Case Report: A Rare Case of a Ventricular Perivascular Epithelioid Cell Tumor With Histologic Characteristics That Resembled a Primary Cardiac Rhabdomyoma.

We present the case of a young male patient with an initial diagnosis of a rhabdomyoma that was surgically treated at a different hospital when he was 17. After a 2-year disease-free period, the patient presented another intra-cardiac mass. He refused surgical treatment and died 5 years later. Post-mortem immunochemistry studies of both tumors led to the diagnosis of a primary malignant cardiac PEComa with histopathologic characteristics that resembled a rhabdomyoma with abundant "spider cells."

Cytidine-5'-Diphosphocholine Protects the Liver From Ischemia/Reperfusion Injury Preserving Mitochondrial Function and Reducing Oxidative Stress.

Cytidine-5'-diphosphocholine (CDP-choline) participates as an intermediary in the synthesis of phosphatidylcholine, an essential component of cellular membranes. Citicoline treatment has shown beneficial effects in cerebral ischemia, but its potential to diminish reperfusion damage in liver has not been explored. In this work, we evaluated the hepatoprotective effect of citicoline and its possible association with inflammatory/oxidative stress and mitochondrial function because they are the main cellular features of reperfusion damage. Ischemia/reperfusion (I/R) in rat livers was performed with the Pringle's maneuver, clamping the 3 elements of the pedicle (hepatic artery, portal vein, and biliary tract) for 30 minutes and then removing the clamp to allow hepatic reperfusion for 60 minutes. The I/R + citicoline group received the compound before I/R. Liver injury was evaluated by measuring aspartate aminotransferase and alanine aminotransferase as well as lactic acid levels in serum; proinflammatory cytokines, proresolving lipid mediators, and nuclear factor kappa B content were determined as indicators of the inflammatory response. Antioxidant effects were evaluated by measuring markers of oxidative stress and antioxidant molecules. Oxygen consumption and the activities of the respiratory chain were used to monitor mitochondrial function. CDP-choline reduced aspartate aminotransferase (AST), alanine aminotransferase (ALT), as well as lactic acid levels in blood samples from reperfused rats. Diminution in tumor necrosis factor alpha (TNF-α) and increase in the proresolving lipid mediator resolvin D1 were also observed in the I/R+citicoline group, in comparison with the I/R group. Oxidative/nitroxidative stress in hepatic mitochondria concurred with deregulation of oxidative phosphorylation, which was associated with the loss of complex III and complex IV activities. In conclusion, CDP-choline attenuates liver damage caused by ischemia and reperfusion by reducing oxidative stress and maintaining mitochondrial function. Liver Transplantation XX XX-XX 2018 AASLD.

Echocardiographic and Histologic Correlations in Patients with Severe Aortic Stenosis: Influence of Overweight and Obesity.

BACKGROUND: Severe aortic stenosis (AS), leads to pathological left ventricular remodeling that may worsen with concomitant overweight and obesity (OW/O). METHODS: We aimed to prospectively analyze the impact of OW/O on ventricular remodeling in severe AS, by evaluating the percentage of intraendomyocardial fibrosis (PIEF) and the percentage of infiltrating intraendocardial lipid vacuoles (PIELV) and its relationship to global longitudinal strain (GLS) in patients with OW/O. RESULTS: 44 patients with severe AS were included, 13 non-obese (29%) and 31 OW/O (71%), all of them with left ventricular ejection fraction ≥ 55%. GLS was evaluated with 2D speckle tracking. During valve replacement, an endocardial biopsy was obtained, where PIEF and PIELV were analyzed. Patients with higher PIEF and PIELV had greater body mass index (p < 0.0001) and worse GLS (p < 0.0053). A GLS cut-off point < -14% had a sensitivity of 75%, and a specificity of 92.8% to detect important PIEF (AUC: 0.928, 95% confidence interval: 0.798-1.00). On multivariate analysis, OW/O and PIELV were independently associated to the PIEF, and OW/O and PIEF were independently associated to GLS. A high correlation between the amount of PIELV and PIEF were found. CONCLUSION: Patients with severe AS and OW/O have greater PIEF and PIELV, suggesting more pathological remodeling. GLS is useful to detect subclinical myocardial injury and is potentially useful for endomyocardial fibrosis detection. The presence of higher PIELF may be a trigger factor for the development of intraendomyocardial fibrosis.

Myocardial Rupture in Acute Myocardial Infarction: Mechanistic Explanation Based on the Ventricular Myocardial Band Hypothesis.

BACKGROUND: Torrent-Guasp explains the structure of the ventricular myocardium by means of a helical muscular band. Our primary purpose was to demonstrate the utility of echocardiography in human and porcine hearts in identifying the segments of the myocardial band. The second purpose was to evaluate the relation of the topographic distribution of the myocardial band with some post-myocardial infarction ruptures. METHODS: Five porcine and one human heart without cardiopathy were dissected and the ventricular myocardial segments were color-coded for illustration and reconstruction purposes. These segments were then correlated to the conventional echocardiographic images. Afterwards in three cases with post-myocardial infarction rupture, a correlation of the topographic location of the rupture with the distribution of the ventricular band was made. RESULTS: The human ventricular band does not show any differences from the porcine band, which confirms the similarities of the four segments; these segments could be identified by echocardiography. In three cases with myocardial rupture, a correlation of the intra-myocardial dissection with the distribution of the ventricular band was observed. CONCLUSIONS: Echocardiography is helpful in identifying the myocardial band segments as well as the correlation with the topographic distribution of some myocardial post-infarction ruptures.

Imagen histológica y angiocardiográfica de miocarditis aguda por dengue / Histologic and Angiographic Imaging of Acute Shock Dengue Myocarditis

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Reconstrucción del tracto de salida normal del ventrículo derecho en la operación de Ross / Normal right ventricle outflow tract reconstruction in the Ross operation

En la actualidad existen procedimientos quirúrgicos que requiren la intervención del tracto de salida normal del ventrículo derecho (TSVD) y su posterior reconstrucción. En este trabajo presentamos la antomía quirúrgica de la raíz normal de la arteria pulmonar y su reconstrucción en la operación de Ross en 13 pacientes operados desde febrero de 1992 hasta febrero de 1994. Se efectuó la excisión de la vávula pulmonar y, para mantener la continuidad del ventrículo derecho con la arteria pulmonar (VD-AP) se colocaron, en todos los casos, conducto de pericardio autólogo con una bioprótesis de pericardio bovino del Instituto Nacional de Cardiología (INC), que fueron elaborados durante la cirugía. El postoperatorio fue satisfactorio en todos y su evolución clínica hasta la fecha es adecuada. El seguimiento, con control ecocardiográfico de todos los conductos valvulados, no ha revelado gradientes transpulmonar ni regurgitación. Estamos convencidos de que es muy importante conocer la anatomía quirúrgica de la raíz pulmonar para evitar daño irreversible. Por otro lado, es de gran valor el conocimiento de las diferentes alternativas que hay para reconstruir el TSVD y su evolución a largo plazo.

Ausencia de lesiones características en la microcirculación de pacientes diabéticos no insulino-dependientes / Absence of characteristic microangiopathic lesions in the heart of patients with non insulin-dependent diabetes mellitus (NIDDM)

La existencia de una posible cardiomiopatía se ha vinculado con la mayor morbi-mortalidad cardiovascular, que presenta el paciente con diabetes mellitus (DM) y se ha propuesto que ésta puede ser secundaria a la afección de los microvasos cardíacos. Cincuenta y dos casos de pacientes fallecidos con historia de infarto del miocardio fueron divididos en 2 grupos: diabéticos y no diabéticos, subdivididos a su vez en normotensos e hipertensos. Los pacientes fueron de igual edad y sexo. El estudio se realizó en forma ciega. Los corazones fijados en formol al 10 por ciento se enstudiaron macro y microscópicamente. En los casos de pacientes con diabetes, la enfermedad coronaria aterosclerosa epicárdica fue más difusa. En los microvasos se observó un mayor engrosamiento de la membrana basal de los capilares con respecto a los no diabéticos (NS). A excepción de estos hallazgos, no se encontraron diferencias entre los cuatro grupos respecto a las lesiones vasculares, presencia de lesiones proliferativas ensoteliales, hipertrofia parenquimatosa o edema intersticial. El grupo de pacientes hipertensos se caracterizó por mayor fibrosis perivascular (NS). Nuestros resultados sugieren que la enfermedad aterosclerosa en más extensa en el pacientes diabético y la cardiomiopatía diabética, de estar presente, no parece estar relacionada con un mayor daño estructural de los microvasos