[Cortical blindness as initial manifestation of nonbacterial thrombotic endocarditis]. / Ceguera cortical como manifestación inicial de endocarditis trombótica no bacteriana.

Nonbacterial thrombotic endocarditis, formerly known as marantic endocarditis, it is an infrequent entity in which sterile, fibrin vegetations develop on heart valve leaflets. It is often diagnosed at the time of autopsy or in latestage malignancies. The most common malignancies associated with nonbacterial thrombotic endocarditis are lung, pancreatic, gastric cancer and adenocarcinomas of an unknown primary site. Diagnosis requires ruling out infective endocarditis and establishing the presence of valvular vegetations using echocardiography. We report the case of a patient with a recent diagnosis of advanced gastric adenocarcinoma who presented with cortical blindness. The computed tomography was compatible with cerebral ischemia. The transoesophageal echocardiogram showed two vegetations in mitral valve. Blood cultures were negative. We emphasize the importance of suspecting nonbacterial thrombotic endocarditis in patients with cancer and systemic embolism.

[Papillary fibroelastoma: retrospective analysis. Clinical presentation and surgical results]. / Fibroelastoma papilar cardíaco: estudio retrospectivo. Presentación clínica y resultados quirúrgicos.

Papillary ibroelastomas are small benign intracardiac tumors known for their embolic potential. Since the introduction of echocardiography with improved resolution and transesophageal imaging techniques, they are being increasingly detected in clinical practice. In recent series, papillary fibroelastoma is considered the most frequent benign tumor of the heart. Our objective was to analyze characteristics and midterm surgical outcome of histologically-confirmed cases of papillary fibroelastoma. We conducted a retrospective study on patients with cardiac tumors submitted to surgical excision between June 1992 and February 2017. Out of 108 patients, 18 had papillary fibroelastomas. Their mean age was 58 years (22-77); 10 were men. The most frequent localizations were the aortic valve (7) and the mitral valve (5). None had significant valvular dysfunction. By transesophageal echocardiography, the tumor size (larger diameter) was 13.33 ± 5.55 mm (6.6-28.0). Two patients, both with tumor in the aortic valve, had suffered a stroke; other two had dyspnoea and atrial flutter, respectively. The remaining 14 patients were asymptomatic and their tumors were incidental findings. In 15 patients the valve was preserved. There was neither surgical mortality nor recurrence after 2.6 years of follow-up. In conclusion, most papillary fibroelastomas can be surgically removed with valve preservation and favorable clinical outcome. However, until the results of randomized trials support the decision, an aggressive surgical approach in asymptomatic patients needs to be defined in the context of surgical expertise.

[Acute aortic regurgitation due to infective endocarditis]. / Insuficiencia aórtica aguda por endocarditis infecciosa.

Acute aortic regurgitation (AAR) due to infective endocarditis (IE) is a serious disease and usually requires surgical treatment. Our study aims to compare the clinical, echocardiographic, and microbiological characteristics as well as in-hospital outcome of patients with AAR according to the severity of heart failure (HF) and to evaluate predictors of in-hospital mortality in a tertiary centre. In a prospective analysis, we compared patients with NYHA functional class I-II HF (G1) vs. functional class III-IV HF (G2). From 06/92 to 07/16, 439 patients with IE were hospitalized; 86 presented AAR: (G1, 39: 45.4% y G2, 47: 54.7%). The G1 had higher prosthetic IE (43.6% vs. 17%, p 0.01). All G2 patients had dyspnoea vs. 30.8% of the G1 (p < 0.0001). There were no differences in clinical, echocardiographic and microbiological characteristics. Surgical treatment was indicated mainly due to infection extension or valvular dysfunction in G1 and HF in G2. In-hospital mortality was 15.4% vs. 27.7% (G1 and G2 respectively p NS). In multivariate analysis, health care-associated acquisition (p 0.001), negative blood cultures (p 0.004), and functional class III-IV HF (p 0.039) were in-hospital mortality predictors. One-fifth of the patients with EI had AAR. Half of them had severe HF which needed emergency surgery and the remaining needed surgery for extension of the infection and / or valvular dysfunction. Both groups remain to have high surgical and in-hospital mortality. Health care-associated acquisition, negative blood cultures and advanced HF were predictors of in-hospital mortality.

Influence of the timing of cardiac surgery on the outcome of patients with infective endocarditis and stroke.

BACKGROUND: The timing of cardiac surgery after stroke in infective endocarditis (IE) remains controversial. We examined the relationship between the timing of surgery after stroke and the incidence of in-hospital and 1-year mortalities. METHODS: Data were obtained from the International Collaboration on Endocarditis-Prospective Cohort Study of 4794 patients with definite IE who were admitted to 64 centers from June 2000 through December 2006. Multivariate logistic regression and Cox regression analyses were performed to estimate the impact of early surgery on hospital and 1-year mortality after adjustments for other significant covariates. RESULTS: Of the 857 patients with IE complicated by ischemic stroke syndromes, 198 who underwent valve replacement surgery poststroke were available for analysis. Overall, 58 (29.3%) patients underwent early surgical treatment vs 140 (70.7%) patients who underwent late surgical treatment. After adjustment for other risk factors, early surgery was not significantly associated with increased in-hospital mortality rates (odds ratio, 2.308; 95% confidence interval [CI], .942-5.652). Overall, probability of death after 1-year follow-up did not differ between 2 treatment groups (27.1% in early surgery and 19.2% in late surgery group, P = .328; adjusted hazard ratio, 1.138; 95% CI, .802-1.650). CONCLUSIONS: There is no apparent survival benefit in delaying surgery when indicated in IE patients after ischemic stroke. Further observational analyses that include detailed pre- and postoperative clinical neurologic findings and advanced imaging data (eg, ischemic stroke size), may allow for more refined recommendations on the optimal timing of valvular surgery in patients with IE and recent stroke syndromes.

Analysis of the impact of early surgery on in-hospital mortality of native valve endocarditis: use of propensity score and instrumental variable methods to adjust for treatment-selection bias.

BACKGROUND: The impact of early surgery on mortality in patients with native valve endocarditis (NVE) is unresolved. This study sought to evaluate valve surgery compared with medical therapy for NVE and to identify characteristics of patients who are most likely to benefit from early surgery. METHODS AND RESULTS: Using a prospective, multinational cohort of patients with definite NVE, the effect of early surgery on in-hospital mortality was assessed by propensity-based matching adjustment for survivor bias and by instrumental variable analysis. Patients were stratified by propensity quintile, paravalvular complications, valve perforation, systemic embolization, stroke, Staphylococcus aureus infection, and congestive heart failure. Of the 1552 patients with NVE, 720 (46%) underwent early surgery and 832 (54%) were treated with medical therapy. Compared with medical therapy, early surgery was associated with a significant reduction in mortality in the overall cohort (12.1% [87/720] versus 20.7% [172/832]) and after propensity-based matching and adjustment for survivor bias (absolute risk reduction [ARR] -5.9%, P<0.001). With a combined instrument, the instrumental-variable-adjusted ARR in mortality associated with early surgery was -11.2% (P<0.001). In subgroup analysis, surgery was found to confer a survival benefit compared with medical therapy among patients with a higher propensity for surgery (ARR -10.9% for quintiles 4 and 5, P=0.002) and those with paravalvular complications (ARR -17.3%, P<0.001), systemic embolization (ARR -12.9%, P=0.002), S aureus NVE (ARR -20.1%, P<0.001), and stroke (ARR -13%, P=0.02) but not those with valve perforation or congestive heart failure. CONCLUSIONS: Early surgery for NVE is associated with an in-hospital mortality benefit compared with medical therapy alone.

[Clinical finding and follow-up of chronic constrictive pericarditis]. / Clínica y seguimiento de la pericarditis constrictiva crónica.

The aim of this study was to describe the etiology, clinical findings, diagnostic methods, treatment, outcome and long-term prognosis of 35 patients with chronic constrictive pericarditis (CCP) that were prospectively analyzed according to a pericardial disease protocol performed in our Institution. Etiology of CCP was idiopathic in 24 patients (68%), and specific in 11 (32%). The majority (34 patients, 97%) underwent pericardiectomy. Perioperative mortality was 12% (4/33) no deaths were registered among patients with idiopathic CCP. Median follow-up was 5.6 years (percentile 25-75: 2.4-7.4 years). The cumulative actuarial survival probability was 97% at 1 year (confidence interval [CI] 80% to 99%); 83% at 5 years, (95% CI 65% to 93%); 78% at 7 years, (95% CI 60% to 90%), and 69% at 10 years (95% CI 50% to 84%). In conclusion, nowadays CCP is generally an idiopathic disease with late diagnosis. The clinical course of the disease produces severe symptoms of congestive heart failure. In a 10 years follow-up 2/3 of patients are alive and improved their quality of life. Idiopathic form of pericarditis did not show mortality during early postoperative period.

[Infective endocarditis. A changing disease]. / Endocarditis infecciosa. Una enfermedad cambiante.

In spite of the advance in diagnosis and antibiotic and surgical treatment, mortality of infective endocarditis remains high. Recent studies in various countries and in Argentina have shown that the clinical profile of infective endocarditis has changed. Currently the patients are older with higher frequency of degenerative valve disease and prosthetic valve and nosocomial endocarditis. The incidence of Staphylococcus aureus as etiological agent has increased. These changes may justify the absence of decrease in mortality and suggests that more aggressive measures are needed based on prospective, well designed and controlled trials.

Contemporary clinical profile and outcome of prosthetic valve endocarditis.

CONTEXT: Prosthetic valve endocarditis (PVE) is associated with significant mortality and morbidity. The contemporary clinical profile and outcome of PVE are not well defined. OBJECTIVES: To describe the prevalence, clinical characteristics, and outcome of PVE, with attention to health care-associated infection, and to determine prognostic factors associated with in-hospital mortality. DESIGN, SETTING, AND PARTICIPANTS: Prospective, observational cohort study conducted at 61 medical centers in 28 countries, including 556 patients with definite PVE as defined by Duke University diagnostic criteria who were enrolled in the International Collaboration on Endocarditis-Prospective Cohort Study from June 2000 to August 2005. MAIN OUTCOME MEASURE: In-hospital mortality. RESULTS: Definite PVE was present in 556 (20.1%) of 2670 patients with infective endocarditis. Staphylococcus aureus was the most common causative organism (128 patients [23.0%]), followed by coagulase-negative staphylococci (94 patients [16.9%]). Health care-associated PVE was present in 203 (36.5%) of the overall cohort. Seventy-one percent of health care-associated PVE occurred within the first year of valve implantation, and the majority of cases were diagnosed after the early (60-day) period. Surgery was performed in 272 (48.9%) patients during the index hospitalization. In-hospital death occurred in 127 (22.8%) patients and was predicted by older age, health care-associated infection (62/203 [30.5%]; adjusted odds ratio [OR], 1.62; 95% confidence interval [CI], 1.08-2.44; P = .02), S aureus infection (44/128 [34.4%]; adjusted OR, 1.73; 95% CI, 1.01-2.95; P = .05), and complications of PVE, including heart failure (60/183 [32.8%]; adjusted OR, 2.33; 95% CI, 1.62-3.34; P<.001), stroke (34/101 [33.7%]; adjusted OR, 2.25; 95% CI, 1.25-4.03; P = .007), intracardiac abscess (47/144 [32.6%]; adjusted OR, 1.86; 95% CI, 1.10-3.15; P = .02), and persistent bacteremia (27/49 [55.1%]; adjusted OR, 4.29; 95% CI, 1.99-9.22; P<.001). CONCLUSIONS: Prosthetic valve endocarditis accounts for a high percentage of all cases of infective endocarditis in many regions of the world. Staphylococcus aureus is now the leading cause of PVE. Health care-associated infection significantly influences the clinical characteristics and outcome of PVE. Complications of PVE strongly predict in-hospital mortality, which remains high despite prompt diagnosis and the frequent use of surgical intervention.

Epidemiologic, clinical, and microbiologic profile of infective endocarditis in Argentina: a national survey. The Endocarditis Infecciosa en la República Argentina-2 (EIRA-2) Study.

BACKGROUND: This study aimed to determine the epidemiologic, clinical, microbiologic characteristics, and inhospital outcome of infective endocarditis (IE) in Argentina and compare the results with those of the 1992 IE national survey. METHODS: A prospective, multicenter study was conducted in 82 hospitals representing 16 of 24 provinces of Argentina. Patients with diagnosis of IE according to the Duke criteria were surveyed during an 18-month period. RESULTS: From 470 surveyed episodes of IE, 390 cases were classified as definite and 80 as possible IE. The mean age of the definite IE cases was 58.5 +/- 17.3 years; male sex, 70.0%; and male-female ratio, 2.3:1. Pathological evidence of IE was available in 26.2%. There was no previously known heart disease in 35.1%, and the proportion of prosthetic valve IE was 15.9%. Causative microorganisms were streptococci, 38.3% (Streptococcus viridans 27.0%, Streptococcus bovis 5.2%, others 6.1%); enterococci, 10.2%; staphylococci, 36.7% (Staphylococcus aureus 29.8%, coagulase-negative staphylococci 6.9%); HACEK group, 6.1%; fungal, 1.4%; and polymicrobial, 2.0%. Blood culture results were negative in 10.8%. Surgical treatment was performed in 26.2%, and the overall inhospital mortality was 24.6%. Patients from the 2002 survey were older (58.5 +/- 17.3 vs 51.3 +/- 18.7 years, P < .01) and more frequently had underlying heart disease (64.9% vs 55.0%, P < .01): degenerative valve disease (11.5% vs 4.8%, P < .01), congenital heart disease (9.5% vs 4.2%, P < .01), and prosthetic valve IE (15.9% vs 8.5%, P < .01). Conversely, the prevalence of rheumatic valve disease was significantly less than in the 1992 survey (5.4% vs 13.0%, P < .01). CONCLUSIONS: The EIRA-2 survey shows that the clinical profile of IE has changed in Argentina. Currently, patients with IE are older and have a higher frequency of underlying heart disease, degenerative valve disease, and prosthetic valve IE than previously. The incidence of staphylococcal IE has increased. Inhospital mortality remains high, suggesting that more aggressive measures are needed for the early identification, prevention, and treatment of IE.

Validation of the 2014 European Society of Cardiology Sudden Cardiac Death Risk Prediction Model in Hypertrophic Cardiomyopathy in a Reference Center in South America.

Sudden cardiac death (SCD) is a common cause of death in hypertrophic cardiomyopathy (HC). Our aim was to conduct an external and independent validation in South America of the 2014 European Society of Cardiology (ESC) SCD risk prediction model to identify patients requiring an implantable cardioverter defibrillator. This study included 502 consecutive patients with HC followed from March, 1993 to December, 2014. A combined end point of SCD or appropriate implantable cardioverter defibrillator therapy was assessed. For the quantitative estimation of individual 5-year SCD risk, we used the formula: 1 - 0.998(exp(Prognostic index)). Our database also included the abnormal blood pressure response to exercise as a risk marker. We analyzed the 3 categories of 5-year risk proposed by the ESC: low risk (LR) <4%; intermediate risk (IR) ≥4% to <6%, and high risk (HR) ≥6%. The LR group included 387 patients (77%); the IR group 39 (8%); and the HR group 76 (15%). Fourteen patients (3%) had SCD/appropriate implantable cardioverter defibrillator therapy (LR: 0%; IR: 2 of 39 [5%]; and HR: 12 of 76 [16%]). In a receiver-operating characteristic curve, the new model proved to be an excellent predictor because the area under the curve for the estimated risk is 0.925 (statistical C: 0.925; 95% CI 0.8884 to 0.9539, p <0.0001). In conclusion, the SCD risk prediction model in HC proposed by the 2014 ESC guidelines was validated in our population and represents an improvement compared with previous approaches. A larger multicenter, independent and external validation of the model with long-term follow-up would be advisable.

Ceguera cortical como manifestación inicial de endocarditis trombótica no bacteriana / Cortical blindness as initial manifestation of nonbacterial thrombotic endocarditis

La endocarditis trombótica no bacteriana, antiguamente conocida como endocarditis marántica, es una entidad infrecuente en la que se desarrollan vegetaciones estériles, compuestas por fibrina en las válvulas del corazón. Suele diagnosticarse en el momento de la autopsia o en enfermedades oncológicas avanzadas. Las neoplasias malignas más frecuentemente asociadas con esta entidad son las de pulmón, páncreas, estómago y adenocarcinomas de origen primario desconocido. Es necesario descartar la endocarditis infecciosa y establecer la presencia de vegetaciones valvulares mediante ecocardiografía. Presentamos el caso de una paciente con diagnóstico reciente de adenocarcinoma de estómago en estadio avanzado que presentó ceguera cortical e imágenes compatibles con isquemia cerebral. El ecocardiograma transesofágico mostró dos vegetaciones en válvula mitral. Los hemocultivos fueron negativos. Se enfatiza la importancia de sospechar endocarditis trombótica no bacteriana en enfermos con cáncer y embolismo sistémico.

Nonbacterial thrombotic endocarditis, formerly known as marantic endocarditis, it is an infrequent entity in which sterile, fibrin vegetations develop on heart valve leaflets. It is often diagnosed at the time of autopsy or in late-stage malignancies. The most common malignancies associated with nonbacterial thrombotic endocarditis are lung, pancreatic, gastric cancer and adenocarcinomas of an unknown primary site. Diagnosis requires ruling out infective endocarditis and establishing the presence of valvular vegetations using echocardiography. We report the case of a patient with a recent diagnosis of advanced gastric adenocarcinoma who presented with cortical blindness. The computed tomography was compatible with cerebral ischemia. The transoesophageal echocardiogram showed two vegetations in mitral valve. Blood cultures were negative. We emphasize the importance of suspecting nonbacterial thrombotic endocarditis in patients with cancer and systemic embolism.

Fibroelastoma papilar cardíaco: estudio retrospectivo: Presentación clínica y resultados quirúrgicos / Fibroelastoma papilar cardíaco: estudio retrospectivo: Presentación clínica y resultados quirúrgicos

Los fibroelastomas papilares cardíacos son pequeños tumores benignos con potencial embolígeno. Con la incorporación de la ecocardiografía y la mejoría en la resolución de las imágenes, el diagnóstico clínico es cada vez más frecuente, y en series recientes su frecuencia supera a la del mixoma cardíaco. Nuestro objetivo fue analizar las características de una serie de casos de fibroelastoma papilar cardíaco con confirmación histológica operados en nuestro hospital. Analizamos retrospectivamente los tumores cardíacos y las características clínicas de los pacientes operados desde junio de 1992 a febrero de 2017. De 108 operados, 18 presentaron fibroelastoma papilar. La edad media del grupo con fibroelastoma papilar fue 58 años (22-77); 10 eran varones. Las localizaciones más frecuentes fueron la válvula aórtica (7) y la válvula mitral (5). Ninguno presentó disfunción valvular significativa. Por ecografía transesofágica, el tamaño (diámetro mayor) fue 13.33 ± 5.55 mm (6.6-28.0). Cuatro pacientes eran sintomáticos; uno presentaba disnea, otro aleteo auricular, dos con fibroelastoma en válvula aórtica habían sufrido un accidente cerebrovascular. Los restantes, asintomáticos, fueron intervenidos preventivamente. En 15 pacientes el tumor se extirpó sin necesidad de reemplazo valvular. No hubo mortalidad quirúrgica ni recidiva tumoral en el seguimiento de 2.6 años. Se concluye que los fibroelastomas papilares cardíacos pueden ser extirpados con buenos resultados a mediano plazo y, en su mayoría, con preservación valvular. Sin embargo, no existen aún estudios aleatorizados que avalen la escisión quirúrgica en pacientes asintomáticos. Por el momento, esta última depende de la experiencia del grupo quirúrgico tratante.

Papillary ibroelastomas are small benign intracardiac tumors known for their embolic potential. Since the introduction of echocardiography with improved resolution and transesophageal imaging techniques, they are being increasingly detected in clinical practice. In recent series, papillary fibroelastoma is considered the most frequent benign tumor of the heart. Our objective was to analyze characteristics and midterm surgical outcome of histologically-confirmed cases of papillary fibroelastoma. We conducted a retrospective study on patients with cardiac tumors submitted to surgical excision between June 1992 and February 2017. Out of 108 patients, 18 had papillary fibroelastomas. Their mean age was 58 years (22-77); 10 were men. The most frequent localizations were the aortic valve (7) and the mitral valve (5). None had significant valvular dysfunction. By transesophageal echocardiography, the tumor size (larger diameter) was 13.33 ± 5.55 mm (6.6-28.0). Two patients, both with tumor in the aortic valve, had suffered a stroke; other two had dyspnoea and atrial flutter, respectively. The remaining 14 patients were asymptomatic and their tumors were incidental findings. In 15 patients the valve was preserved. There was neither surgical mortality nor recurrence after 2.6 years of follow-up. In conclusion, most papillary fibroelastomas can be surgically removed with valve preservation and favorable clinical outcome. However, until the results of randomized trials support the decision, an aggressive surgical approach in asymptomatic patients needs to be defined in the context of surgical expertise.

Insuficiencia aórtica aguda por endocarditis infecciosa / Acute aortic regurgitation due to infective endocarditis

La insuficiencia aórtica aguda (IAOA) por endocarditis infecciosa (EI) es grave y generalmente requiere tratamiento quirúrgico. Se compararon los pacientes con IAOA grave por EI e insuficiencia cardíaca (IC) en clase funcional I-II NYHA (G1) con los pacientes en clase funcional III-IV (G2) en relación a características clínicas, ecocardiográficas, microbiológicas y evolución hospitalaria y se evaluaron los predictores de mortalidad, en un centro de alta complejidad. Desde 06/92 a 07/16, de 439 pacientes con EI, 86 presentaron IAOA: (G1, 39: 45.4% y G2, 47: 54.7%). El G1 presentó mayor EI protésica (43.6% vs. 17.0%; p < 0.01). Los 47 casos G2 presentaban disnea vs. 12 (30.8%) G1 (p < 0.0001). No hubo diferencias en cuanto a las características clínicas, ecocardiográficas y microbiológicas. El tratamiento quirúrgico fue principalmente por extensión de la infección y/disfunción valvular en el G1 y por IC en el G2. La mortalidad hospitalaria fue del 15.4% vs. 27.7% (G1 y G2 respectivamente, p NS). Fueron predictores en el análisis multivariado: la infección intrahospitalaria (p 0.001), los hemocultivos negativos (p 0.004) y la presencia de IC clase funcional III-IV (p 0.039).Una quinta parte de los pacientes con EI presentaron IAOA. Aquellos con IC grave requirieron tratamiento quirúrgico de emergencia y con IC con clase funcional I-II requirieron cirugía por extensión de la infección y/o disfunción valvular. La mortalidad quirúrgica y hospitalaria continúan siendo elevadas en ambos grupos y fueron predictores de mortalidad hospitalaria: la infección intrahospitalaria, los hemocultivos negativos y la IC avanzada.

Acute aortic regurgitation (AAR) due to infective endocarditis (IE) is a serious disease and usually requires surgical treatment. Our study aims to compare the clinical, echocardiographic, and microbiological characteristics as well as in-hospital outcome of patients with AAR according to the severity of heart failure (HF) and to evaluate predictors of in-hospital mortality in a tertiary centre. In a prospective analysis, we compared patients with NYHA functional class I-II HF (G1) vs. functional class III-IV HF (G2). From 06/92 to 07/16, 439 patients with IE were hospitalized; 86 presented AAR: (G1, 39: 45.4% y G2, 47: 54.7%). The G1 had higher prosthetic IE (43.6% vs. 17%, p 0.01). All G2 patients had dyspnoea vs. 30.8% of the G1 (p < 0.0001). There were no differences in clinical, echocardiographic and microbiological characteristics. Surgical treatment was indicated mainly due to infection extension or valvular dysfunction in G1 and HF in G2. In-hospital mortality was 15.4% vs. 27.7% (G1 and G2 respectively p NS). In multivariate analysis, health care-associated acquisition (p 0.001), negative blood cultures (p 0.004), and functional class III-IV HF (p 0.039) were in-hospital mortality predictors. One-fifth of the patients with EI had AAR. Half of them had severe HF which needed emergency surgery and the remaining needed surgery for extension of the infection and / or valvular dysfunction. Both groups remain to have high surgical and in-hospital mortality. Health care-associated acquisition, negative blood cultures and advanced HF were predictors of in-hospital mortality.

Role of stress Echo on the Prognosis of Hypertrophic Cardiomyopathy / Rol del eco estrés en el pronóstico de la miocardiopatía hipertrófica

Background: Patients with hypertrophic cardiomyopathy (HCM) frequently present with confusing and misleading symptoms. In these instances stress tests may help to stratify the risk of future events. Objective: The purpose of this study was to assess the prognostic usefulness of the different variables obtained with exercise stress echo (ESE) in patients with HCM. Methods: A retrospective and observational study was performed in 110 patients evaluated with ESE. Patients were divided into 3 groups according to their left ventricular outflow tract obstruction level (LVOTO): 1) persistent LVOTO (peak instantaneous gradient at rest obtained by continuous Doppler = 30 mmHg), 2) latent LVOTO (gradient = 50 mmHg with exercise); and no LVOTO. Median follow-up was 2.7 years. The primary endpoint was the composite of death, sudden death, sustained ventricular tachycardia or hospitalization for heart failure. Results: Persistent LVOTO was present in 19.1% of cases, latent LVOTO in 31.8% and no LVOTO in 49.1%. Ventricular function, wall thicknesses and diameters were similar for the three groups. Poor prognostic variables were significantly higher for persistent LVOTO. The latent LVOTO group developed more symptoms, electrocardiographic changes and mitral regurgitation after exercise than the group without LVOTO, although it was not associated with a higher number of events.Variables that were associated with increased rate of events during follow-up were the presence of gradient = 30 mmHg at rest (p=0.07), electrocardiographic changes during the test (p=0.020) and the inverse relationship of METs (p=0.07). Conclusions: Patients with HCM who achieved a high exercise capacity, expressed as METs = 7, showed excellent mid- to long-term outcomes. LVOTO at rest and electrocardiographic changes during maximal stress exercise were associated with an increased number of events during follow-up.

Introducción: Los pacientes con miocardiopatía hipertrófica (MCH) presentan con frecuencia síntomas confusos y equívocos. En estas instancias, las pruebas de esfuerzo pueden ayudar a la estratificación de riesgo de eventos futuros. Objetivo: Evaluar el valor pronóstico de las diferentes variables obtenidas mediante el eco estrés con ejercicio (EEE) en pacientes con diagnóstico de MCH. Material y métodos: Estudio retrospectivo y observacional. Se evaluaron 110 pacientes mediante EEE, los cuales se dividieron según el grado de obstrucción a nivel del tracto de salida del ventrículo izquierdo (OTSVI) en: 1) OTSVI persistente (gradiente máximo instantáneo obtenido en reposo mediante Doppler continuo = 30 mm Hg), 2) OTSVI latente (gradiente = 50 mm Hg ante el ejercicio) y 3) sin OTSVI. La mediana de seguimiento fue de 2,7 años. Se definió punto final primario a la ocurrencia de muerte, muerte súbita, taquicardia ven-tricular sostenida o internación por insuficiencia cardíaca. Resultados: El 19,1% de los pacientes presentaron OTSVI persistente, el 31,8% OTSVI latente y el 49,1% no presentaban OTSVI. La función ventricular, los espesores parietales y los diámetros fueron similares para los tres grupos. Las variables de mal pronóstico fueron significativamente mayores para la OTSVI persistente. El grupo con OTSVI latente desarrolló más síntomas, alteraciones electrocardiográficas e insuficiencia mitral posejercicio que el grupo sin OTSVI, aunque no se asoció con un número mayor de eventos. Las variables que se asociaron con más eventos en el seguimiento fueron la presencia de gradiente = 30 mm Hg en reposo (p = 0,07), alteraciones electrocardiográficas durante la prueba (p = 0,020) y los MET en su relación inversa (p = 0,07). Conclusiones: Los pacientes con MCH que alcanzaron una alta capacidad de ejercicio, expresada como MET = 7, presentaron excelentes resultados a mediano-largo plazo. La OTSVI en reposo y los cambios del electrocardiograma durante el esfuerzo máximo se asociaron con más eventos en el seguimiento.

Doppler tisular y estratificación del riesgo en la miocardiopatía hipertrófica / Tissue Doppler and Risk Stratification in Hypertrophic Cardiomyopathy

Introducción El Doppler pulsado tisular ha demostrado beneficio en la detección temprana de la miocardiopatía hipertrófica y en el diagnóstico diferencial de esta con otras causas secundarias de hipertrofia. Objetivo Determinar el valor pronóstico de las velocidades miocárdicas sistólicas tisulares preservadas en pacientes con diagnóstico de miocardiopatía hipertrófica. Material y métodos Se incluyeron 146 pacientes con diagnóstico de miocardiopatía hipertrófica, los cuales fueron evaluados en forma prospectiva mediante un estudio de ecocardiograma Doppler. Se obtuvieron las velocidades sistólicas tisulares del promedio de las velocidades septales y laterales; se compararon los pacientes con velocidades miocárdicas sistólicas tisulares preservadas (Sa = 8 cm/seg; cuartil superior) con los que presentaban velocidades disminuidas. Se definió como punto final primario a la presencia de muerte súbita, accidente cerebrovascular, insuficiencia cardíaca o internación de causa cardiovascular en el seguimiento. Resultados El 29% (n = 43) presentó velocidades miocárdicas sistólicas tisulares preservadas en las imágenes del Doppler tisular, con más frecuencia de varones (76,7% vs. 53,4%; p = 0,009) y sin diferencias en la edad. Los diámetros ventriculares y los espesores fueron similares, en tanto que el área auricular fue significativamente menor (23,7 ± 6,7 cm² vs. 28,8 ± 8 cm²; p < 0,001). En el seguimiento (mediana de 2,7 años), el número de eventos aumentó significativamente a medida que disminuyeron las velocidades sistólicas en el Doppler pulsado tisular. Ningún paciente del grupo velocidades miocárdicas sistólicas tisulares preservadas presentó el punto final combinado, con diferencias significativas con respecto al grupo control (0% vs. 21,6%; p = 0,001) y un valor predictivo negativo del 100%. Conclusiones En nuestra población de pacientes portadores de miocardiopatía hipertrófica, la presencia de velocidades miocárdicas sistólicas tisulares preservadas en el Doppler pulsado tisular permitió identificar a una subpoblación de pacientes de riesgo bajo, con un escaso número de eventos en el seguimiento, con un valor predictivo negativo elevado.

Usefulness of Tissue Doppler Imaging to Identify Low Risk Patients with Diagnosis of Hypertrophic Cardiomyopathy Background Pulsed tissue Doppler imaging is a useful tool for the early detection of hypertrophic cardiomyopathy and the differential diagnosis of this disease from other secondary causes of hypertrophy. Objective The aim of the study was to determine the prognostic significance of preserved systolic tissue velocities in patients with diagnosis of hypertrophic cardiomyopathy. Methods One hundred and forty six patients with diagnosis of hypertrophic cardiomyopathy were prospectively included by means of a Doppler echocardiography study. Systolic tissue velocities were obtained from the averaged septal and lateral velocities. Patients with preserved systolic tissue velocities (Sa = 8 cm/s; upper quartile) were compared with those presenting decreased velocities. The primary endpoint was defined as the presence of sudden death, stroke, heart failure, or hospitalization for cardiovascular causes at follow up. Results Twenty nine percent of patients (n = 43) presented preserved systolic tissue velocities in the tissue Doppler images, mostly in men (76.7% vs. 53.4%, p = 0.009) and with no age differences. Ventricular diameter and thickness were similar between the two groups while the atrial area was significantly lower (23.7 ± 6.7 vs. 28.8 ± 8, p <0.01). At follow up (median of 2.7 years), the number of events increased significantly as systolic pulsed tissue Doppler velocities decreased. No patient from the group with preserved systolic tissue velocities presented the combined endpoint, with significant differences with respect to the control group (0% vs. 21.6%, p = 0.001), and a negative predictive value of 100%. Conclusions In our population with hypertrophic cardiomyopathy, the presence of preserved systolic tissue velocities in pulsed tissue Doppler imaging identified low-risk patients with a very low number of events at follow up and high negative predictive value.

Miocardiopatía no compactada: Estratificación de riesgo de muerte súbita para indicación de cardiodesfibrilador automático implantable / Non-Compaction Cardiomyopathy: Risk Stratification of Sudden Death for Automatic Cardioverter Defibrillator Implantation

Introducción La miocardiopatía no compactada es una entidad rara. Su historia natural incluye insuficiencia cardíaca, eventos tromboembólicos, arritmias y muerte súbita (MS). En ausencia de datos de estudios aleatorizados o registros, las Guías ACC/AHA/HRS 2008 recomiendan el cardiodesfibrilador automático implantable (CDAI) en todos los pacientes con miocardiopatía no compactada para reducir el riesgo de muerte súbita. Objetivo Describir la evolución de pacientes con miocardiopatía no compactada de acuerdo con criterios seleccionados de estratificación de riesgo de muerte súbita para decidir el implante de un CDAI. Material y métodos Se analizaron 80 pacientes. El diagnóstico se estableció mediante criterios ecocardiográficos y de resonancia magnética nuclear cardíaca. Los criterios para el implante de un CDAI como prevención secundaria incluyeron muerte súbita y taquicardia ventricular sostenida (TVS); como prevención primaria comprendieron fracción de eyección del ventrículo izquierdo (FEVI) < 30% o ≥ 2 factores de riesgo (antecedentes familiares de muerte súbita [AFMS], síncope y TV no sostenida). Resultados Grupo CDAI (n = 26) para prevención secundaria (n = 3): 3 pacientes sufrieron muerte súbita (2 TVS). Grupo CDAI para prevención primaria (n = 23): 10 pacientes tuvieron FEVI < 30%, 1 FEVI < 30% + AFMS, 1 FEVI < 30% + síncope, 5 FEVI < 30% + TV no sostenida, 3 TV no sostenida + síncope, 2 TV no sostenida + AFMS y 1 TVS en estudio electrofisiológico. El seguimiento fue de una mediana de 16,61 meses. Dos pacientes fueron sometidos a un trasplante cardíaco, 3 recibieron choques apropiados y 4 pacientes, choques inapropiados. Grupo sin CDAI (n = 54): 4 pacientes tuvieron síncope y 4 TV no sostenida. El seguimiento fue de una mediana de 12,15 meses. Dos pacientes fallecieron por insuficiencia cardíaca y 3 fueron sometidos a un trasplante cardíaco. Conclusiones El 32,5% de los pacientes con miocardiopatía no compactada recibieron un CDAI, el 88,5% por prevención primaria; el 11,5% recibieron choques apropiados. No hubo muerte súbita en pacientes sin CDAI; la muerte sobrevino por progresión de la insuficiencia cardíaca. Este registro sugiere que los pacientes con miocardiopatía no compactada podrían estratificarse para seleccionar a aquellos que tienen mayor riesgo de muerte súbita y podrían beneficiarse con el implante de un CDAI.

Background Non-compaction cardiomyopathy is a rare disease. The natural history of this condition includes heart failure, thromboembolic events, arrhythmias and sudden death (SD). In the absence of information from randomized studies or registries, the 2008 ACC/AHA/HRS guidelines recommended cardioverter defibrillator implantation in all patients with non-compaction cardiomyopathy to reduce the risk of sudden death. Objective To describe the outcomes of patients with non-compaction cardiomyopathy according to the selection criteria used for risk stratification of sudden death for automatic implantable cardioverter defibrillator therapy. Material and Methods A total of 80 patients were analyzed. The diagnosis was made using criteria from echocardiography and cardiac magnetic resonance imaging. The criteria used for implantation of an ICD were sudden death and sustained ventricular tachycardia (SVT) for secondary prevention, and left ventricular ejection fraction (LVEF) <30% or ≥2 risk factors (family history of sudden death [FHSD], syncope and non-sustained VT). Results Group ICD (n=26) for secondary prevention (n=3): 3 patients presented sudden death (2 SVT). Group ICD for primary prevention (n=23): 10 patients had LVEF <30%, 1 LVEF <30% + FHSD, 1 LVEF <30% + syncope, 5 LVEF <30% + nonsustained VT, 3 non-sustained VT + syncope, 2 non-sustained + FMSD and 1 VT in the electrophysiologic study. Median follow-up was 16.61 months. Two patients underwent heart transplantation, 3 received appropriate ICD shocks, while ICD shocks were inappropriate in 4 patients. Group without ICD (n=54): 4 patients presented syncope and 4 non-sustained VT. Median follow-up was 12.15 months. Two patients died due to heart failure and 3 underwent cardiac transplantation. Conclusions ICD was implanted in 32.5% of patients with non-compaction cardiomyopathy, 88.5% for primary prevention; appropriate shocks were received in 11.5% of patients. None of the patients who did not receive an ICD presented sudden death; these patients died due to heart failure progression. This registry suggests that patients with non-compaction cardiomyopathy might be stratified to select those at a higher risk of sudden death who might beneficiate from ICD therapy.