evolves slowly over decades with low annual event rates, therapeutic trials in the disease rely on surrogate measures of disease severity and progression rather than hard endpoints such as mortality. Exercise capacity is one potential surrogate endpoint. Purpose To examine the relationship between peak oxygen uptake (VO2) and six-minute walk test distance (6MWD) in patients with non-obstructive hypertrophic cardiomyopathy (nHCM) and describe the association with clinical parameters and quality of life. Methods We examined baseline data from patients with nHCM enrolled in a randomised placebo-controlled trial of trimetazidine therapy. The study was conducted in a single European centre. Cardiopulmonary exercise testing (CPET) was performed to assess peak oxygen consumption (pVO2), ventilatory anaerobic threshold (VAT) and ventilatory equivalent for carbon dioxide (minute ventilation to carbon dioxide production (VEVCO2). Health related quality of life was assessed with the Minnesota Living with Heart Failure Questionnaire (MLHFQ). ECG and echocardiography were performed on the same day and biomarkers (NT-proBNP and cardiac Troponin T, cTnT) were measured prior to exercise testing. Results In 51 patients (age 50 ± 13 years; 71% male) with nHCM, pVO2 ranged from 10 to 24.3 ml/kg/min and 6MWD from 188 to 650 metres. 6MWD correlated with peak VO2 (Pearson r=0.41, 95% CI 0.15 to 0.67, p=0.003) and anaerobic threshold (r=0.32, 95%CI 0.04 to 0.55, p=0.024). After adjusting for age and sex, NT-proBNP concentration correlated with peak VO2 (radi=-0.35, 95% CI -0.69 to -0.01, p=0.042) but not 6MWD (radj=-0.05, 95% CI -0.42 to 0.32, p=0.779). Health related quality of life assessed by the Minnesota Living with Heart Failure Questionnaire (MLHFQ) correlated moderately with 6MWD (radi=-0.54, 95% CI -0.79 to -0.29, p<0.001) but weakly with peak VO2 (radi 0.28, 95% CI -0.55 to -0.01, p=0.042). I Conclusions There is a modest correlation between peak VO2 and 6MWD in nHCM. The pVO2 correlates more strongly with NT-proBNP whereas 6MWD better reflects quality of life. These data suggest pVO2 and 6MWD provide complementary information about health status in nHCM.Relationship between 6MWD and CPETCharacteristics of study cohort

infections).In children and adults, adenoviral DNA was detected only in single cases with acute or chronic myocarditis.Conclusions: In adults, we observed 3 times more often an immune-mediated than an infectious acute myocarditis provoking a rapid immunosuppressive therapy after acute onset of symptoms provided that a cardiac viral infection was excluded.Children suffer 2,3 times more often than adults from virus-induced acute myocarditis and 1,3 times more often from infectious chronic myocarditis than adults, stressing the relevance of the diagnostic molecular pathology in the successful management and therapy of these patients.

La ablación septal percutánea es una alternativa terapéutica en la miocardiopatía hipertrófica obstructiva. Debido a su introducción relativamente reciente, no hay información sobre eficacia y seguridad a muy largo plazo. Este estudio multicéntrico evalúa sus resultados en seguimiento superior a 10 años.Se incluyó consecutivamente a pacientes tratados con ablación septal en cinco centros entre 1998 y 2003. Se han analizado datos clínicos, hemodinámicos y ecocardiográficos basales y de seguimiento.Se ha incluido a 45 pacientes (media de edad, 62,4 ± 14 años), de los que 31 eran mujeres y 39 (86,6%) estaban en clase funcional III-IV. El grosor del septo era 21,8 ± 3,5 mm; el gradiente máximo basal por ecocardiografía, 77 ± 39 mmHg, y la insuficiencia mitral era de grado al menos moderado en 22 pacientes (48,8%). Durante la hospitalización, 3 casos precisaron implante de marcapasos definitivo y 1 paciente sufrió perforación ventricular por electrodo de marcapasos, que requirió cirugía. Tras seguimiento de 12,3 (11,0-13,5) años, 2 pacientes (4,4%) sufrieron muerte cardiaca (insuficiencia cardiaca y postrasplante); 3, implante de desfibrilador automático implantable (1 caso por prevención primaria y 2 por taquicardia ventricular sostenida tras cirugía cardiaca), y 2, cirugía cardiaca (endocarditis e insuficiencia mitral). En la última evaluación clínica, la clase funcional era I-II en 39 (86,6%) (p < 0,0001); el gradiente máximo basal, 16 ± 23 mmHg (p < 0,0001), y la insuficiencia mitral, nula o ligera en 34 pacientes (75,5%) (p < 0,03).Estos resultados a más de 10 años indican seguridad y eficacia a muy largo plazo para la ablación septal. No hubo incidencia significativa de arritmias ventriculares sintomáticas o muerte súbita.Percutaneous transluminal septal ablation is an alternative treatment in patients with hypertrophic obstructive cardiomyopathy. However, due to the relatively new introduction of this technique, there is no information on its very long term results (>10 years).The present study included consecutive patients treated in 5 centers between 1998 and 2003. We analyzed clinical, hemodynamic, and echocardiographic data at baseline and follow-up.A total of 45 patients were included; there were 31 (69%) women, the mean age was 62.4 (14) years, and 39 patients (86.6%) showed functional class III or IV. Septal thickness was 21.8 (3.5) mm, the peak resting gradient on echocardiography was 77 (39) mmHg, and mitral regurgitation was at least moderate in 22 patients (48.8%). During hospitalization, permanent pacemaker implantation was required in 3 patients and ventricular perforation (by pacing lead) occurred in 1 patient, requiring surgery. After a follow-up of 12.3 years (11.0-13.5 years), 2 patients (4.4%) died from cardiac causes (heart failure and posttransplantation), 3 patients required an implantable cardioverter-defibrillator (1 for primary prevention and 2 due to sustained ventricular tachycardia after cardiac surgery), and 2 underwent cardiac surgery (due to endocarditis and mitral regurgitation). In the last clinical review, functional class was I-II in 39 patients (86.6%) (P

AIMS:Non-compaction of the left ventricle (LVNC) is a disorder of endomyocardial morphogenesis that results in multiple trabeculations in the left ventricular myocardium. The current literature suggests that LVNC in adults is rare and associated with a poor prognosis. Given that the disorder is present at birth and that several studies have reported asymptomatic familial disease in some patients, we hypothesized that there is a long pre-clinical phase of the disease. The aim of this study was to define the prognosis and familial incidence of LVNC.METHODS AND RESULTS:This study cohort comprised 45 patients (mean age at diagnosis 37 years) consecutively identified at a referral centre for cardiomyopathy over a 10-year period. Twenty-eight patients (62%) had dyspnoea at presentation; 41 (91%) an abnormal ECG; and 30 (66%) left ventricular dilatation and impaired systolic function. Nine patients (20%) had non-sustained ventricular tachycardia on 24 h Holter monitoring. Mean survival from death or transplantation was 97% at 46 months. There were three thromboembolic events in two patients (4%). On systematic family screening, 8 of 32 (25%) asymptomatic relatives had a range of echocardiographic abnormalities, including LVNC, LVNC with impaired systolic function, and left ventricular enlargement without LVNC.CONCLUSION:This study demonstrates that LVNC is associated with a better prognosis than previously reported. In patients with familial disease, relatives may have features consistent with dilated cardiomyopathy rather than LVNC.

Objective: To measure coronary flow reserve (CFR), an index of microvascular function, in Anderson-Fabry disease (AFD) at baseline and after enzyme replacement therapy (ERT).Methods and results: Mean (SD) myocardial blood flow (MBF) at rest and during hyperaemia ( adenosine 140 mg/kg/min) was measured in 10 male, non-smoking patients (53.8 (10.9) years, cholesterol 5.5 (1.3) mmol/l) and in 24 age matched male, non-smoking controls (52.0 (7.6) years, cholesterol 4.5 (0.6) mmol/l) by positron emission tomography ( PET). Resting and hyperaemic MBF and CFR ( hyperaemic/resting MBF) were reduced in patients compared with controls ( 0.99 (0.17) v 1.17 (0.25) ml/g/ min, p< 0.05; 1.37 (0.32) v 3.44 (0.78) ml/g/ min, p< 0.0001; and 1.41 (0.39) v 3.03 (0.85), p< 0.0001, respectively). This coronary microvascular dysfunction was independent of cholesterol concentrations. PET was repeated in five patients after 10.1 (2.3) months of ERT; resting and hyperaemic MBF and CFR were unchanged after ERT ( 0.99 (0.16) v 0.99 ( 0.16) ml/g/ min; 1.56 (0.29) v 1.71 (0.3) ml/g/ min; and 1.6 (0.37) v 1.74 (0.28), respectively; all not significant).Conclusions: The results of the present study show that patients with AFD have very abnormal coronary microvascular function. These preliminary data suggest that ERT has no effect on coronary microvascular dysfunction. Further work is necessary to determine whether treatment at an earlier stage in the course of the disease may improve coronary microvascular function in patients with AFD.

Acute coronary syndromes are characterized by the expression of proinflammatory cytokines such as C-reactive protein (CRP). Sustained upregulation of inflammatory markers is associated with an adverse prognosis. Vitamin E is known to have significant anti-inflammatory properties and has been associated with a reduction in cardiovascular events in some studies of high-risk patients. The mechanism of benefit remains controversial. We conducted a randomized, double-blind placebo controlled trial of vitamin E 400 IU daily for 6 months in 110 patients with acute coronary syndromes. Serum samples were collected at enrollment and at 2, 4, and 6 months. CRP, interleukin-6 and the soluble cell adhesion molecules were measured. Vitamin E levels increased significantly in the treatment group (from 31 μmol/l at baseline to 51 μmol/l, p < .0001) and were unchanged in the placebo group (32 μmol/l at baseline to 34 μmol/l, p = NS). CRP levels fell in both the vitamin E group and the placebo group over the treatment period (from 17.2 ± 2.9 to 6.1 ± 0.8 mg/l and from 21.5 ± 4.9 to 5.9 ± 0.9 mg/l, p = NS for the difference between active and placebo groups). However, vitamin E treatment was associated with significantly lower 6 month CRP levels in smokers versus smokers on placebo (4.7 ± 0.71 mg/l vs. 8.26 ± 1.5 mg/l, p = .02). Vitamin E reduces CRP levels in smokers with acute coronary syndromes for up to 6 months after hospitalization.