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.

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.

Subcostal right ventricular free wall strain in patients with pulmonary hypertension / Strain subcostal de la pared libre del ventriculo derecho en pacientes con hipertensión pulmonar

Abstract Right ventricular longitudinal strain (RVLS) is frequently used as a measure of right ventricular systolic function. Abnormal RV strain is associated with poor prognosis in patients with pulmonary hyper tension (PH); however, the measure is not always easy to obtain in patients with poor apical acoustic windows. Objective: This study aims to analyze the RVLS and determine if there is a difference when measured from the apical and subcostal views. Methods: In this cross-sectional study, we analyzed 22 adult outpatients (≥ 18 years old), 81% female, mean age 49.9 ± 17.3 years, with a diagnosis of PH using right heart catheterization, followed from January 2016 to January 2020. Results: RVLS measured in the RV free wall from the apical views was -15% (-19% to -10%) and subcostal views -14.5% (-18% to -11%) were highly correlated (Person's r = 0.969, p < 0.0001). Segment by segment analysis did not show significant differences either: basal four-chamber vs. sub costal view was -16.5% (-21% to -11%) vs. -15.5% (-20% to -11%), p = 0.99, mid four-chamber view vs. subcotal view was -16.5% (-21% to -12%) vs. -16.5% (-20% to -11%), p = 0.87, apical four-chamber view vs. subcostal view was -12% (-18% to -8%) vs. -13.5% (-19% to -10%), p = 0.93. Conclusion: Subcostal RVLS free wall is a feasible and accurate alternative to conventional RVLS free wall from the apical view in patients with pulmonary hypertension and could be useful in patients with poor acoustic apical four-chamber windows.

Resumen El strain longitudinal del ventrículo derecho (SLVD) permite medir la función sistólica del ventrículo derecho (VD). La disminución del strain (deformación) del VD se asocia con mal pronóstico en pacientes con hipertensión pulmonar (HP), pero no siempre es fácil de obtener en pacientes con mala ventana acústica apical. Objetivo: Este estudio tiene como objetivo analizar el SLVD y determinar si las vistas apical y subcostal son comparables. Métodos: En este estudio transversal, se incluyeron 22 pacientes adultos ambulatorios (≥18 años), 81% mujeres, edad promedio 49.9 ± 17.3 años, con diagnóstico de HP mediante cateterismo cardíaco derecho, seguidos desde enero de 2016 hasta enero de 2020. Se midió la deformación de la pared libre del ventrículo derecho desde las vistas de cuatro cámaras apical y cuatro cámaras subcostal. Resultados: El SLVD medido en la pared libre del VD desde la vista apical fue -15% (-19% a -10%) vs. -14.5% (-18% a -11%) cuando se midió desde la vista subcostal (p = 0,99). El análisis segmento por s egmento tampoco mostró diferencias significativas: el segmento basal apical vs. subcostal fue -16.5% (-21% a -11%) vs. -15.5% (-20% a -11%), p = 0.99, el segmento medio apical vs. la vista subcotal fue -16.5% (-21% a -12%) vs. a -16.5% (-20% a -11%), p = 0.87, el segmento apical vs. la vista subcostal fue -12% (-18% a -8%) frente a -13.5% (-19% a -10%), p = 0.93. Conclusión: En pacientes con HP, el SLVD obtenido en la pared libre subcostal es una alternativa útil en los casos con ventana acústica apical subóptima.

Subcostal right ventricular free wall strain in patients with pulmonary hypertension.

Right ventricular longitudinal strain (RVLS) is frequently used as a measure of right ventricular systolic function. Abnormal RV strain is associated with poor prognosis in patients with pulmonary hypertension (PH); however, the measure is not always easy to obtain in patients with poor apical acoustic windows. OBJECTIVE: This study aims to analyze the RVLS and determine if there is a difference when measured from the apical and subcostal views. METHODS: In this cross-sectional study, we analyzed 22 adult outpatients (= 18 years old), 81% female, mean age 49.9 ± 17.3 years, with a diagnosis of PH using right heart catheterization, followed from January 2016 to January 2020. RESULTS: RVLS measured in the RV free wall from the apical views was -15% (-19% to -10%) and subcostal views -14.5% (-18% to -11%) were highly correlated (Person's r = 0.969, p < 0.0001). Segment by segment analysis did not show significant differences either: basal four-chamber vs. subcostal view was -16.5% (-21% to -11%) vs. -15.5% (-20% to -11%), p = 0.99, mid four-chamber view vs. subcotal view was -16.5% (-21% to -12%) vs. -16.5% (-20% to -11%), p = 0.87, apical four-chamber view vs. subcostal view was -12% (-18% to -8%) vs. -13.5% (-19% to -10%), p = 0.93. CONCLUSION: Subcostal RVLS free wall is a feasible and accurate alternative to conventional RVLS free wall from the apical view in patients with pulmonary hypertension and could be useful in patients with poor acoustic apical four-chamber windows.

El strain longitudinal del ventrículo derecho (SLVD) permite medir la función sistólica del ventrículo derecho (VD). La disminución del strain (deformación) del VD se asocia con mal pronóstico en pacientes con hipertensión pulmonar (HP), pero no siempre es fácil de obtener en pacientes con mala ventana acústica apical. Objetivo: Este estudio tiene como objetivo analizar el SLVD y determinar si las vistas apical y subcostal son comparables. Métodos: En este estudio transversal, se incluyeron 22 pacientes adultos ambulatorios (=18 años), 81% mujeres, edad promedio 49.9 ± 17.3 años, con diagnóstico de HP mediante cateterismo cardíaco derecho, seguidos desde enero de 2016 hasta enero de 2020. Se midió la deformación de la pared libre del ventrículo derecho desde las vistas de cuatro cámaras apical y cuatro cámaras subcostal. Resultados: El SLVD medido en la pared libre del VD desde la vista apical fue -15% (-19% a -10%) vs. -14.5% (-18% a -11%) cuando se midió desde la vista subcostal (p = 0,99). El análisis segmento por s egmento tampoco mostró diferencias significativas: el segmento basal apical vs. subcostal fue -16.5% (-21% a -11%) vs. -15.5% (-20% a -11%), p = 0.99, el segmento medio apical vs. la vista subcotal fue -16.5% (-21% a -12%) vs. a -16.5% (-20% a -11%), p = 0.87, el segmento apical vs. la vista subcostal fue -12% (-18% a -8%) frente a -13.5% (-19% a -10%), p = 0.93. Conclusión: En pacientes con HP, el SLVD obtenido en la pared libre subcostal es una alternativa útil en los casos con ventana acústica apical subóptima.

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.

Value of Electrocardiography to Distinguish Fabry Disease from Sarcomeric Hypertrophic Cardiomyopathy.

Fabry disease (FD) is a rare genetic disorder that leads to left ventricular hypertrophy (LVH), frequently misdiagnosed as hypertrophic cardiomyopathy (HCM). We sought to assess the value of electrocardiography for distinguishing FD from HCM. We retrospectively reviewed and compared standard electrocardiograms and echocardiograms from 26 patients with FD and LVH and 33 sarcomeric patients with HCM, matched for gender, age, and degree of LVH. The mean age of patients with FD was 46 years (interquartile range) (28 to 53) and of HCM 50 (30 to 61) years (p = 0.27). Of them, 16 (61%) and 25 (76%) were male, respectively (p = 0.26). Indexed left ventricular mass was 166 g/m2 in FD versus 181 g/m2 in HCM (p = 0.88). All patients with FD and 30 (91%) with HCM were in sinus rhythm (p = 0.25). A higher prevalence of right bundle branch block (RBBB) was observed in FD (27%) versus HCM (6%) (p = 0.03). The PR interval was shorter in FD, 140 ms (120-160) versus 160 ms (140 to 180) (p = 0.004). P-wave duration was longer in patients with FD, 100 ms (80 to 120) versus 80 ms (80 to 100) (p = 0.01). The PQ interval (PR interval minus P-wave duration) was shorter in patients with FD, 40 ms (20 to 45) versus 80 ms (40 to 80) (p = 0.001). There were no differences regarding P-wave amplitude, QRS complex duration, corrected QT length, conduction or repolarization abnormalities, Sokolow-Lyon index, and Cornell index. After multivariate adjustments for RBBB, PR interval, P-wave duration, and PQ interval, a PQ interval ≤40 ms and RBBB were significantly associated with FD. In conclusion, there are electrocardiogram characteristics, such as the presence of RBBB or a PQ interval ≤40 ms, that may be helpful for screening and reducing the delay in FD diagnosis.

[Experience in infective endocarditis over 30 years in a hospital in Buenos Aires]. / Experiencia en endocarditis infecciosa a lo largo de 30 años en un hospital de Buenos Aires.

Infective endocarditis (IE) is a disease that in recent decades has shown changes in its presentation, diagnosis and treatment. This is a prospective study of 252 patients admitted at a reference hospital in Buenos Aires, Argentina, with a diagnosis of IE and they were grouped according to the decade of admission: Group A: from January 1988 to December 1997 (89 patients -35.3%-), Group B: from January 1998 to December 2007 (88 patients -34.9%-), and Group C: from January 2008 to December 2018 (75 patients -29.8%). The characteristics were analyzed and compared: age, sex, underlying heart disease, blood cultures and germs, presence of vegetations, surgical treatment and in-hospital mortality. Over the three decades, the predisposing heart condition showed that rheumatic valve disease decreased significantly (p < 0.0001) while the IE in cardiac devices also increased significantly (p < 0.0001). The percentage of blood culture-negatives decreased significantly over the years (p < 0.0001). In-hospital mortality showed a downward trend in the last decade (p = 0.069). The development of complications during hospitalization, the indication for surgery, and the presence of heart failure on admission were independent predictors of in-hospital mortality. The presence of vegetations and febrile syndrome on admission were independent predictor for lower mortality. The comparison over the years showed important changes in the epidemiological profile of IE. Probably due to advances in diagnostic techniques, treatment, and the implementation of interdisciplinary IE teams in the last decade, in-hospital mortality shows a strong tendency to decrease.

La endocarditis infecciosa (EI) es una enfermedad que en las últimas décadas ha mostrado cambios en su presentación, diagnóstico y tratamiento. Se realizó un estudio prospectivo de 252 pacientes ingresados en un hospital de referencia en Buenos Aires, Argentina, con diagnóstico de EI, agrupados según década de ingreso: Grupo A: enero 1988 a diciembre 1997 (89 pacientes ­35.3%­), Grupo B: enero 1998 a diciembre 2007 (88 pacientes ­34.9%­), y Grupo C: enero 2008 a diciembre 2018 (75 pacientes ­29.8%). Se analizaron y compararon las características: edad, sexo, cardiopatía de base, hemocultivos y gérmenes, presencia de vegetaciones, tratamiento quirúrgico y mortalidad intrahospitalaria. Durante las tres décadas, la cardiopatía predisponente mostró que la enfermedad valvular reumática disminuyó significativamente (p < 0.0001) mientras que la EI en los dispositivos cardíacos aumentó significativamente (p < 0.0001). El porcentaje de hemocultivos negativos disminuyó significativamente a lo largo de los años (p < 0.0001). La mortalidad hospitalaria mostró una reducción en la última década (p = 0.069). El desarrollo de complicaciones durante la hospitalización, la indicación de cirugía y la presencia de insuficiencia cardíaca al ingreso fueron predictores independientes de mortalidad hospitalaria. La presencia de vegetaciones y síndrome febril al ingreso fueron predictores independientes de menor mortalidad. La comparación a través de los años mostró cambios importantes en el perfil epidemiológico de la EI. Probablemente por el avance en las técnicas diagnósticas, el tratamiento y la implementación de equipos interdisciplinarios de EI de la última década, la mortalidad intrahospitalaria marca una fuerte tendencia a la reducción.

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.

[Cardiac metastasis from primary anaplastic thyroid carcinoma]. / Metástasis cardíaca de carcinoma anaplásico de tiroides.

Cardiac neoplasms are rare entities in the clinical practice. Cardiac metastatic involvement is 20 to 40 times more frequent than the primary form, representing 95% of all cardiac tumors; however, they are frequently underdiagnosed because of their clinical and oncologic features. In this report, we present two cases of cardiac metastasis from primary anaplastic thyroid carcinoma: a 69-years-old woman with right ventricular metastasis and a 61-years-old man with right atrial metastasis. Both patients died during their hospitalization and one of them underwent an autopsy.

Las neoplasias cardíacas son entidades poco frecuentes en la práctica clínica cardiológica y dentro de éstas, la afectación metastásica es 20 a 40 veces más frecuente que la forma primaria, correspondiendo al 95% de todos los tumores cardíacos; no obstante, debido a las características clínicas y oncológicas del tumor primario, los tumores cardíacos metastásicos son habitualmente subdiagnosticados. En este trabajo se presentan dos casos de pacientes con carcinoma anaplásico de tiroides, una mujer de 69 años con metástasis en ventrículo derecho y un varón de 61 años con metástasis en aurícula derecha. Ambos pacientes fallecieron durante la internación y a uno de ellos se le realizó autopsia.

Metástasis cardíaca de carcinoma anaplásico de tiroides / Cardiac metastasis from primary anaplastic thyroid carcinoma

Resumen Las neoplasias cardíacas son entidades poco frecuentes en la práctica clínica cardiológica y dentro de éstas, la afectación metastásica es 20 a 40 veces más frecuente que la forma primaria, corres pondiendo al 95% de todos los tumores cardíacos; no obstante, debido a las características clínicas y oncológicas del tumor primario, los tumores cardíacos metastásicos son habitualmente subdiagnosticados. En este trabajo se presentan dos casos de pacientes con carcinoma anaplásico de tiroides, una mujer de 69 años con metástasis en ventrículo derecho y un varón de 61 años con metástasis en aurícula derecha. Ambos pacientes fallecieron durante la internación y a uno de ellos se le realizó autopsia.

Abstract Cardiac neoplasms are rare entities in the clinical practice. Cardiac metastatic involvement is 20 to 40 times more frequent than the primary form, representing 95% of all cardiac tumors; however, they are frequently underdiagnosed because of their clinical and oncologic features. In this report, we present two cases of cardiac metastasis from primary anaplastic thyroid carcinoma: a 69-years-old woman with right ventricular metastasis and a 61-years-old man with right atrial metastasis. Both patients died during their hospitalization and one of them underwent an autopsy.

Experiencia en endocarditis infecciosa a lo largo de 30 años en un hospital de Buenos Aires / Experience in infective endocarditis over 30 years in a hospital in Buenos Aires

Resumen La endocarditis infecciosa (EI) es una enfermedad que en las últimas décadas ha mostrado cambios en su presentación, diagnóstico y tratamiento. Se realizó un estudio prospectivo de 252 pacientes ingresados en un hospital de referencia en Buenos Aires, Argentina, con diagnóstico de EI, agrupados según década de ingreso: Grupo A: enero 1988 a diciembre 1997 (89 pacientes -35.3%-), Grupo B: enero 1998 a diciembre 2007 (88 pacientes -34.9%-), y Grupo C: enero 2008 a diciembre 2018 (75 pacientes -29.8%). Se analizaron y compararon las características: edad, sexo, cardiopatía de base, hemocultivos y gérmenes, presencia de vegetaciones, tratamiento quirúrgico y mortalidad intrahospitalaria. Durante las tres décadas, la cardiopatía predisponente mostró que la enfermedad valvular reumática disminuyó significativamente (p < 0.0001) mientras que la EI en los dispositivos cardíacos aumentó significativamente (p < 0.0001). El porcentaje de hemocultivos negativos disminuyó significativamente a lo largo de los años (p < 0.0001). La mortalidad hospitalaria mostró una reducción en la última década (p = 0.069). El desarrollo de complicaciones durante la hospitalización, la indicación de cirugía y la presencia de insuficiencia cardíaca al ingreso fueron predictores independientes de mortalidad hospitalaria. La presencia de vegetaciones y síndrome febril al ingreso fueron predictores independientes de menor mortalidad. La comparación a través de los años mostró cambios importantes en el perfil epidemiológico de la EI. Probablemente por el avance en las técnicas diagnósticas, el tratamiento y la implementación de equi pos interdisciplinarios de EI de la última década, la mortalidad intrahospitalaria marca una fuerte tendencia a la reducción.

Abstract Infective endocarditis (IE) is a disease that in recent decades has shown changes in its presentation, diagno sis and treatment. This is a prospective study of 252 patients admitted at a reference hospital in Buenos Aires, Argentina, with a diagnosis of IE and they were grouped according to the decade of admission: Group A: from January 1988 to December 1997 (89 patients -35.3%-), Group B: from January 1998 to December 2007 (88 patients -34.9%-), and Group C: from January 2008 to December 2018 (75 patients -29.8%). The characteristics were analyzed and compared: age, sex, underlying heart disease, blood cultures and germs, presence of veg etations, surgical treatment and in-hospital mortality. Over the three decades, the predisposing heart condition showed that rheumatic valve disease decreased significantly (p < 0.0001) while the IE in cardiac devices also increased significantly (p < 0.0001). The percentage of blood culture-negatives decreased significantly over the years (p < 0.0001). In-hospital mortality showed a downward trend in the last decade (p = 0.069). The devel opment of complications during hospitalization, the indication for surgery, and the presence of heart failure on admission were independent predictors of in-hospital mortality. The presence of vegetations and febrile syndrome on admission were independent predictor for lower mortality. The comparison over the years showed important changes in the epidemiological profile of IE. Probably due to advances in diagnostic techniques, treatment, and the implementation of interdisciplinary IE teams in the last decade, in-hospital mortality shows a strong tendency to decrease.

Rapidly progressive aortic stenosis treated with transcatheter aortic valve implantation in a patient with Fabry disease: a case report.

BACKGROUND: Fabry disease (FD) is a rare lysosomal storage disease, caused by mutations in the gene encoding the enzyme α-galactosidase A (α-Gal A). Cardiac involvement is one of the main causes of death and it is characterized by progressive concentric left ventricular hypertrophy (LVH), which in most cases is symmetric. Mild thickening of the left-sided valves is seen in as many as a quarter of patients. Severe aortic stenosis is an extremely rare disorder in FD. CASE SUMMARY: In this report, we describe the case of a 57-year-old male, who was diagnosed with a cardiac variant of FD 10 years ago. Since the patient had severe LVH, he was started on enzyme replacement therapy when he was 47 years old with an intravenous infusion of 0.2 mg/kg of agalsidase alpha every 14 days. The patient remained stable and asymptomatic for 9 years, until he presented with dyspnoea in New York Heart Association functional class II-III and severe aortic stenosis (aortic valve area: 0.97 cm2) together with severe systolic dysfunction [ejection fraction (EF): 29%]. Because of the patient's comorbidities and high surgical risk, he underwent successful transfemoral transcatheter aortic valve implantation (TAVI). At 2 months following TAVI, the patient was asymptomatic and, in spite of his Fabry cardiomyopathy, the EF had increased to 45%. DISCUSSION: To our knowledge, this is the first case in the literature to demonstrate a rapid progression of aortic stenosis with severe impairment of left ventricular function and worsening in functional class in a patient with FD, who following TAVI improved his EF, with disappearance of symptoms and ventricular arrhythmias.

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.

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.

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.