Multimodality imaging of the anomalous origin of the right pulmonary artery from the ascending aorta in an adult (hemitruncus arteriosus).

Anomalous origin of the pulmonary artery branches is a rare phenomenon. We describe a case of an adult with anomalous origin of the right pulmonary artery (hemitruncus arteriosus) associated with patent ductus arteriosus. Non-invasive imaging studies played an important role in the diagnosis and follow-up. Angiography allowed to determine the severity of pulmonary hypertension. He underwent surgical closure of patent ductus arteriosus, redirection of right pulmonary artery and atrioseptostomy with decrease of the pulmonary pressure in the follow-up. A high index of clinical suspicion of this entity is required in adults with heart failure, recurrent hemoptysis and pulmonary hypertension, because it could go unnoticed.

Elastographic ultrasound: an additional image tool in Sjögren's syndrome.

AIM: To evaluate the stiffness of parotid and submandibular glands using elastography ultrasound and to correlate it with B-mode ultrasonographical, clinical and serological features, salivary profibrotic and inflammatory chemokines, and salivary gland fibrosis. METHODS: We performed B-mode and elastography ultrasound of major salivary glands of 26 patients with primary Sjögren's syndrome. We registered the shear wave velocity (SWV) and correlated it with the morphologic ultrasonographic changes assessed by the Hocevar scale. We assessed the European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI), EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), non-stimulated whole salivary flow rate (NSWSF), C3 and C4 levels, anti-Ro/La antibodies, salivary inflammatory (C-X-C motif ligand 13 [CXCL13], CXCL10, CXCL8, C-C motif ligand 2 [CCL2], interleukin 10 [IL-10] and IL-6) and pro-fibrotic (CXCL14, CCL28, tumor necrosis factor-related apoptosis-inducing ligand and transforming growth factor ß) chemokines and cytokines and evaluated the presence of fibrosis in the minor salivary gland. RESULTS: Ninety-two percent of patients were women; mean age was 51.1 ± 11 years; median disease duration was 6.1 years; 92.3% had oral symptoms and 26.9% fibrosis. The median B-mode score was 22.2 points and the median SWV 2.5 m/s (τ = 0.53, P = 0.001). The SWV correlated with the NSWSF (τ = -0.53, P = 0.001), ESSDAI (τ = 0.31, P = 0.03), glandular ESDDAI domain (τ = 0.36, P = 0.02), C4 levels (τ = -0.32, P = 0.04), salivary CXCL13 (τ = 0.29, P = 0.03) and CXCL10 (τ = 0.30, P = 0.003), but not with age and fibrosis. CONCLUSION: WV correlated with the B-mode ultrasound score, systemic and glandular activity and in a large degree with CXCL10, an inflammatory chemokine, but not with fibrosis. An increased SWV might represent chronic glandular inflammation rather than fibrotic changes in these patients.

Asymptomatic Coronary Artery Calcifications in Men with Systemic Lupus Erythematosus.

OBJECTIVE: To determine whether the prevalence and extent of asymptomatic coronary artery atherosclerosis are increased in men with systemic lupus erythematosus (SLE) compared with age- and sex-matched controls, and to define the associated risk factors. METHODS: Ninety-five patients with SLE (mean ± SD age, 34.7 ± 10.1 yrs) and 100 control subjects (age 34.8 ± 9.7 yrs) with no history of coronary artery disease were screened for coronary artery calcification using multidetector computed tomography. The extent of calcification was measured using the Agatston score. The frequency of risk factors for calcification was compared between patients and controls, and the relationship between clinical and immunological characteristics and the presence of coronary artery calcification was investigated. RESULTS: Coronary artery calcification was more frequent in patients than controls [18% vs 7%, respectively (OR 2.89, 95% CI 1.07-8.65)]. These factors were independently associated with the presence of calcifications: age (OR 1.12, 95% CI 1.04-1.20), SLE diagnosis (OR 3.38, 95% CI 1.07-10.64), diabetes mellitus (OR 6.88, 95% CI 1.50-31.62), Framingham risk score (OR 1.12, 95% CI 1.00-1.23), and glomerular filtration rate (OR 0.98, 95% CI 0.96-1.00). Among patients with SLE, coronary artery calcifications were observed starting at age 32 years, within 2.3 years of diagnosis. Increasing age (OR 1.18, 95% CI 1.06-1.31), Systemic Lupus International Collaborating Clinics score (OR 2.85, 95% CI 1.21-6.73), and cumulative dose of prednisone (OR 1.04, 95% CI 1.01-1.08) were independent risk factors. CONCLUSION: Men with SLE are at an increased risk of coronary artery calcifications than age- and sex-matched controls. Among patients with SLE, the increased risk is associated to older age, increasing chronic damage, and cumulative dose of corticosteroids.

Normal function of a 43-year-old Braunwald Cutter heart valve.

We present the case of a 72-year-old woman diagnosed with rheumatic fever at the age of 6. In 1972, she was diagnosed with mitral valve insufficiency and mitral valve stenosis, then in 1974, a decision was made to perform mitral valve replacement surgery with a 32-mm Braunwald-Cutter ball cage prosthesis. An echocardiogram performed in 2014 revealed normal biventricular systolic function, mechanical prosthesis in mitral position with maximum speed of 1.9 m/s, maximum gradient of 15 mmHg, mean gradient of 6 mmHg, severe tricuspid valve insufficiency, inferior vena cava measuring 15 mm with more than 50% collapse and pulmonary artery systolic pressure of 40 mmHg. We report the use of the 32-mm Braunwald-Cutter ball cage prosthesis with the longest longevity that remains functional after more than 43 years of implantation.

Evaluación de las características de la disección aórtica en la población mexicana mediante angiotomografía computarizada / Evaluation of the characteristics of aortic dissection by computed angio-tomography in the Mexican population

Resumen Objetivo: Revisión y análisis de la disección aórtica (DA) en la población mexicana. Método: Revisión retrospectiva de 434 expedientes electrónicos de pacientes con angiotomografía de aorta entre noviembre de 2014 y octubre de 2015. Se obtuvo una muestra de 32 pacientes con diagnóstico de DA de primera vez. Se realizó un análisis de las DA según género, grupo etario, clasificación de Stanford/De Bakey y mortalidad a 6 meses del diagnóstico. Se realizó análisis de significación estadística mediante la Chi-cuadrada para las variables independientes de género, síndrome de Marfan, hipertensión arterial sistémica y enfermedad ateromatosa calcificada en asociación con subtipos, sitios de reentrada y datos de hipoperfusión. Resultados: El 65.6% de los pacientes fueron masculinos, con un promedio de edad de 54.5 años, y el 34.4% fueron femeninos, con un promedio de edad de 42.5 años. El subtipo B/3 fue el más frecuentemente diagnosticado. La tasa de mortalidad a 6 meses fue del 18.7%. Se halló asociación significativa con p marginal en pacientes con síndrome de Marfan y subtipos de DA según Stanford (p = 0.0506), así como asociación significativa en pacientes con aneurisma de aorta abdominal y subtipos de DA según Stanford (p = 0.047104). Conclusiones: La DA es una emergencia en la cual el diagnóstico por imagen y el manejo oportuno son fundamentales para mejorar el pronóstico. En nuestra muestra encontramos asociación significativa de pacientes con antecedente de síndrome de Marfan y aneurisma aórtico abdominal con disecciones según la categoría de Stanford. El resto de las variables independientes no mostraron asociación significativa, en probable relación con el tamaño de la muestra.

Abstract Objective: To review aortic dissection (AD) in the Mexican population. Method: A retrospective study was conducted using 434 medical records of patients with aortic angio-tomography between November 2014 and October 2015. A sample was obtained of 32 patients with a first time diagnosis of AD. An analysis was performed of the dissections according to gender, age group, Stanford/De Bakey classification, and mortality rate 6 months after diagnosis. Statistical analysis was performed by obtaining the Chi squared index for the independent variables of gender, Marfan syndrome, systemic arterial hypertension, as well as calcified atheromatous disease in association with dissection subtypes, re-entry sites, and hypo-perfusion signs. Results: The patients included 65.6% males with a mean age of 54.5 years, and 34.4% females with mean age of 42.5 years. The most common dissection subtype was B/3. Mortality rate at 6 months was 18.7%. There was a significant association, with a marginal P in patients with Marfan syndrome and Stanford subtypes of AD (P = .0506). There was a significant association in patients with abdominal aortic aneurysm, when compared with Stanford subtypes of AD (P = .047104). Conclusions: AD is an emergency in which diagnosis and timely management are essential to improve prognosis. In the sample presented here, a significant association was found in patients with a history of Marfan syndrome and abdominal aneurysms with dissections according to the Stanford classification. The rest of the independent variables did not show any significant association, probably related to the size of the sample.

Differential expression of osteopontin, and osteoprotegerin mRNA in epicardial adipose tissue between patients with severe coronary artery disease and aortic valvular stenosis: association with HDL subclasses.

BACKGROUND: Previous studies suggest a relationship of the epicardial adipose tissue (EAT) with progression and calcification of the atherosclerotic plaque; however, it is unknown if this tissue expresses genes that may participate on these processes and if the expression of these genes is regulated by high-density lipoprotein (HDL) subclasses. METHODS: To explore this possibility, we determined the mRNA expression by qPCR of a pro-calcifying gene (osteopontin (OPN)), and two anti-calcifying genes (osteoprotegerin (OPG) and osteonectin (ON)), in biopsies of EAT obtained from 15 patients with coronary artery disease (CAD) determined by angiography, and 15 patients with diagnostic of aortic valve stenosis but without CAD as control group. We determined the distribution and composition of HDL subclasses by electrophoresis and their statistical relationship with the gene expression in EAT. RESULTS: EAT from CAD patients showed a higher expression level of OPN and OPG than control group, whereas ON expression was similar between groups. Large HDL subclasses were cholesterol-poor in CAD patients as estimated by the cholesterol-to-phospholipid ratio. A linear regression model showed an independent association of OPN expression with HDL3a-cholesterol, and OPG expression with the relative proportion of HDL3b protein. Logistic analysis determined that OPN expression was positively associated with the presence of atherosclerotic plaque CONCLUSION: OPN, ON, and OPG genes are transcribed in EAT; to the exception of ON, the level of expression was different in CAD patients and control group, and correlated with some HDL subclasses, suggesting a new role of these lipoproteins.

[Intrapancreatic lipoma: a rare mesenquimatous tumor with an uncertain physiopathogenesis]. / Lipoma intrapancreático: un tumor mesenquimatoso poco frecuente de fisiopatología incierta.

Intrapancreatic lipoma is a mesenquimal tumor that currently is still considered a strange entity. There are numerous hypotheses about its pathophysiology; however, none has been clarified. Diagnosis is usually incidental, and computed tomography has been performed for other indications, with high sensibility and specificity, making histopathology confirmation unnecessary, hence suggesting image-follow up solely.