Electrocardiogram analysis in Anderson-Fabry disease: a valuable tool for progressive phenotypic expression tracking.

Background: Electrocardiogram (ECG) has proven to be useful for early detection of cardiac involvement in Anderson-Fabry disease (AFD); however, little evidence is available on the association between ECG alterations and the progression of the disease. Aim and Methods: To perform a cross sectional comparison of ECG abnormalities throughout different left ventricular hypertrophy (LVH) severity subgroups, providing ECG patterns specific of the progressive AFD stages. 189 AFD patients from a multicenter cohort underwent comprehensive ECG analysis, echocardiography, and clinical evaluation. Results: The study cohort (39% males, median age 47 years, 68% classical AFD) was divided into 4 groups according to different degree of left ventricular (LV) thickness: group A ≤ 9 mm (n = 52, 28%); group B 10-14 mm (n = 76, 40%); group C 15-19 mm (n = 46, 24%); group D ≥ 20 mm (n = 15, 8%). The most frequent conduction delay was right bundle branch block (RBBB), incomplete in groups B and C (20%,22%) and complete RBBB in group D (54%, p < 0.001); none of the patients had left bundle branch block (LBBB). Left anterior fascicular block, LVH criteria, negative T waves, ST depression were more common in the advanced stages of the disease (p < 0.001). Summarizing our results, we suggested ECG patterns representative of the different AFD stages as assessed by the increases in LV thickness over time (Central Figure). Patients from group A showed mostly a normal ECG (77%) or minor anomalies like LVH criteria (8%) and delta wave/slurred QR onset + borderline PR (8%). Differently, patients from groups B and C exhibited more heterogeneous ECG patterns: LVH (17%; 7% respectively); LVH + LV strain (9%; 17%); incomplete RBBB + repolarization abnormalities (8%; 9%), more frequently associated with LVH criteria in group C than B (8%; 15%). Finally, patients from group D showed very peculiar ECG patterns, represented by complete RBBB + LVH and repolarization abnormalities (40%), sometimes associated with QRS fragmentation (13%). Conclusions: ECG is a sensitive tool for early identification and long-term monitoring of cardiac involvement in patients with AFD, providing "instantaneous pictures" along the natural history of AFD. Whether ECG changes may be associated with clinical events remains to be determined.

Incidence, predictors, and effects of acute kidney injury after percutaneous valve implantation.

BACKGROUND: Acute kidney injury is a common complication after surgical aortic replacement and entails a worse prognosis. Percutaneous valve implantation is an alternative to surgical replacement. We sought to elucidate incidence, predictors, and effects of acute kidney injury after percutaneous aortic valve implantation. METHODS: A cohort of consecutive patients who underwent percutaneous aortic valve implantation at one center were studied, excluding those who died in the first 24 hours and those on prior hemodialysis. RESULTS: A total of 131 patients (age, 80.8 ± 6 years; 42% male; Euroscore II, 10.27 ± 6.9) were included. Acute kidney injury was defined as a reduction >25% in glomerular filtration rate (GFR). GFR was 62.09 ± 22 mL/min/1.73 m² at baseline, 62.7 ± 25 mL/min/1.73 m² after the procedure, and 68.03 ± 25 mL/min/1.73 m² at discharge. Acute kidney injury appeared in 17 patients (13%). Of these, 11 recovered and only 6 (4.5%) showed impaired GFR >25% upon discharge. Patients with acute kidney injury showed longer hospitalization length (median 7 days [IQR, 5-12 days] vs 3 days [IQR, 2-6 days]; P=.01) and higher 30-day mortality rate (17.6% vs 0.9%; P=.01). The only independent predictor for acute kidney injury development was Euroscore II (odds ratio, 1.192; confidence interval, 1.042-1.326; P=.01). CONCLUSION: Incidence of acute kidney injury after transcatheter aortic valve implantation was 13% in our cohort. Patients with acute kidney injury showed longer hospitalization and higher 30-day mortality rate. Euroscore II was an independent predictor of acute kidney injury.

Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy.

BACKGROUND: A previous study suggested that severe left-ventricular hypertrophy (maximum wall thickness > or = 30 mm) in patients with hypertrophic cardiomyopathy is associated with a risk of sudden cardiac death sufficient to warrant consideration for implantation of a cardioverter defibrillator (ICD). However, the prognostic significance of left-ventricular hypertrophy in relation to other clinical risk factors is poorly characterised. METHODS: We studied 630 patients consecutively referred to one hospital in London, UK (mean age 37 years [SD 16]; 382 male; mean follow-up 59 months). Patients underwent two-dimensional and doppler echocardiography, upright exercise testing, and Holter monitoring. FINDINGS: 39 patients died suddenly or had an appropriate ICD discharge; nine died from progressive heart failure; 11 from other cardiovascular causes and 23 from non-cardiac causes. There was a trend towards higher probability of sudden death or ICD discharge with increasing wall thickness (p=0.029, relative risk per 5 mm increment 1.31 [95% CI 1.03-1.66]). Of the 39 patients who died suddenly or had an ICD discharge, ten had a wall thickness of 30 mm or more. Patients with wall thickness of 30 mm or more had higher probability of sudden death or ICD discharge than patients with wall thickness less than 30 mm (p=0.049, 2.07 [1.00-4.25]. When considered together, the number of additional risk factors (one to three) was a better predictor of risk of sudden death or ICD discharge than wall thickness (p=0.0001, relative risk per additional factor 2.00 [1.43-2.79] vs p=0.058, 1.26 per 5 mm increment [0.99-1.60]). There was no relation between the pattern of hypertrophy and survival. INTERPRETATION: The risk of sudden death associated with a wall thickness of 30 mm or more in patients without other risk factors is insufficient to justify aggressive prophylactic therapy. Most sudden deaths occurred in patients with wall thickness less than 30 mm, so the presence of mild hypertrophy cannot be used to reassure patients that they are at low risk.

[Epidemiological changes in infectious endocarditis. A prospective study, 1992-96]. / Cambios epidemiológicos en la endocarditis infecciosa. Estudio prospectivo 1992-96.

BACKGROUND: To know the present epidemiological situation of the infective endocarditis in our environment and its evolution in the last few years. RESULTS: The incidence of infective endocarditis was 0.85 per thousand patients admitted to hospital, with a mean age of 43 years. The predisposed factors more frequently found were: drug addiction (32%) and cardiac prosthetic valves (23%). In the greatest number of our patients the cardiac valves involved were: tricuspid (28%), mitral (27%) and prosthetic valves (23%). The causative organism were: S. aureus (19 cases), Streptococcus (15 cases) and S. epidermidis (11 cases). The echocardiography study resulted diagnostic in 90% of the patients, valve replacements were performed in 22% of the cases. The overall mortality rate was 10%. CONCLUSIONS: The current profile of infective endocarditis is characterized by a high incidence of parenterally drug addict patients or prosthetic valves carriers. Increase of the infections of S aureus and a decrease of Streptococcus infections, as well as a less overall mortality.