Prevalence of Fabry disease in patients with left ventricular hypertrophy and renal involvement (PrEFaCe). / Prevalencia de la enfermedad de Fabry en los pacientes con hipertrofia ventricular izquierda e insuficiencia renal (PrEFaCe).

INTRODUCTION AND AIMS: Fabry disease (FD) causes glycosphingolipid accumulation in the vascular endothelium, with predominantly cardiac and renal involvement. Its prevalence in patients with concomitant involvement of these two organs is unknown. The objective of the study was to determine the prevalence of FD in patients with left ventricular hypertrophy and any degree of chronic kidney disease. PATIENTS AND METHODS: Patients with ventricular thickness ≥13mm and kidney disease from 29 Spanish hospitals were included. Sociodemographic variables and target organ involvement of FD were collected. Laboratory determinations of EF were carried out, with an enzymatic activity test±genetic test in men and direct genetic test in women. RESULTS: Eight hundred ninety-eight patients with left ventricular hypertrophy and chronic kidney disease were included. The presence of heart failure and cardiorenal syndrome was common (46.1% and 40.1%). Three patients (2 men and 1 woman) were diagnosed with FD, based on the presence of a pathogenic variant in the GLA gene and classic signs of FD, resulting in a prevalence of 0.33% (CI 95% 0.06-1%). Six patients (0.66%) presented genetic variants of unknown significance, without showing classic signs of FD, while in 13 patients (3.2%) performing the blood test was impossible. CONCLUSIONS: FD is an important cause of left ventricular hypertrophy and chronic kidney disease. Genetic diagnosis is crucial for avoiding biases and ensuring accurate identification of FD, especially in women. The results support the inclusion of this disease in the differential diagnosis of patients with ventricular hypertrophy ≥13mm and chronic kidney disease.

Intravenous metoprolol during ongoing STEMI ameliorates markers of ischemic injury: a METOCARD-CNIC trial electrocardiographic study.

Besides its protective effect against neutrophil-mediated injury at reperfusion, intravenous (IV) metoprolol was recently shown to reduce the progression of ischemic injury in a pig model of ST-segment elevation myocardial infarction (STEMI). Here, we tested the hypothesis that IV metoprolol administration in humans with ongoing STEMI blunts the time­dependent progression of ischemic injury assessed by serial electrocardiogram (ECG) evaluations before reperfusion. The METOCARD-CNIC trial randomized 270 anterior STEMI patients to IV metoprolol or control before reperfusion by percutaneous coronary intervention (PCI). In 139 patients (69 IV metoprolol, 70 controls), two ECGs were available (ECG-1 before randomization, ECG-2 pre-PCI). Between-group ECG differences were analyzed using univariate and multivariate regression models. No significant between-group differences were observed on ECG-1. On ECG-2, patients who received IV metoprolol had a narrower QRS than those in the control group (84 ms vs. 90 ms, p = 0.029), a lower prevalence of QRS distortion (10% vs. 26%, p = 0.017), and a lower sum of anterior and total ST-segment elevation (10.1 mm vs. 13.6 mm, p = 0.014 and 10.4 mm vs. 14.0 mm, p = 0.015, respectively). Adjusted analysis revealed similar results. Significant associations were observed between ECG-2 variables and cardiac magnetic resonance imaging measurements (extent of myocardial edema, infarct size, microvascular obstruction, and left-ventricular ejection fraction) after STEMI. In summary, IV metoprolol administration before reperfusion ameliorates ECG markers of myocardial ischemia in anterior STEMI patients. These data confirm that IV metoprolol is able to reduce ischemic injury and highlight the ability of ECG analysis to provide relevant real-time information on the effect of cardioprotective therapies before reperfusion.

QRS distortion in pre-reperfusion electrocardiogram is a bedside predictor of large myocardium at risk and infarct size (a METOCARD-CNIC trial substudy).

BACKGROUND: QRS distortion is an electrocardiographic (ECG) sign of severe ongoing ischemia in the setting of ST-segment elevation acute myocardial infarction (STEMI). We sought to evaluate the association between the degree of QRS distortion and myocardium at risk and final infarct size, measured by cardiac magnetic resonance (CMR). METHODS: A total of 174 patients with a first anterior STEMI reperfused by primary angioplasty were prospectively recruited. Pre-reperfusion ECG was used to divide the study population into three groups according to the absence of QRS distortion (D0) or its presence in a single lead (D1) or in 2 or more contiguous leads (D2+). Myocardium at risk and infarct size were determined by CMR one week after STEMI. Multiple regression analysis was used to study the association of QRS distortion with myocardium at risk and infarct size, with adjustment for relevant clinical and ECG variables. RESULTS: 101 patients (58%) were in group D0, 30 (17%) in group D1, and 43 (25%) in group D2+. Compared with group D0, presence of QRS distortion (groups D2+ and D1) was associated with a significantly adjusted larger extent of myocardium at risk (group D2+: absolute increase 10.4%, 95% CI 6.1-14.8%, p<0.001; group D1: absolute increase 3.3%, 95% CI 1.3-7.9%, p=0.157) and larger infarct size (group D2+: absolute increase 10.1%, 95% CI 5.5-14.7%, p<0.001; group D1: absolute increase 4.9%, 95% CI 0.08-9.8%, p=0.046). CONCLUSIONS: Distortion in the terminal portion of the QRS complex on pre-reperfusion ECG in two or more leads is independently associated with larger myocardium at risk and infarct size in the setting of primary angioplasty-reperfused anterior STEMI. QRS distortion in only one lead is independently associated with larger infarct size in this setting. Our findings suggest that QRS distortion analysis could be included in risk-stratification of patients presenting with anterior STEMI.

Prognostic impact of atrial fibrillation in acute coronary syndromes: results from the ARIAM registry.

AIMS: The prognostic ability of atrial fibrillation (AF) in acute coronary syndromes (ACS) is unclear. Studies regarding patient outcomes with respect to the timing of AF are scarce and conflicting. The present study aimed to determine the frequency, predictors and impact on clinical outcome of AF in patients with ACS. METHODS: We analysed 39,237 consecutive patients with ACS included in the ARIAM registry between January /2001 and December 2011. Patients with AF were compared with patients in sinus rhythm. We differentiate between new-onset AF and previous AF cases to analyse mortality and other major adverse cardiac events (MACE) during hospitalization. RESULTS: Of the patients, 2851 (7.3%) developed AF; 1568 (55%) of these were new-onset AF and 1283 (45%) had previous AF. The AF group had a higher risk profile at baseline and poorer clinical presentation at admission than non-AF patients. Compared with previous AF patients, new-onset AF presented with fewer comorbidities, including hypertension, diabetes, prior myocardial infarction, and chronic renal impairment. The inhospital mortality for new-onset AF, previous AF, and non-AF patients were 14, 11.6, and 5.2%, respectively (new-onset AF unadjusted HR 2.19, 95% CI 1.9-2.53, p<0.001; adjusted HR 1.70, 95% CI 1.12-3.4, p<0.001). After propensity score analysis, only new-onset AF persisted as an independent predictor for mortality (HR 1.62, 95% CI 1.09-2.89, p<0.001). Other MACE such as reinfarction, malignant arrhythmias, and heart failure were also more frequent in new-onset AF patients than in previous AF or non-AF patients. CONCLUSIONS: These findings suggest that the presence of new-onset AF during ACS is associated with a significant increase in mortality, even after adjusting for confounding variables.

Current indications for infective endocarditis antibiotic prophylaxis.

Indications of endocarditis prophylaxis have changed in the past years, because of the absence of any evidence that justified its use. The last guidelines only recommend prophylaxis in patients with underlying cardiac conditions with the higher risk of adverse outcomes, including patients with a previous history of infective endocarditis, patients with prosthetic heart valve or prosthetic material used for valve repair, patients with a valvulopathy after cardiac transplantation, and patients with an specific congenital heart disease. The list of procedures in which prophylaxis is necessary has been limited too. Nowadays it is recommended in patients who undergo any dental procedure that involves the gingival tissues or periapical region of a tooth and for those invasive procedures of the oral cavity or an invasive procedure of the respiratory tract that involves incision or biopsy of the respiratory mucosa. In this revision we try to expose the recent tendencies recommended by the international guidelines.