Prevalence of papillary muscle hypertrophy in fabry disease.

BACKGROUND AND AIMS: Fabry disease (FD) is an X-linked genetic lysosomal disease, in which a deficit in the alpha-galactosidase A enzyme results in lysosomal build-up of globotriaosylceramide in several organs, causing cardiac, renal and cerebrovascular complications. The aim of this study was to assess the prevalence of papillary muscle hypertrophy (PMH) in patients with FD. METHODS: A group of 63 patients with FD and a positive genetic diagnosis were studied and were divided into two groups: one included 24 patients with FD and LVH and another group included 39 patients with FD and without LVH. Papillary muscles were measured from the left parasternal short axis view, defining PMH as a diastolic thickness greater than 11 mm in any diameter. RESULTS: Patients with FD and LVH had a high prevalence of anterolateral PMH (66.6%), and such prevalence was lower for the posteromedial PMH (33.3%). However, patients who had not yet developed LVH had a high prevalence of anterolateral PMH (33.3%). CONCLUSIONS: Patients with FD in the pre-clinical stage (without LVH) have a high prevalence of PMH, especially involving the anterolateral papillary muscle. This finding could be an early marker for the development of LVH, allowing to suspect the disease during its early stages, and begin enzyme replacement therapy in the appropriate patients.

Genetic Deletion of Galectin-3 Exacerbates Age-Related Myocardial Hypertrophy and Fibrosis in Mice.

BACKGROUND/AIMS: Aging is accompanied by progressive and adverse cardiac remodeling characterized by myocardial hypertrophy, fibrosis, and dysfunction. We previously reported that galectin-3 (Gal-3) is a critical regulator of inflammation and fibrosis associated with hypertensive heart disease and myocardial infarction. Nevertheless, the role and mechanism of Gal-3 in age-related cardiac remodeling have not been previously investigated. We hypothesized that Gal-3 plays a critical role in cardiac aging and that its deficiency exacerbates the underlying mechanisms of myocardial hypertrophy and fibrosis. METHODS: Male C57BL/6 (control) (n=24) and Gal-3 knockout (KO) (n=29) mice were studied at 24 months of age to evaluate the role of Gal-3 in cardiac aging. We assessed 1) survival rate; 2) systolic blood pressure (SBP) by plethysmography; 3) myocardial hypertrophy, apoptosis, and fibrosis by quantification of histological and immunohistochemical analysis; 4) cardiac expression of angiotensin (Ang) II, Ang (1-7) by Radioimmunoassay; 5) transforming growth factor-ß (TGF-ß), sirtuin (SIRT) 1, SIRT 7 and metalloproteinase 9 (MMP-9) by RT-qPCR and 6) ventricular remodeling and function by echocardiography. RESULTS: We found that aged Gal-3 KO mice had a lower survival rate and exhibited exacerbated myocardial hypertrophy and fibrosis without changes in SBP. Similarly, myocardial apoptosis and MMP-9 mRNA expression was significantly increased in the hearts of Gal-3 KO mice compared to controls. Additionally, cardiac Ang II and TGF-ß expression were higher in aged Gal-3 KO mice while SIRT1 and SIRT7 expression were reduced. CONCLUSION: Our findings strongly suggest that Gal-3 is involved in age-related cardiac remodeling by regulating critical mechanisms associated with the development of pathological hypertrophy. The gene deletion of Gal-3 reduced the lifespan and markedly increased age-dependent mechanisms of myocardial hypertrophy, apoptosis, and fibrosis, including Ang-II, TGF-ß, and MMP-9. At the same time, there was diminished cardiac-specific expression of SIRT1 and SIRT7, which are extensively implicated in delaying age-dependent cardiomyopathies.

Hypothyroid cardiomyopathy: A reversible phenocopy of hypertrophic cardiomyopathy.

We present the case of a 46-year-old patient with hypothyroidism secondary to Hashimoto's thyroiditis who was admitted with decompensation in the form of myxedema. A 2-D echocardiogram shows a septal asymmetric hypertrophy, with low-voltage QRS complex in the ECG and a bull's-eye map of longitudinal strain with preserved apical strain with reduction of mid and basal strain that results in "cherry on the top" pattern, similar to the most frequent phenocopy of hypertrophic cardiomyopathy, as is the cardiac amyloidosis, and that, unlike this pathology, reverted after the patient reached the euthyroid state.

Speckle tracking echocardiography in the indeterminate form of Chagas disease.

BACKGROUND: Chagas disease is one of the most common diseases in Latin-America, and cardiac involvement is a significant cause of death. Assessment of myocardial strain may detect early myocardial damage. OBJECTIVES: To determine differences in longitudinal strain using speckle tracking to assess regional and global left ventricular function in patients with the indeterminate form of Chagas disease, in comparison with a control group. METHODS: This is a retrospective matched case-control study, conducted in a single center. We evaluated 45 adult patients with Chagas disease, diagnosed with 2 serological methods, without evidence of cardiac involvement, who were compared with 45 healthy control subjects, who were sex- and age-matched. All patients underwent Doppler echocardiography and longitudinal strain with speckle tracking. RESULTS: Median age was 59 years, and 60% were female. Echocardiographic parameters were similar in patients with Chagas and control subjects. In patients with Chagas, global strain differed significantly from that of control subjects (-17 vs -20.3, P < .001). Segmental strain showed 7 abnormal segments in patients with Chagas (P < .05). CONCLUSIONS: In patients with the indeterminate form of Chagas disease, global and segmental longitudinal peak systolic strain is reduced compared with healthy subjects, thus suggesting that it could be a sensitive technique to detect early myocardial damage. These findings could provide useful information regarding the pathophysiology of cardiac involvement and understand whether they might have prognostic usefulness or help develop strategies to modify the course and prognosis of patients with Chagas disease. A longitudinal prospective study would be necessary to validate our findings.

Tissue Doppler Imaging and strain rate of the left atrial lateral wall: age related variations and comparison with parameters of diastolic function.

BACKGROUND: Strain Rate Imaging (SRI) is one of the most used techniques to study left atrial (LA) and diastolic function. Its availability in low-income countries is diminished since it requires additional expensive software, among other limitations. In contrast, Tissue Doppler Imaging (TDI) is widely available and easy to use. We hypothesize TDI could detect changes in LA and diastolic function associated with age similarly to SRI. The aim of this study is to evaluate the effects of age on LA and diastolic function assessed by LA lateral wall TDI online by spectral pulse, and to compare them with age-related variations of LA SRI and other parameters of diastolic function in a population of healthy adults. MATERIALS AND METHODS: Ninety-one healthy adults were prospectively evaluated. In apical four - chamber view the LA lateral wall was divided in three portions. Peak velocities of basal and mid portions were measured with TDI online by spectral pulse and with SRI by speckle tracking. A first positive wave (S'la and SRS) and two negative waves (E'la and SRE, and A'la and SRA respectively) were obtained. E'la/A'la ratio and SRE/SRA ratio were analyzed. The distribution of the variables by age subgroups was described and analyzed. Correlation analyses were performed. RESULTS: The median age was 42 years old and 54.9% were female. E'la/A'la showed a negative good correlation with age. E'la/A'la and SRE/SRA ratios changed from > 1 to < 1 in the age group of 41-50 years old, while this occurred in the group of 51-60 years old for the E/A ratio. Lateral and septal mitral annulus E´ showed decrease with age and prolongation of E-wave deceleration time was observed in the age group over 61 years old. CONCLUSION: Normal values​​ according to age group of TDI of the LA lateral wall were obtained. Age-related changes in LA and diastolic function could be detected as early with TDI as with SRI. Future studies are required to explore if this method could be used to address in part LA or diastolic function in other populations with established cardiovascular disease or at risk of presenting it, which could be useful in low-income settings, where SRI is not available.

Mechanical dispersion in Fabry disease assessed with speckle tracking echocardiography.

BACKGROUND: Fabry disease (FD) is a rare X-linked storage disorder caused by deficiency of the lysosomal enzyme α-galactosidase A, and it typically causes multiorgan dysfunction. The main cause of death is heart disease resulting from left ventricular (LV) diastolic dysfunction, LV systolic dysfunction, severe LV hypertrophy (LVH), and sudden death. In several cardiac disorders, LV systolic dysfunction and ventricular arrhythmias are associated with mechanical dispersion (MD). MD has until now not been studied in patients with FD. OBJECTIVE: To investigate the prevalence of MD in patients with FD. METHODS: Complete echocardiographic data and speckle tracking echocardiographic data were collected. MD is an index of inter-segmental discoordination of contraction and is defined as the standard deviation (SD) of the time-to-peak longitudinal negative strain in 17 LV segments with a value >49 milliseconds. Patients with FD were divided into the following 2 groups: group I (patients with FD but no LVH, n = 64) and group II (patients with FD and LVH, n = 25). These groups were compared with a group of healthy subjects (group III, n = 50). Parametric variables were expressed as mean ± SD, and nonparametric variables were expressed as median and inter-quartile range. A P value <.05 was considered significant. RESULTS: A total of 113 patients with FD were included in this study. Of these, 24 (21%) were excluded because of poor imaging quality or presence of comorbidities, and the final study population consisted of 89 patients (mean age of 33.5 ± 14.5 years, 64% female). Group II patients were older than group I patients (46 ± 13 years vs 27 ± 11 years, P < .0001). There was no difference in LV ejection fraction between the 3 groups. There was also no difference in MD between groups I and III (32.4 ms [26-39] vs 32 ms [26-39]). In group II, the MD in 19 patients (76%) was 56 ms (39-80). CONCLUSIONS: To the best of our knowledge, this is the first study to assess the prevalence of MD in patients with FD. MD was observed in 76% of patients with FD and LVH. The use of MD in strain echocardiography may be beneficial in the assessment of patients with FD who develop heart failure.

Clinical significance of tissue Doppler imaging in chronic Chagas disease.

INTRODUCTION: Chagas disease (ChD) is one of the main parasitic diseases in Latin-America. Its heart involvement is the most important cause of death. The aim of this study is to evaluate if Doppler Tissue Imaging (DTI) may have a predictive value for later events in subjects with chronic ChD. METHODS: we analyses DTI variables of 543 patients with chronic ChD for the evaluation of predicting factors of events. Major adverse cardiovascular events (MACE) were considered as stroke, heart failure resistant to treatment, sustained ventricular tachycardia, implantable cardioverter-defibrillator, sudden death, and cardiovascular death. The following findings were also included in total evens: heart failure, bradycardia, ventricular arrhythmia, new conduction system abnormalities, and new echocardiographic abnormalities. Multivariate analysis with logistic regression was used in order to assess the Doppler and DTI parameters predicting events. Variables with a P-value ≤ .5 in the univariate analysis were included in the multivariate analysis. RESULTS: In patients with chronic ChD, the analysis of DTI parameters showed that S' wave and E' wave of the lateral wall of the left ventricle were significant predictors of MACE (OR: 0.83; 95% CI: 0.71-0.96; P-value: .015 and OR: 0.80; 95% CI: 0.66-0.98; P-value: .031, respectively). CONCLUSIONS: This study found that patients with chronic ChD who had events showed significantly lower parameters in the DTI. What is more, this study showed that even lower DTI parameters are significant predictors of events.

Tissue Doppler imaging of the atrial lateral wall: Correlation with atrial strain rate and parameters of diastolic function.

OBJECTIVE: To evaluate the velocity profile of the lateral wall of the left atrium (LA) using pulsed-wave color tissue Doppler imaging (TDI) on line and its correlation with two-dimensional strain rate imaging (SRI) by speckle tracking, of equal segments and phases, and other parameters of diastolic function in a population of individuals without known cardiovascular disease. MATERIALS AND METHODS: A total of 91 adults aged 18-74 years, without cardiovascular history, hypertension, diabetes, dyslipidemia or obesity, were evaluated. A Doppler echocardiogram was performed. The lateral wall of the LA was divided subjectively into three portions, the atrial roof was excluded; the peak velocities of the basal and midportions, and their average, were measured with pulsed-wave TDI on line and SRI. Using both techniques, a first positive wave (S'la and SRS-reservoir phase) and two negative waves (E'la and SRE-conduit phase-and A'la and SRA-atrial systole-respectively) were obtained. E'la/A'la ratio and SRE/SRA ratio were analyzed. RESULTS: The median age was 42 years, and 54.9% of the subjects were female. S'la presented moderate correlation with S' lateral mitral annulus. Both E'la and the E'la/A'la relationships showed moderate to good correlation with various diastolic dysfunction parameters evaluated and SRI. A'la did not present a statistically significant correlation with any of the variables evaluated. CONCLUSION: Evaluation of the lateral wall of the LA by pulsed-wave color TDI on line is feasible in the vast majority of patients. In the conduit phase, E'la and the E'la/A'la relationship showed correlation with the parameters that evaluate diastolic function and SRI.

Comparison of left atrial size and function in hypertrophic cardiomyopathy and in Fabry disease with left ventricular hypertrophy.

BACKGROUND: Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) are two diseases with a different pathophysiology, both cause left ventricular hypertrophy (LVH) and myocardial fibrosis. Although remodeling and systolic dysfunction of the left atrium (LA) are associated with atrial fibrillation and stroke in HCM, changes in the size and function of the LA have not been well studied in FD with LVH. METHODS: The following groups were studied prospectively, and their respective findings compared: 19 patients with non-obstructive HCM (Group I), 20 patients with a diagnosis of Fabry cardiomyopathy (Group II), and 20 normal subjects matched for sex and age (Group III). Left ventricular mass index was measured using Devereux' formula, left atrial volume with Simpson's biplane method and left atrial mechanical function, including strain and strain rate, was measured using the speckle tracking technique. Strain and strain rate of the reservoir were measured during the three phases: reservoir (SR S), passive conduit (SR E) and atrial contraction (SR A). RESULTS: Patients with HCM had a larger left atrial volume than patients with FD (48.16 ± 14.3 mL/m2 vs 38.9 ± 14.9 mL/m2 respectively, P < .001), but in both disorders there was a severe decrease in left atrial function: reservoir strain in the apical four-chamber view: 17.47% in HCM vs 22.5% in FD, P = .24), strain rate in the apical chamber view: SR A: -0.80/seconds in HCM vs -1.04/seconds in FD (P = .88), SR S: 0.69/seconds in HCM vs 0.93 in FD (P = .12), SR E: -0.80 seconds in HCM vs -0.97/seconds in FD (P = .18). CONCLUSIONS: In this echocardiographic study we used speckle tracking to assess left atrial mechanical function and showed that FD is associated to an atrial cardiomyopathy, affecting the three phasic functions of the LA. Although in patients with HCM left atrial volume is larger than in patients with FD, both disorders exhibit severe decrease in left atrial function. These findings should be considered, given the potentially serious complications that can occur with the two diseases.

[Clinical examination and the Valsalva maneuver in heart failure]. / Examen clínico y maniobra de Valsalva en la insuficiencia cardíaca.

Congestion in heart failure patients with reduced ejection fraction (HFrEF) is relevant and closely linked to the clinical course. Bedside blood pressure measurement during the Valsalva maneuver (Val) added to clinical examination may improve the assessment of congestion when compared to NT-proBNP levels and left atrial pressure (LAP) estimation by Doppler echocardiography, as surrogate markers of congestion in HFrEF. A clinical examination, LAP and blood tests were performed in 69 HFrEF ambulatory patients with left ventricular ejection fraction ≤ 40% and sinus rhythm. Framingham Heart Failure Score (HFS) was used to evaluate clinical congestion; Val was classified as normal or abnormal, NT-proBNP was classified as low (< 1000 pg/ml) or high (≥ 1000 pg/ml) and the ratio between Doppler early mitral inflow and tissue diastolic velocity was used to estimate LAP and was classified as low (E/e'< 15) or high (E/e' ≥ 15). A total of 69 patients with HFrEF were included; 27 had a HFS ≥ 2 and 13 of them had high NT-proBNP. HFS ≥ 2 had a 62% sensitivity, 70% specificity and a positive likelihood ratio of 2.08 (p=0.01) to detect congestion. When Val was added to clinical examination, the presence of a HFS ≥ 2 and abnormal Val showed a 100% sensitivity, 64% specificity and a positive likelihood ratio of 2.8 (p = 0.0004). Compared with LAP, the presence of HFS = 2 and abnormal Val had 86% sensitivity, 54% specificity and a positive likelihood ratio of 1.86 (p = 0.03). In conclusion, an integrated clinical examination with the addition Valsalva maneuver may improve the assessment of congestion in patients with HFrEF.

Bone cement cardiac and pulmonary embolism.

This case describes a complication of bone cement use. A 65-year-old male patient with back spine trauma caused by a fall, underwent a percutaneous vertebroplasty. Five years later, he consulted for palpitations, and the electrocardiogram showed supraventricular arrhythmia. A transthoracic two-dimensional echocardiography showed a hyperechogenic linear structure of 7 cm length, running from the lateral wall of the right ventricle to the right atrium through the tricuspid valve. This foreign body, which was suspicious for bone cement embolism, appeared rigid and was attached at the lateral wall of the right ventricle, with its proximal end free in the right atrium. The tip of the cement embolus was inside the myocardium of the lateral wall of the right ventricle, with risk of cardiac perforation. A fluoroscopy was performed, which confirmed the presence of cement within the right heart, with great mobility in each cardiac cycle. Chest computed tomography (CT) and multidetector CT three-dimensional reconstruction confirmed the presence of cement within the right heart. Chest CT showed two pulmonary embolisms, one in the right upper lobe and one in the left lower pulmonary lobe. This case emphasizes the risk of late clinical manifestations of cardiac and pulmonary embolism of methylmethacrylate after percutaneous vertebroplasty, suggesting that the risk of such embolism might be underestimated. We propose routine chest radiography, two-dimensional echocardiography, and chest CT after every percutaneous vertebroplasty, to detect asymptomatic cardiac and pulmonary embolism and thereby prevent serious delayed cardiopulmonary failures.

Pulmonary Edema Assessed by Ultrasound: Impact in Cardiology and Intensive Care Practice.

Pulmonary edema is a frequent condition found in adult patients hospitalized in cardiology wards and intensive care units. Ultrasonography is a diagnostic modality with a high sensitivity for the detection of extravascular lung water, visualized as B lines, and usually caused by cardiogenic or noncardiogenic pulmonary edema. This paper highlights a simple method for the assessment of patients with pulmonary edema, which allows for a differential diagnosis of its possible mechanism and contributes to therapeutic intervention guiding and monitoring.

Papillary fibroelastoma: clinical and echocardiographic features and initial approach in 54 cases.

INTRODUCTION: Papillary fibroelastoma (PFE) is a benign cardiac tumor that is currently detected more often due to the technological improvements in echocardiography. OBJECTIVES: To describe the echocardiographic features of PFE and correlate them with the clinical presentation and initial treatment. MATERIALS AND METHODS: A prospective analysis of patients with a diagnosis of PFE was conducted between 2000 and 2015. We assessed the clinical history, symptoms at the time of diagnosis, echocardiographic features, and initial treatment. RESULTS: Fifty-four patients with a diagnosis of PFE by echocardiography were included. The incidence was 0.038%. Mean age was 62±16 years; 50% were male. Forty-six percent of patients had symptoms at the time of diagnosis, the most frequent of which was transient ischemic accident (TIA). Embolic episodes occurred in 31% of patients, mainly to the brain. PFEs were valvular in 70.4% of cases and multiple in 13%. Mean maximum length was 1.18±0.58 cm, and 44% were mobile. PFEs >1.5 cm were most often found in the heart valves (56.8% vs 10.8%; P=.045). There was no significant relation between size, mobility, location and number of tumors, and the presence of embolism or symptoms. Most frequent treatment was oral anticoagulation in 48.6%, followed by simple tumor resection in 42.8% of cases. CONCLUSIONS: PFE is a small tumor, predominantly valvular and benign, but entails a high incidence of cerebral embolism. The initial approach should be individualized according to clinical manifestations, comorbidities, and the experience of the surgical center.

Apical left ventricular hypertrophy and mid-ventricular obstruction in fabry disease.

We report the case of a rare cardiac presentation of Fabry disease. Although concentric left ventricular hypertrophy is a major cardiac finding in Fabry disease, there is no case report of dynamic obstruction at mid-left ventricular level. We describe a 59-year-old-woman suffering from a severe form of Fabry disease, mimicking an apical hypertrophic cardiomyopathy with mid-ventricular obstruction. Differentiation of Fabry disease from hypertrophic cardiomyopathy is crucial given the therapeutic and prognostic differences. Fabry disease should always be suspected in an adult, independently of the pattern of left ventricular hypertrophy.

Severe mitral regurgitation after radiotherapy.

We present the case of a 69-year-old patient with a history of gynecological neoplasia and a pulmonary metastasis, who in 1996 underwent chemotherapy and mediastinal radiotherapy followed by cancer remission. Ten years later she presented with heart failure and her Doppler echocardiogram showed severe mitral regurgitation with pulmonary hypertension. In 2011, she underwent a mitral valve replacement with a biological prosthesis and the pathology exam revealed valve damage consistent with radiotherapy-induced changes. This unusual mechanism of mitral regurgitation can be demonstrated clearly by echocardiography and should be disseminated among cardiology physicians and in patients who have survived for long periods after radiotherapy, it is important to remember that cardiac complications may indeed occur, and the treating physician is responsible for detecting them.

Cardiovascular adaptive response to training in elite athletes. Comparison between endurance and non-endurance athletes.

INTRODUCTION: The different structural modifications that have been described in the heart of the high-performance athlete depend on factors such as age, gender, type of sport, and the intensity and time dedicated to training. OBJECTIVES: Evaluation of elite athletes through echocardiography for the description of cardiac structure and function, and the comparison between athletes with cardiorespiratory endurance and the rest of the athletes. METHODS: We performed the echocardiographic examination in 224 elite athletes, 96 women and 128 men aged 15 to 38 years (21.7 ± 5.3 years) and they were divided into 2 groups: "Endurance Group" (cardiorespiratory endurance) and "Non-Endurance Group" which included the rest of the sports. Univariate comparison between the two groups was performed by measuring 14 echocardiographic variables. RESULTS: In men, statistically significant higher values were identified in the endurance group for interventricular septum, left ventricular posterior wall, relative wall thickness (RWT), left ventricular mass index and left atrial dimension. In women, the endurance group had significantly lower heart rate values, and significantly higher left ventricular diastolic dimension with normal RWT. CONCLUSIONS: Most of the echocardiographic variables showed higher sample means in the endurance athletes. In the subgroup of men from the Endurance Group, eccentric hypertrophy prevailed with a greater increase in wall thickness, as well as in the diameter of the left atrium, while in women the variables indicated eccentric hypertrophy at the expense of an increase in left ventricle diameter, without increased wall thickness.

Introducción: Las diferentes modificaciones estructurales que han sido descritas en el corazón del deportista de alto rendimiento dependen de factores como la edad, el género, el tipo de deporte, la intensidad y el tiempo dedicados al entrenamiento. OBJETIVOS: Evaluación de atletas de élite por medio de la ecocardiografía para la descripción de la estructura y la función cardíacas, y la comparación entre deportistas de resistencia cardiorrespiratoria y el resto de los deportistas. Métodos: Realizamos el examen ecocardiográfico en 224 deportistas de elite, 96 mujeres y 128 varones con edades de 15 a 38 años (21.7 ± 5.3 años) y se dividieron en 2 grupos: "Grupo de Resistencia" (resistencia cardiorrespiratoria) y "Grupo de no Resistencia" el cual incluyó al resto de los deportes. Se realizó la comparación univariada de 14 variables ecocardiográficas entre los dos grupos. RESULTADOS: En los hombres se identificaron valores estadísticamente significativos más altos en el grupo de resistencia para septum interventricular, pared posterior, grosor parietal relativo, índice de masa del ventrículo izquierdo y aurícula izquierda. En las mujeres, el grupo de resistencia tuvo valores de frecuencia cardíaca más bajos con diámetro diastólico e índice de masa del ventrículo izquierdo significativamente mayores. CONCLUSIONES: La mayoría de las variables ecocardiográficas mostró valores mayores en los atletas de resistencia. En los hombres del Grupo de Resistencia, predominó la hipertrofia excéntrica con mayor incremento en el grosor parietal y del diámetro de la aurícula izquierda, mientras que en las mujeres las variables indicaron hipertrofia excéntrica a expensas de un aumento del diámetro del ventrículo izquierdo, sin incremento del grosor parietal.

Esophageal carcinoma and transthoracic echocardiography.

We report a rare case of a patient with esophageal carcinoma diagnosed using transthoracic echocardiography. This examination proved to be useful in the identification of a paracardiac mediastinal mass. Images of the esophageal carcinoma, of the stent in the esophagus, and the bubbles inside the stent generated with the ingestion of a carbonated beverage, have not been previously published. Therefore, we believe our findings may be useful to other echocardiographers.

Assessment of coronary flow with transthoracic color Doppler echocardiography in patients with hypertrophic cardiomyopathy.

BACKGROUND: Recent advances in echocardiography have allowed assessment of flow velocity in the epicardial coronary arteries of patients with ischemic heart disease, using transthoracic color Doppler echocardiography (TTDE). However, few data are available regarding coronary blood flow in patients with hypertrophic cardiomyopathy (HCM). OBJECTIVE: To assess the epicardial coronary arteries of patients with HCM. MATERIAL AND METHODS: A group of 25 patients with HCM was assessed prospectively (mean age 57 ± 21 years, 11 male) using TTDE; flow velocities in the epicardial coronary arteries were measured and compared with those obtained in 10 age- and gender-matched controls. Analysis of the diastolic spectral waveform included flow velocity integral (VTI), peak velocity, deceleration time, and deceleration slope measurements, while systolic waveform analysis included peak flow measurement and morphology assessment (positive, absent or negative). An analysis of variance (ANOVA) test was used for multiple comparisons for variables with a normal distribution, and a Kruskal-Wallis test was used for variables with non-Gaussian distribution. RESULTS: Patients with HCM exhibited an increase in diastolic flow velocity with a rapid deceleration slope and a systolic slope which was decreased, absent or reversed, compared to normal subjects. On linear regression analysis there was no correlation with the type of hypertrophy or magnitude of the intraventricular pressure gradient in patients with obstructive HCM. CONCLUSION: In patients with HCM, noninvasive assessment with TTDE revealed abnormal findings in the distal flow of the epicardial coronary arteries, very similar to those seen in the no-reflow phenomenon. These findings were independent of the type of hypertrophy and magnitude of the intraventricular pressure gradient in patients with obstructive HCM.

Two-dimensional speckle tracking echocardiography for early detection of myocardial damage in young patients with Fabry disease.

Fabry disease (FD) is characterized by left ventricular hypertrophy (LVH). Conventional echocardiography is not sensitive enough to perform the preclinical diagnosis To assess whether longitudinal myocardial strain of the left ventricle (LV), using speckle tracking, is useful to detect early myocardial involvement in FD. Forty-four patients with FD who were diagnosed with genetic testing were prospectively included and were compared to a sex-matched control group. They were divided into three groups: 22 with LVH (Group I), 22 without LVH (Group II), and 22 healthy volunteers (Group III). LV longitudinal strain was measured from the apical views. An ANOVA test was used for multiple comparisons for variables with a normal distribution, and a Kruskal-Wallis test was used for variables with non-Gaussian distribution. Longitudinal LV strain was different in the three groups: it was ≥-15% in at least one segment in all Group I patients, in 50% of patients of Group II and in no patient of Group III. Seventy percent of the segments with abnormal strain in Group II were located in the basal regions (32/46). These findings show that the presence of at least one strain value ≥-15% demonstrates subclinical myocardial dysfunction in patients with preclinical FD. Longitudinal myocardial LV strain measured with speckle tracking is a useful tool to detect early myocardial involvement in young patients with FD. This information allows the detection and treatment of myocardial dysfunction at an early stage, which is of high clinical importance.

An Experimental Model of Myocardial Infarction for Studying Cardiac Repair and Remodeling in Knockout Mice.

Cardiovascular disease is the most prevalent cause of death in Western countries, with acute myocardial infarction (MI) being the most prevalent form. This paper describes a protocol for studying the role of galectin 3 (Gal-3) in the temporal evolution of cardiac healing and remodeling in an experimental animal model of MI. The procedures described include an experimental model of MI with a permanent coronary ligature in male C57BL/6J (control) and Gal-3 knockout (KO) mice, an echocardiography procedure to study cardiac remodeling and systolic function in vivo, a histological evaluation of interstitial myocardial fibrosis with picrosirius red-stained and rhodamine-conjugated lectin-stained sections for studying myocyte hypertrophy by the cross-sectional area (MCSA), and the quantification of infarct size and cardiac remodeling (scar thinning, septum thickness, and expansion index) by planimetry in slices stained with Masson's trichrome and triphenyl tetrazolium chloride. Gal-3 KO mice with MI showed disrupted cardiac remodeling and an increase in the scar thinning ratio and the expansion index. At the onset of MI, myocardial function and cardiac remodeling were also severely affected. At 4 weeks post MI, the natural evolution of fibrosis in infarcted Gal-3 KO mice was also affected. In summary, the experimental model of MI is a suitable model for studying the temporal evolution of cardiac repair and remodeling in mice with the genetic deletion of Gal-3 and other animal models. The lack of Gal-3 affects the dynamics of cardiac repair and disrupts the evolution of cardiac remodeling and function after MI.