Sarcoma sinovial primario de pericardio / Primary pericardial synovial sarcoma

Resumen El sarcoma sinovial primario del pericardio es un tumor muy raro y de mal pronóstico y se sabe poco en cuanto al manejo terapéutico. Presentamos el caso de una paciente de 51 años a quien se le realizó resección quirúrgica incompleta, quimioterapia y radioterapia. Hasta donde sabemos, este es el primer caso de un sarcoma sinovial primario de pericardio que luego de operado se mantuvo asintomático durante 5 años hasta que en una TAC de control se le detectaron metástasis cardiacas que comprometían las cavidades derechas y con quimioterapia, la ecocardiografía demostró la reso lución completa de las mismas.

Abstract Primary pericardial synovial sarcoma is an extraor dinarily very rare tumor with a poor prognosis, and little is known about its therapeutic management. We describe the case of a 51-year-old woman patient who underwent incomplete surgical resection, chemotherapy, and radiotherapy. To the best of our knowledge, no pri mary pericardial synovial sarcoma has been described which, after surgery, remains asymptomatic for 5 years, and until a control CT scan detects cardiac metastases that compromised the lumen of the right cavities and with chemotherapy, echocardiography demonstrated complete resolution of cardiac metastases.

[Primary pericardial synovial sarcoma]. / Sarcoma sinovial primario de pericardio.

Primary pericardial synovial sarcoma is an extraordinarily very rare tumor with a poor prognosis, and little is known about its therapeutic management. We describe the case of a 51-year-old woman patient who underwent incomplete surgical resection, chemotherapy, and radiotherapy. To the best of our knowledge, no primary pericardial synovial sarcoma has been described which, after surgery, remains asymptomatic for 5 years, and until a control CT scan detects cardiac metastases that compromised the lumen of the right cavities and with chemotherapy, echocardiography demonstrated complete resolution of cardiac metastases.

El sarcoma sinovial primario del pericardio es un tumor muy raro y de mal pronóstico y se sabe poco en cuanto al manejo terapéutico. Presentamos el caso de una paciente de 51 años a quien se le realizó resección quirúrgica incompleta, quimioterapia y radioterapia. Hasta donde sabemos, este es el primer caso de un sarcoma sinovial primario de pericardio que luego de operado se mantuvo asintomático durante 5 años hasta que en una TAC de control se le detectaron metástasis cardiacas que comprometían las cavidades derechas y con quimioterapia, la ecocardiografía demostró la resolución completa de las mismas.

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.

Registro OBELISCO-SAC. OBsErvaciones de cLinicas, Instituciones y Servicios de Cardio-Oncología SAC / OBELISCO-SAC Registry. OBsErvations from cLinics, InstitutionS and Cardio-Oncology services SAC

RESUMEN Introducción: La cardio-oncología (CO) es una nueva disciplina, que genera nuevas áreas de trabajo en las instituciones. Desconocemos cuántos equipos de CO existen en nuestro país, su estructura y el manejo de los pacientes. Objetivos: Nuestro objetivo primario es reportar cuántos centros de CO existen en nuestro país, y de ellos cuántos trabajan de acuerdo con las recomendaciones de guías y consensos. Secundariamente, definir la especialidad y formación de los médicos integrantes, si se realiza evaluación de riesgo previo al inicio del tratamiento oncológico, cómo se evalúa la función ventricular y cómo se utilizan los biomarcadores. Material y métodos: Registro nacional, multicéntrico, transversal, descriptivo, y prospectivo que incluyó 51 instituciones generales, de oncología y/o de cardiología que referían poseer grupos de trabajo o servicios de CO. Resultados: De los 51 centros, pertenecen al ámbito público el 47,1% y al privado el 52,9%. El 49% se halla en la Ciudad Autónoma de Buenos Aires, el 17,6% en la Provincia de Buenos Aires y el resto en otros lugares del país. Sobre 47 centros, el 48,9% considera funcionar de acuerdo con las recomendaciones de Guías Internacionales y el Consenso de la Sociedad Argentina de Cardiología. El 27,7% de los centros realiza siempre estratificación de riesgo cardio-oncológico o cardiovascular antes de iniciar el tratamiento. Un 35,3% de los centros deriva siempre a cardiología a los pacientes que iniciarán un tratamiento potencialmente cardiotóxico, un 47,1% ocasionalmente. El 43,1% de los centros realiza ecocardiografía basal en todos los pacientes, el 56,9% solo en algunos. Durante el seguimiento el ecocardiograma se indica de acuerdo con el esquema utilizado en el 64,7% y en el resto según su evolución. Todos los centros evalúan la fracción de eyección ventricular izquierda mediante ecocardiografía, en el 68,1% bidimensional. El 63,8% utiliza el análisis de la deformación longitudinal sistólica global. El 47,1% deriva algunos pacientes a resonancia cardíaca y el 35,3% a tomografía cardíaca. Solo el 7,8% utiliza biomarcadores. El 5,9% indica siempre prevención primaria con antagonistas neurohormonales. El dexrazoxano es utilizado en el 5,9%, la antraciclina liposomal en el 74,5%. Frente a la aparición de cardiotoxicidad, el 76,5% inicia tratamiento cardioprotector. El 41% suspende la quimioterapia, el 47% la modifica. Conclusiones: este es el primer registro nacional de CO. Brinda información y un panorama actual del estado de esta subespecialidad en nuestro país. Casi la mitad de los centros consideró funcionar de acuerdo con Guías y Consensos. Solo un tercio de los pacientes que van a iniciar tratamiento oncológico potencialmente cardiotóxico son derivados a CO. El método más utilizado en nuestro país para evaluar la función ventricular es el ecocardiograma bidimensional, los biomarcadores son poco utilizados.

ABSTRACT Background: Cardio-oncology (CO) is a new discipline that generates new work areas within the institutions. We ignore how many CO teams exist in our country, their structure and how patients are managed. Objectives: Our primary objective is to report how many CO centers exist in our country, and how many of them work according to the recommendations of guidelines and consensus statements. We also want to define the specialty and specific training of the physicians involved, determine if they perform risk assessment before cancer treatment, establish the method used to assess ventricular function and how biomarkers are used. Methods: The OBELISCO registry is a national, multicenter, cross-sectional, descriptive and prospective registry including 51 general hospitals, cancer centers and institutions specialized in cardiology with CO work groups or services. Results: Of the 51 centers, 47.1% were public hospitals and 52.9% were private centers. Most centers were in the Autonomous City of Buenos Aires (49%) and in the Province of Buenos Aires and the rest were distributed throughout the country. Of 47 centers, 48.9% considered that their institution had CO services complying with the recommendations of international guidelines and of the consensus statement of the Argentine Society of Cardiology. Global cardio-oncological or cardiovascular risk assessment is always performed in 27.7% of the centers before starting treatment. Patients who will start potentially cardiotoxic treatment are always referred to cardiology in 35.3% of the centers and are sometimes referred to cardiology in 47.1%. Baseline echocardiography is performed in all the patients before starting treatment in 43.1% of the centers and only in some patients in 56.9%. During follow-up, echocardiography is indicated according to the treatment schedule used in 64.7% and according to the patients' outcome in the rest of the centers. All the centers evaluate left ventricular ejection fraction with echocardiography, and 68.1% use twodimensional echocardiography. Global longitudinal systolic strain is used in 63.8% of the centers. Only 47.1% order cardiac magnetic resonance imaging in some patients, and 35.3% indicate cardiac computed tomography scan. Biomarkers are used in only 7.8% of the centers. Primary prevention with neurohormonal antagonist drugs is always indicated in 5.9% of the centers. Dexrazoxane is used in only 5.9% and liposomal anthracycline in 74.5% If cardiotoxicity develops, 76.5% indicate cardioprotection, 41% discontinue chemotheraphy and 47% modify cancer treatment. Conclusions: This is the first national CO registry. It provides information and a current outlook of the status of this subspecialty in our country. Almost 50% of the centers considered to be functioning in line with guidelines and consensus statements. Only one third of the patients who will initiate cancer treatment with potentially cardiotoxic drugs are referred to CO. Two-dimensional echocardiography is the method most used in our country to evaluate ventricular function; biomarkers are scarcely used.

Speckle Tracking Imaging in Patients with Pulmonary Hypertension.

BACKGROUND: Right ventricular (RV) systolic dysfunction is a strong predictor of mortality in pulmonary hypertension (PH). The goal of this study was to investigate whether right atrium (RA) and RV myocardial strain related to PH using speckle tracking echocardiography provide a superior estimation of RV systolic function than 2-dimensional (2D)-echo. METHODS: This cross-sectional study analyzed 22 patients with a diagnosis of PH stratified by right heart catheterization, and they were compared to a control group of 22 age- and sex-matched healthy subjects. RESULTS: Global longitudinal peak systolic strain measured in the RV free wall from the apical 4 chamber view was -15% vs. -14.5% when measured from the subcostal view (p = 0.99). Mean longitudinal strain during reservoir phase, and longitudinal strain rate during atrial reservoir and passive conduit function was significantly impaired measured in the right atrial free wall in patients with PH. CONCLUSIONS: This study showed impaired LV contractility in patients with PH assessed by speckle tracking strain. RV free wall longitudinal strain does not correlate with any of the measurements of RV systolic function obtained by 2D echocardiography. A major strength of RV longitudinal strain is its ability to assess the RV function without the limitations of 2D parameters. The subcostal RV strain is a feasible and accurate alternative to conventional RV strain from the apical view in patients with poor acoustic apical 4-chamber windows. The RA strain and strain rates values may be a valuable additive to assess right-sided heart function.

Perspectives on the COVID-19 pandemic impact on cardio-oncology: results from the COVID-19 International Collaborative Network survey.

BACKGROUND: Re-allocation of resources during the COVID-19 pandemic has resulted in delays in care delivery to patients with cardiovascular disease and cancer. The ability of health care providers to provide optimal care in this setting has not been formally evaluated. OBJECTIVES: To assess the impact of COVID-19 resource re-allocation on scheduling, testing, elective procedures, telemedicine access, use of new COVID-19 therapies, and providers' opinions on healthcare policies among oncology and cardiology practitioners. METHODS: An electronic survey was conducted by a cardio-oncology collaborative network through regional and state chapters of the American College of Cardiology, American Society of Clinical Oncology, and the International Cardio-Oncology Society. Descriptive statistics were reported by frequency and proportion for analyses, and stratified categorically by geographic region and specialty. RESULTS: One thousand four hundred fifteen providers (43 countries) participated: 986 cardiologists, 306 oncologists, and 118 trainees/internal medicine. 63% (195/306) of oncologists vs 92% (896/976) of cardiologists reported cancellations of treatments/elective procedures (p = 0.01). 46% (442/970) of cardiologists and 25% (76/303) of oncologists modified the scope of their practice (p = < 0.001). Academic physicians (74.5%) felt better supplied with personal protective equipment (PPE) vs non-academic (74.5% vs 67.2%; p = 0.018). Telemedicine was less common in Europe 81% (74/91), and Latin America 64% (101/158), than the United States, 88% (950/1097) (p = < 0.001). 95% of all groups supported more active leadership from medical professional societies. CONCLUSIONS: These results support initiatives to promote expanded coverage for telemedicine, increased access to PPE, better testing availability and involvement of medical professional societies to help with preparedness for future health care crisis.

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.

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.

Use of tissue doppler imaging for the early detection of myocardial dysfunction in patients with the indeterminate form of Chagas disease.

INTRODUCTION: Chagas disease is one of the most common diseases in Latin America and heart involvement is the main cause of death. This study aimed to determine differences in tissue Doppler imaging (TDI) parameters in the assessment left and right ventricular function in patients with the indeterminate form of Chagas disease compared to those in healthy controls. METHODS: We compared 194 patients with the indeterminate form of Chagas disease to 72 age-matched healthy individuals. We considered p-values <0.05 to be statistically significant. RESULTS: TDI analysis of the right ventricular (RV) showed lengthened isovolumic relaxation time (IRT) and higher RV index of myocardial performance (RIMP) and left ventricle (LV) index of myocardial performance (LIMP) in the Chagas group than in the control group, indicating RV and LV systolic and diastolic myocardial damage. TDI analysis of the myocardial velocities of the interventricular septum and the lateral wall of the LV also showed a systolic and diastolic myocardial damage. CONCLUSIONS: The study results demonstrated early LV systolic and diastolic myocardial damage in the RV and LV in patients with the indeterminate form of Chagas disease by TDI. These early findings of RV and LV dysfunction may help identify patients who will progress to heart failure during the disease course. TDI should be included in initial patient evaluations because it allows adequate follow-up and treatment.

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.

Use of tissue doppler imaging for the early detection of myocardial dysfunction in patients with the indeterminate form of Chagas disease

Abstract INTRODUCTION: Chagas disease is one of the most common diseases in Latin America and heart involvement is the main cause of death. This study aimed to determine differences in tissue Doppler imaging (TDI) parameters in the assessment left and right ventricular function in patients with the indeterminate form of Chagas disease compared to those in healthy controls. METHODS: We compared 194 patients with the indeterminate form of Chagas disease to 72 age-matched healthy individuals. We considered p-values <0.05 to be statistically significant. RESULTS: TDI analysis of the right ventricular (RV) showed lengthened isovolumic relaxation time (IRT) and higher RV index of myocardial performance (RIMP) and left ventricle (LV) index of myocardial performance (LIMP) in the Chagas group than in the control group, indicating RV and LV systolic and diastolic myocardial damage. TDI analysis of the myocardial velocities of the interventricular septum and the lateral wall of the LV also showed a systolic and diastolic myocardial damage. CONCLUSIONS: The study results demonstrated early LV systolic and diastolic myocardial damage in the RV and LV in patients with the indeterminate form of Chagas disease by TDI. These early findings of RV and LV dysfunction may help identify patients who will progress to heart failure during the disease course. TDI should be included in initial patient evaluations because it allows adequate follow-up and treatment.

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.

Twenty Years of Clinical Experience with Cardiac Myxomas: Diagnosis, Treatment, and Follow Up.

BACKGROUND: Cardiac myxomas are the most frequent cardiac tumors. Although histologically benign, in some cases myxomas may be lethal, due to impairment of cardiac dynamics and their thromboembolic potential. The study aimed to assess the clinical presentation of cardiac myxomas and their correlation with echocardiographic features and to describe the perioperative results and long-term outcome of surgically treated patients. METHODS: A prospective study of 53 patients with cardiac myxomas who were operated the Hospital Argerich, followed clinically and with echocardiography from 1993 until 2013. All patients underwent echocardiographic studies. RESULTS: The patient's mean age was 53 ± 16 years (62.3% were women). The most common findings were dyspnea followed by embolic events. Most tumors were localized in the left atrium (77.4%), mainly in the fossa ovalis (63%). Mean size of the tumors was 4.76 x 3.50 cm. Tumors were generally mobile (88%) and went beyond the valve plane, causing mild mitral or tricuspid valve obstruction (58%) and dilation of the respective atrial chamber. Patients whose tumors were obstructive had higher pulmonary artery systolic pressures (50 vs 33 mmHg p < 0.01). According to the echocardiographic appearance 67% of tumors had a smooth surface and the remaining 32% had a villous surface. All patients with embolic manifestations had tumors with a villous surface. CONCLUSIONS: Clinical presentation relates to the ultrasound characteristics of myxomas. Smooth tumors are larger, occur with obstructive symptoms, and benefit from an elective surgery, whereas villous myxomas entailed a high embolic risk and require prompt surgical treatment.

Mitral valve calcium assessment: An independent predictor of balloon valvuloplasty results.

OBJECTIVE: Percutaneous mitral valvuloplasty (PMV) is an effective treatment for patients with mitral valve stenosis. Echocardiographic score (ES) is a useful predictor of outcomes. However, mitral valve calcification (MVC) has been shown to predict immediate results even in patients with otherwise low ES. We sought to evaluate the usefulness of MVC assessment as a predictor of immediate and long-term outcomes after PMV. METHODS: PMV was performed in 168 consecutive patients. Clinical and echocardiographic variables were analyzed. Patients were classified into 2 groups: group 1: minimal MVC and group 2: moderate to severe MVC. Primary success was defined as post-PMV mitral valve area (MVA) ≥ 1.5 cm2 in the absence of major complications. Restenosis (RE) was defined as a decrease in MVA >50% of initial gain or a final MVA <1.5 cm2. RESULTS: Mean age was 46.5 ± 11 years, and 86.9% (146) were women. Forty-two patients (25%) had mild MVC (group 1), and 75% of the patients had moderate to severe MVC (group 2). Procedural success was achieved in 95.2% and 76.2% for groups 1 and 2, respectively, p = 0.01. MVA after PMV was 1.82 cm2 (IQR 25-75 = 1.60-2.00) in group 1 and 1.67 cm2 (IQR 25-75 = 1.44-1.97) in group 2, p = 0.02. After 48 months, 28.2% of patients presented RE. Multivariate analysis identified the presence of MVC as an independent predictor of poor immediate results (HR = 0.12, 95% IC 0.03-0.91) and RE (HR = 1.94, 95% CI = 1.02-5.21). CONCLUSION: Our study shows that the presence of MVC may predict immediate and long-term outcomes after PMV.

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.

Papillary Fibroelastoma of the Left Ventricle in a Radiation-treated Cancer Patient.

We present the case of a 69-year-old female patient with a history of endometrial carcinoma in 1996, who underwent a total hysterectomy and bilateral adnexectomy. The patient also received 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 and a papillary fibroelastoma in the left ventricle. In 2011, she underwent a mitral valve replacement with a biological prosthesis and the pathology exam revealed valve damage consistent with radiotherapy- induced changes and confirmed the presence of a papillary fibroelastoma. This unusual mechanism of papillary fibroelastoma 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.

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.

Speckle tracking echocardiography to assess regional ventricular function in patients with apical hypertrophic cardiomyopathy.

AIM: To explore regional systolic strain of midwall and endocardial segments using speckle tracking echocardiography in patients with apical hypertrophic cardiomyopathy (HCM). METHODS: We prospectively assessed 20 patients (mean age 53 ± 16 years, range: 18-81 years, 10 were male), with apical HCM. We measured global longitudinal peak systolic strain (GLPSS) in the midwall and endocardium of the left ventricle. RESULTS: The diastolic thickness of the 4 apical segments was 16.25 ± 2.75 mm. All patients had a normal global systolic function with a fractional shortening of 50% ± 8%. In spite of supernormal left ventricular (LV) systolic function, midwall GLPSS was decreased in all patients, more in the apical (-7.3% ± -8.8%) than in basal segments (-15.5% ± -6.93%), while endocardial GLPPS was significantly greater and reached normal values (apical: -22.8% ± -7.8%, basal: -17.9% ± -7.5%). CONCLUSION: This study shows that two-dimensional strain was decreased mainly confined to the mesocardium, while endocardium myocardial deformation was preserved in HCM and allowed to identify subclinical LV dysfunction. This transmural heterogeneity in systolic strain had not been previously described in HCM and could be explained by the distribution of myofibrillar disarray in deep myocardial areas. The clinical application of this novel finding may help further understanding of the pathophysiology of HCM.

Initial Results and Long-Term Follow-up of Percutaneous Mitral Valvuloplasty in Patients with Pulmonary Hypertension.

BACKGROUND: Percutaneous balloon mitral valvuloplasty (PMV) is an attractive therapeutic approach in patients with mitral stenosis. The aim of this study was to assess the immediate and long-term clinical, echocardiographic and haemodynamic outcomes of PMV in patients with severe pulmonary hypertension (PAH). METHODS: Percutaneous balloon mitral valvuloplasty was performed in 157 consecutive patients; 60 patients (38.2%) had significant PAH defined as baseline pulmonary artery mean pressure (PAMP) ≥ 30mm Hg (Group 1) and 97 patients (61.8%) had PAMP ≤ 30mmHg (Group 2). Pulmonary artery systolic pressure (PASP), mortality, need for mitral valve replacement or new PMV, and valve restenosis were evaluated during follow-up. RESULTS: Mean age was 44.2 years and 88.5% (139 patients) were women. Primary success was achieved in 79.6% of the patients (125 patients) without differences between the groups. Mitral valve area increased from 0.90cm2 to 1.76cm2, PASP fell from 57mmHg to 35mmHg in Group 1 and from 38mmHg to 30mmHg in Group 2. Median PASP in Group 1 was 35, 32, 36, 38 and 34mmHg at 12, 24, 36, 48 and 60 months. There were no significant differences in mitral valve area, PASP and clinical status between the groups. CONCLUSION: Percutaneous balloon mitral valvuloplasty is a safe and effective technique for the treatment of patients with mitral stenosis and PAH. A significant decrease in pulmonary pressure was observed after valvuloplasty. Although there was a gradual decrease of MVA at long-term follow-up, most patients remained asymptomatic and PASP was stable.