Eduard Claver

Myocardial infiltration in Gaucher's disease detected by cardiac MRI☆

fraction 66%, non-dilated left ventricle (EDV 57 ml/m 2 )n or hypertrophied (mass 53 g/m 2 ) with ejection fraction 74%. Mitral valve leaflets were thickened with opening restriction motion. Late gadolinium enhancement (LGE) sequences showed patchy intramyocardial hyperenhancement with multiple foci in the basal and middle LV inferior and inferolateral segments, consistent with interstitial infiltrative fibrosis induced by the characteristic scattered foci of sphingolipids (glucosylceramide). At the light of these results, imiglucerase dose has been doubled, attempting to minimize heart infiltration. Endomyocardial biopsy has so far been the preferred approach to confirm the deposit of glucosylceramide in the myocardium. To the best of our knowledge no image of CMR showing LGE in the heart in GD has been previously published. In Fabry–Anderson disease, a pathological accumulation of glycosphingolipids occurs also in the myocardium, and LGE has been observed in the basal lateral segments of the LV [4]. In our case LGE is localized in both inferior and inferolateral segments. Two aspects that could be clue in the differential diagnosis between these entities are the patchy appearance of LGE (in Fabry–Anderson disease is more homogeneous) and the concomitant infiltration and gadolinium retention on LGE sequences of both liver and spleen in GD. Due to the lack of histological correlation it is not possible to ascertain if LGE represents areas of sphingolipid infiltration with reactive myocardial fibrosis. As in other storage diseases, it can be postulated that lipid infiltration could be the trigger for a deleterious pathway activation that would lead to varying degrees of fibrosis, being able to detect it by means of

Clinical Predictors of Left Main Coronary Artery Disease in High-Risk Patients With a First Episode of Non-ST-Segment Elevation Acute Coronary Syndrome

INTRODUCTION AND OBJECTIVES Risk stratification in non-ST-elevation acute coronary syndrome makes use of clinical variables that can identify patients at an increased risk of complications. Our objective was to identify clinical variables that predict significant stenosis (i.e., >50%) of the left main coronary artery in high-risk patients who have had a first episode of non-ST-elevation acute coronary syndrome but who do not have a history of coronary artery disease. METHODS The study included 102 high-risk patients with no history of coronary artery disease who were admitted because of non-ST-elevation acute coronary syndrome. All underwent coronary angiography. Patients were divided into two groups: those with significant left main coronary artery stenosis (n=14) and those without (n=88). RESULTS Univariate analysis showed that the variables significantly associated with left main coronary artery stenosis were age >65 years (57.1% vs 15.9%, P=.002), diabetes mellitus (71.4% vs 33.0%, P=.006), chronic renal failure (28.6% vs 5.7%, P=.019), left heart failure (71.4% vs 6.8%, P< .0001), cardiogenic shock (21.4% vs 1.1%, P=.008), and a low left ventricular ejection fraction at admission (49.9% [14.7%] vs 58.8% [9.9%], P=.044). In the multivariate analysis, the only significant independent predictor of left main coronary artery disease was left heart failure. CONCLUSIONS The presence of left heart failure at initial assessment of high-risk patients with non-ST-elevation acute coronary syndrome but without a history of coronary artery disease could be a useful predictor of significant left main coronary artery disease.

Variables clínicas predictoras de enfermedad del tronco común en pacientes de alto riesgo con un primer episodio de síndrome coronario agudo sin elevación del segmento ST

Introduccion y objetivos En la estratificacion del syndrome coronario agudo disponemos de variables clinicas para identificar a los pacientes con alto riesgo de presentar complicaciones. Analizamos si los pacientes de alto riesgo, sin antecedentes de cardiopatia isquemica, que presentan un primer episodio de sindrome coronario agudo sin elevacion del segmento ST presentan variables clinicas predictoras de estenosis significativa (> 50%) del tronco comun. Metodos Se analizo a 102 pacientes de alto riesgo sin antecedentes de cardiopatia isquemica con sindrome coronario agudo sin elevacion del segmento ST, a los que se les practico coronariografia. Establecemos 2 grupos: con estenosis significativa del tronco comun (n = 14) y el resto (n = 88). Resultados Variables relacionadas con la estenosis del tronco comun en el analisis univariado: edad > 65 anos (el 57,1 frente al 15,9%; p = 0,002), diabetes mellitus (el 71,4 frente al 33,0%; p = 0,006), insuficiencia renal cronica (el 28,6 frente al 5,7%; p = 0,019), insuficiencia cardiaca izquierda (el 71,4 frente al 6,8%; p Conclusiones La presencia de insuficiencia cardiaca izquierda en la evaluacion inicial del sindrome coronario agudo sin elevacion del ST, en pacientes de alto riesgo sin antecedentes de cardiopatia isquemica, puede ser un predictor util de enfermedad del tronco comun.

Double-chambered left ventricle: coronary embolism as the first presentation of an extremely unusual cardiac anomaly.

Materials and methods A 27-year-old man presented with chest pain, mild hypertension (140/90) and no personal or family history of coronary artery disease. Of remark, 1 year before admission, he had suffered an episode of expression aphasia and dizziness that lasted 20 min and disappeared spontaneously without further sequelae. A cerebral computed tomographic (CT) scan showed no acute lesions and he had been discharged with the diagnosis of migraine with aura. He had been asymptomatic since

Incomplete Shone's complex in the sixth decade of life: echo and cardiac magnetic resonance imaging assessment.

A 51-year-old woman was referred to the Cardiology outpatient clinic for a murmur. She was asympthomatic. Transthoracic echocardiogram showed a parachute mitral valve ( Panel s 1A – C and 2A ), with anomalous and elongated chordae converging into a major papillary muscle (posteromedial) with two heads ( Panel s 1B and 2B , see Supplementary material online, Videos S 1 –S 3 ). Transmitral gradient was normal, and non-significant mitral …

Coronary Embolization in Hypertrophic Cardiomyopathy With Left Ventricular Apical Aneurysm. Does Follow-Up With Cardiac Magnetic Resonance Have a Role?

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Atypical cardiac magnetic resonance findings in a case of suspected endomyocardial fibrosis

Endomyocardial fibrosis (EMF) is the leading cause of restrictive cardiomyopathy worldwide. Cardiac magnetic resonance (CMR) with assessment of myocardial late gadolinium enhancement (LGE) allows the diagnosis of the disease by means of the characterization of the fibrous tissue deposited on the endocardium. We sought to discuss the CMR findings in a young man from Equatorial Guinea with a history of exertional dyspnoea and ascites. On chest radiography ( Panel A ), an enlarged cardiac silhouette is …