Viviana Serra

Usefulness of tissue Doppler on early detection of cardiac disease in Fabry patients and potential role of enzyme replacement therapy (ERT) for avoiding progression of disease

AIMS Cardiac involvement, including progressive cardiomyopathy, is common in Fabry disease and is a leading cause of premature mortality. We sought to determine if tissue Doppler imaging (TDI) could identify Fabry disease patients at risk for the development of cardiomyopathy and if enzyme replacement therapy (ERT) with agalsidase alfa might slow or prevent the progression of cardiac involvement. METHODS AND RESULTS Fabry disease patients were enrolled in this prospective, observational study. Echocardiography was performed at baseline and periodically throughout the study. A single investigator, blinded to both the type of assessment (baseline or follow-up) and enzyme replacement status of the patient, evaluated all echocardiograms. Seventy-six patients (26 male, 50 females) were enrolled in the study. Twenty men and 13 women were treated with agalsidase alfa during the study. At baseline, increasing interventricular septum thickness was significantly associated with decreasing TDI velocities. Twenty-nine patients >18 years old (23 females) had no evidence of cardiac involvement at baseline (normal LVM and normal TDI velocities). In this cohort, 80% (16 of 20) of patients not on ERT progressed to demonstrating an abnormal TDI velocity during follow-up, whereas only 33% (3 of 9) of patients on ERT progressed to TDI abnormalities (P= 0.031). CONCLUSION In Fabry disease, reduced TDI velocity seems to be the initial sign of cardiac involvement that occurs before the development of cardiac hypertrophy. ERT with agalsidase alfa delays the onset of cardiac involvement and should be considered at an earlier stage of the disease, even in the absence of left ventricular hypertrophy.

Quantification of Aortic Valve Area Using Three-Dimensional Echocardiography

INTRODUCTION AND OBJECTIVES To determine whether the reproducibility of left ventricular outflow tract (LVOT) area measurement is greater with three-dimensional echocardiographic (3D-echo) planimetry than with conventional 2D-echo. To determine the LVOT circularity index by means of 3D-echo. To determine the usefulness of measuring the LVOT area by 3D-echo for quantifying the severity of valvular aortic stenosis. METHODS The study included 40 patients, of whom 22 had an aortic stenosis. The LVOT area was measured using both 2D-echo and 3D-echo, and the circularity index, using 3D-echo alone. In addition, the severity of valvular aortic stenosis was categorized using both 2D-echo and 3D-echo. RESULTS The levels of inter- and intra-observer agreement on LVOT area measurements were better with 3D-echo. The circularity index was 1.50 (0.25), and there was a very poor linear correlation with LVOT area (r=-0.34; P=.47). Patients with valvular aortic stenosis were categorized according to the severity of their stenoses using both 2D-echo and 3D-echo. The level of agreement between the two techniques was poor (kappa=0.36). CONCLUSIONS Measurements of the LVOT area made using 3D-echo were more reproducible than those made using 2D-echo. Consequently, 3D-echo may be a better technique for assessing the LVOT area. In addition, 3D-echo showed that the LVOT is elliptical in form and that its size is not related to its circularity. Moreover, 3D-echo could also be helpful in classifying the severity of valvular aortic stenosis.

Diastolic heart failure in the elderly: In-hospital and long-term outcome after the first episode

Our aim was to describe the incidence and predictors of in-hospital mortality and long-term mortality and morbidity in elderly patients after a first admission due to diastolic HF (DHF). Six hundred and seventy nine consecutive elderly patients with a first admission to hospital due to DHF comprised our study group. Mean age was 83.3+/-6.7 (464 women--68.3%). A history of dilated cardiomyopathy was associated to increased in-hospital mortality and age and pulmonary artery systolic pressure were identified as independent markers of bad long-term outcome. Thus, patients with DHF have high mortality during and after the first admission.

Contrast agents provide a faster learning curve in dipyridamole stress echocardiography

Aim: Interobserver variability is an important limitation of the stress echocardiography and depends on the echocardiographer training. Our aim was to evaluate if the use of contrast agents during dipyridamole stress echocardiography would improve the agreement between an experienced and a non-experienced observer in stress echo and therefore if contrast would affect the learning period of dypyridamole stress echo. Methods and results: Two independent observers without knowledge of any patient data interpreted all stress studies. One observer was an experienced one and the other had experience in echocardiography but not in stress echo. Two observers analysed 87 non-selected and consecutive studies. Out of the 87 studies, 46 were performed without contrast administration, whereas i.v. contrast (2.5 g Levovist® by two bolus at rest and at peak stress) was administered in 41. In all cases, second harmonic imaging and stress digitalisation pack was used. The agreement between observers showed a κ index of 0.58 and 0.83 without and with contrast administration, respectively. Conclusions: The use of contrast agents provides a better agreement in the evaluation of stress echo between an experienced and a non-experienced observer in stress echo. Adding routinely contrast agents could probably reduce the number of exams required for the necessary learning curve in stress echocardiography.

Myocardial contrast echocardiography in coronary artery disease

Myocardial contrast echocardiography (MCE) allows the assessment of myocardial perfusion by imaging the coronary microcirculation. The development of new contrast agents and new diagnostic tools far assessing myocardial perfusion by means of MCE has led to a new field of applications far patients suffering from ischemic heart disease. Several studies have shown that MCE is a feasible and accurate method to evaluate patients with: a) acute coronary syndromes: MCE is useful before the epicardial reperfusion to delineate the area at risk and to assess the collateral-derived myocardial blood flow, and after the epicardial reperfusion to detect the non-reflow phenomenon; b) chronic coronary syndromes: MCE allows the detection of significant coronary stenosis by means of stress methods and methods without any stress; c) myocardial viability and hibernating myocardium: MCE helps to predict functional recovery of akinetic segments. In these settings, MCE is not only useful as a diagnostic tool but also provides prognostic information. MCE is a technique in constant development. Among the latest advances we note the development of transesophageal probes with second-harmonic image that allows assessment of myocardial perfusion in a more accurate way. This technique should introduce MCE into new clinical fields, especially the evaluation of myocardial perfusion during cardiac operations. Another recent development is in parametric imaging techniques. These consist in obtaining time curves for all the pixels in the image instead of working only with a few separate regions of interest. A parameter scan is computed far any pixel showing their value as a color overlay in the parametric image. Summarizing, we can say that MCE is crossing from the experimental laboratory to the daily clinical practice far the evaluation of ischemic heart disease. MCE provides an interesting tool that offers the potential of a complete evaluation of patients with chronic coronary artery disease. This includes both diagnostic and prognostic evaluation.

What to do with an atrial thrombus

This case report is about an 84-year-old woman admitted with right atrial thrombus attached to a pacemaker lead, diagnosed by transesophageal echocardiography. Surgical treatment was rejected due to the high operative risk, and percutaneous stenting was performed with success; unfortunately, the outcome was fatal and the patient died. We review here the clinical features of right atrial thrombi, including the epidemiology, clinical manifestations, diagnosis, and treatment, which is sometimes controversial.

Microbubbles: Basic Principles

Echocardiography represents a basic tool for diagnosis of cardiac pathology. During the last few years technological advances have led to new imaging methods with more applications. The availability of wide-band transducers has been fundamental for the utilization of ultrasound contrast agents.

Utilidad de la ecocardiografía de contraste con segundo armónico en un paciente con miocardiopatía hipertrófica y obstrucción mesoventricular

La ecocardiografia es una tecnica utilizada de rutina en la evaluacion de la funcion cardiaca. La definicion de los bordes endocardicos es fundamental para valorar de forma correcta la contractilidad global y segmentaria del ventriculo izquierdo. En algunas ocasiones esto resulta dificil debido a la inadecuada ventana acustica de los pacientes. Las nuevas tecnicas ecocardiograficas podrian ayudar a diagnosticar de forma precisa e incruenta determinadas enfermedades que pasaban inadvertidas con las tecnicas tradicionales. Presentamos el caso de un paciente diagnosticado en el ecocardiograma basal de alteraciones en la contractilidad segmentaria, tipo hipocinesia lateral y apical, en el que la utilizacion de la imagen armonica con ecocardiografia de contraste permitio cambiar el diagnostico inicial. El paciente presentaba realmente una miocardiopatia hipertrofica severa con obstruccion mesoventricular, sin alteraciones segmentarias de la contractilidad.

Myocardial perfusion in real-time using power modulation: In vivo evidence for microcirculatory damage after acute myocardial infarction

BACKGROUND AND OBJECTIVES In addition to the myocardium, the microvasculature may be also damaged in acute myocardial infarction. The aim was to evaluate the capability of myocardial contrast echocardiography in the detection of microvasculature damage after myocardial infarction. PATIENTS AND METHODS Twelve patients with recent acute myocardial infarction and five control subjects with normal coronary arteries and without history of myocardial infarction were studied. Myocardial contrast echocardiography with power modulation was performed, and quantitative data were measured off-line. Power modulation uses a combination of low (0.1) and high (1.7) mechanical indexes, allowing a real-time evaluation of myocardial perfusion. Contrast agent was administered as a 3-min bolus. The quantitative analysis was performed off-line by a different blinded investigator. The refilling velocity was calculated as the difference between the peak myocardial refilling value and the value at 1 s after the impulse divided by the time from the first second after the impulse to the peak refilling value. RESULTS Eighty-one myocardial segments (75%) were analysed qualitatively and quantitatively in AMI patients, and 18 (60%) in control subjects (P=NS). The peak refilling intensity was not significantly different in patients and control subjects (6.62+/-5.85 vs. 7.53+/-4.06 dB, respectively). However, time to peak refilling intensity was significantly longer (5.25+/-1.57 vs. 4.00+/-0.53, P=0.004) and the velocity of refilling was significantly lower (2.74+/-5.34 vs. 6.58+/-8.02, P=0.028) in patients with myocardial infarction. CONCLUSION There is microvasculature damage after myocardial infarction that is reflected as a delayed velocity of refilling in myocardial contrast echocardiography.

Ecocardiografía de contraste en la valoración del trombo intraventricular postinfarto

791 Los trombos intraventriculares son frecuentes tras un infarto de miocardio1,2. Sus caracteristicas ecocardiograficas pueden ser utiles en la prediccion del potencial emboligeno, y se han asociado a un mayor riesgo la movilidad y la protrusion en la cavidad ventricular3. La ecocardiografia a veces presenta limitaciones en su diagnostico y valoracion, especialmente en pacientes con ventana acustica deficiente. Presentamos el caso de un paciente con trombo intraventricular demostrado mediante ecocardiografia bidimensional tras un infarto de miocardio, en el que la utilizacion de un agente ecopotenciador resulto de gran utilidad en la valoracion de sus caracteristicas. Se trata de un paciente de 51 anos ingresado por un infarto anterior Killip I, reperfundido con tratamiento trombolitico. Tres dias despues se realizo un ecocardio-