Isolated cardiac valve involvement in smoldering adult T-cell leukemia/lymphoma.

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive mature T-cell neoplasm caused by infection with the Human T-cell Lymphotropic Virus Type 1 (HTLV-1). Cardiac involvement in patients with ATLL is infrequent, and when it happens it is usually seen in aggressive ATLL subtypes. However, ATLL presenting as isolated cardiac valve involvement is extremely rare. To date, only three histologically proven cases of ATLL with isolated cardiac valve involvement have been reported. Herein, we describe a 61-year-old Peruvian man who presented heart failure symptoms secondary to progressive cardiac valve infiltration. The patient underwent mitral valve replacement with a mechanical prosthesis. Histopathological evaluation of the resected valve revealed leaflet thickening with a nodular appearance due to fibrous tissue containing atypical T-lymphocytes with Foxp3 expression, infiltrating all layers of the resected valve. Interestingly, tumor cells were distributed around an incidental venous malformation (i.e., cavernous hemangioma). Postoperative evaluation demonstrated positive serology for HTLV-1, and a diagnosis of ATLL was established. Postoperative positron emission tomography/computed tomography did not show lesions outside the heart and cell blood counts were within normal range with low level of circulating CD4+ CD25+ lymphoma cell counts (7%); therefore, patient's disease was considered as smoldering ATLL and a "watch and wait" strategy was pursued. Currently, the patient is alive with no progression of disease after 18 months from diagnosis. Isolated cardiac valve involvement by ATLL should be considered in the differential diagnosis of HTLV-1 carriers with progressive heart failure, even when systemic lymphoma involvement is absent or not apparent.

[Clinical profile and therapeutic strategies in patients with arrhythmogenic cardiomyopathy treated in a national reference institute]. / Perfil clínico y estrategias terapéuticas en pacientes con miocardiopatía arritmogénica tratados en un instituto de referencia nacional.

Objective: To determine the epidemiological, clinical, electrocardiographic, imaging characteristics and main therapeutic strategies performed in patients with arrhythmogenic cardiomyopathy treated in a national reference cardiovascular institute. Materials and methods: Observational, descriptive and retrospective study that attempts to identify the clinical characteristics, complementary tests and therapeutic strategies performed in patients with arrhythmogenic cardiomyopathy treated at the Instituto Nacional Cardiovascular - INCOR EsSalud in Lima, Peru. Results: Thirteen patients were found with arrhythmogenic cardiomyopathy. The median age at which the diagnosis was made was 38.2 years and 69.3% were male. The most frequent clinical manifestations were tachycardic palpitations (92.3%), presyncope (84.6%) and heart failure (69.2%). 23% of the patients suffered a cardiac arrest. All the patients presented at least one episode of ventricular tachycardia, 92.3% with complete left bundle branch block morphology and upper axis. 76.9% received an implantable cardioverter defibrillator (ICD), 15.3% underwent ablation and 15.3% received a heart transplant. 84.6% of the patients live to this day. Conclusions: Arrhythmogenic cardiomyopathy predominantly affected the young and male population. All the patients had a potentially fatal ventricular arrhythmia. Biventricular disease by echocardiography and cardiac magnetic resonance occurred in 69.2% and 100% of the cases, respectively. The therapeutic strategies used were antiarrhythmic medical treatment, placement of an ICD as secondary prevention, ablation, and heart transplantation. To date, 84.6% of patients survive.

Estratificación hemodinámica mediante ecocardiografía transtorácica en pacientes con infarto agudo de miocardio con segmento ST elevado / Hemodynamic stratification using transthoracic echocardiography in patients with ST segment elevation acute myocardial infarction

Objetivo: Evaluar la factibilidad de realizar una estratificación hemodinámica en pacientes con infarto agudo de miocardio con segmento ST elevado, mediante el uso de ecocardiografía transtorácica. Materiales y métodos: Estudio descriptivo, prospectivo y transversal que incorporó a 30 pacientes en ritmo sinusal con diagnóstico de infarto agudo de miocardio con ST elevado, ingresados a la unidad coronaria del Hospital Edgardo Rebagliati Martins. Se estimó: gasto cardíaco, presión de llenado de ventrículo izquierdo, resistencia vascular sistémica y resistencia vascular pulmonar. Resultados: La edad promedio fue 67 ± 10.6 años, predominio masculino (86.7%). La estimación del gasto cardiaco y la presión de llenado del ventrículo izquierdo fue posible en el 100% de los individuos. La resistencia vascular sistémica y resistencia vascular pulmonar pudieron ser estimadas en 29 (96.6%) y 28 (93.3%) pacientes, respectivamente. La estratificación hemodinámica fue: estadio I (normal): 18 pacientes (60%), estadio II (congestivo): 6 pacientes (20%), estadio III (hipovolemia, compromiso de VD): 5 pacientes (16.7%) y estadio IV (Choque): 1 paciente (3.3%). Conclusiones: Se concluye que es factible realizar la estratificación hemodinámica ecocardiográfica transtorácica en la población estudiada

Objective: To evaluate the feasibility of performing a hemodynamic stratification in patients with ST segment elevation acute myocardial infarction using transthoracic echocardiography. Materials and methods: A descriptive, prospective and cross-sectional study that included 30 patients in sinus rhythm with ST segment elevation acute myocardial infarction, admitted to the coronary care unit of Hospital Edgardo Rebagliati Martins. Cardiac output, left ventricle filling pressure, systemic vascular resistance and pulmonary vascular resistance were estimated. Results: The mean age was 67 ± 10.6 years old, with male predominance (86.7%). The estimation of cardiac output and left ventricle filling pressure was possible in 100% of the individuals. The systemic vascular resistance and pulmonary vascular resistance could be estimated in 29 (96.6%) and 28 (93.3%) patients, respectively. The hemodynamic stratification was as follows: stage I (normal): 18 patients (60%), stage II (congestive): 6 patients (20%), stage III (hypovolemia, RV infarction): 5 patients (16.7%) and stage IV (shock): 1 patient (3.3%). Conclusions: It is concluded that a transthoracic echocardiographic hemodynamic stratification may be performed in the studied population