There are few radiological descriptions of amyloid goiter, basically in adult patients of oriental origin. We present a ten-year-old boy with Still's disease and secondary thyroid amyloidosis, describing the US, CT and MR findings.
Objectives to evaluate the association between late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (cMRI) and prognosis in patients with non-ischemic dilated cardiomyopathy (NIDM). Background Risk stratification in NIDM needs to be improved. Methods A total of 210 patients with NIDM and cMRI from 2005 to 2018 were included in our population. Outcomes were retrospectively assessed by medical records. The pattern of LGE was classified as midwall, subepicardial, or both patterns. Primary endpoint was sudden cardiac death (SCD) and aborted SCD. Secondary endpoints were global mortality and a composite endpoint of cardiovascular mortality and heart failure hospitalization. Demographic and clinical parameters were also evaluated. Patients with LGE (LGE+) were more likely to be male (80,6% vs 66,7%, p=0,03). No significant differences were observed between LGE+ and LGE− patients in comorbidities, NYHA class, left ventricular ejection fraction (LVEF), left bundle branch block or neurohormonal treatment. Results Of 210 patients (71,4% men, median age 59,8 years) with a median follow up of 5,6 years (3,24–8,15), 72 patients (34,3%) had non ischemic LGE (LGE+). Mean LVEF was 34%. SCD or aborted SCD occurred in 11 patients (5,2%). 6 patients (9,5%) with LGE+ reached the primary endpoint vs 5 (4,07%) of LGE− patients (p=0,19). The adjusted OR for the presence of LGE in the composite endpoint (cardiovascular mortality and heart failure hospitalization) was 2,45, confidence interval (CI): 1,16–5,17, (p=0,02). LGE presence was not associated with global mortality. The subepicardial pattern of LGE was associated with SCD and aborted SCD: 3 out of 11 patients (27, 1%) with subepicardial pattern of LGE suffered from SCD or aborted SCD (p=0,02). Conclusions In our cohort of 210 patients with NIDM, LGE presence was not associated with SCD and aborted SCD, probably because of low event rate in a relatively small population. However, LGE presence was associated with the composite endpoint of cardiovascular mortality and heart failure hospitalization. The subepicardial pattern of LGE identified patients at high risk of SCD and aborted SCD.
Objectives to evaluate the relationship between left atrial (LA) peak strain and left ventricular (LV) diastolic function and determine whether LA strain could be used to detect elevated filling pressures in a population of patients with preserved LV ejection fraction (LVpEF). Background the assessment of diastolic function is complex and multiparameter. 2016 ASE and EACVI algorithm has simplified this approach, but there are still patients with discrepant values leading to an indeterminate diagnosis, specially in patients with preserved ejection fraction. Methods we prospectively included 129 patients in our cohort. Inclusion criteria were LVEF > 50% and sinus rhythm. Patients with atrial fibrillation, significant mitral valvulopathy or poor quality imaging were excluded. Two-dimensional speckle tracking was used to measure peak LA strain in all LA segments (using apical 4 chamber, 2 chamber and 3 chamber views). We evaluated the association of LA global peak strain and LA 4 chamber peak strain with the different categories of diastolic function according to the 2016 ASE and EACVI algorithm. Results Both LA global peak strain and 4 chamber LA peak strain showed a progressive decrease with worsening diastolic dysfunction (DD) severity (Table 1). A global LA peak strain cutoff value of 28,35% was very accurate to differentiate normal from elevated filling pressures (normal function or grade 1 DD from grades 2 and 3 DD). Diagnostic accuracy: 80.20%; area under the curve: 0,80 (0,72-0,88); A significant group of patients (21,1%) could not be categorized using 2016 algorithm. Conclusions Both LA global peak strain and 4 chamber LA peak strain demonstrated a progressive decrease with worsening DD severity. Global LA peak strain value of 28,35% was an accurate cutoff to differenciate patients with normal vs elevated filling pressures. LA strain values and diastolic function Total LA global peak STRAIN LA 4C STRAIN N (%) p-value p-value Total 129 30.90 %(11.77) 30.43 (12.36) Diastole <0.0001 <0.0001 Normal function 36 (28.13) 39.72% (9.59) 39.98 (10.59) Indeterminate Diastolic Function 23 (17.97) 31.07% (7.97) 30.19 (7.50) Indeterminate DD 4 (3.13) 30.68 % (6.72) 29.75 (7.31) grade 1 DD 22 (17.19) 32.69 % (12.54) 32.98 (14.46) grade 2 DD 36 (28.13) 23.90 % (9.32) 23.53 (9.49) grade 3 DD 7 (5.47) 15.43 % (5.72) 15.85 (7.32)
Introduction Due to the complexity of congenital heart disease and limitations of transthorathic echocardiogram (TTE), especially in adult patients, it is not unusual to need other image techniques to assess cardiac anatomy and function. The most common primary anomaly of tricuspid valve (TV) is Ebstein anomaly, but there are other much rarer primary anomalies of this valve consisting in prolapse, cord retraction.... without downward displacement of the leaflet, generally causing tricuspid regurgitation (TR) that can be severe and sometimes intervention is needed, preferably reparation. Due to anatomical issues, it is difficult to assess anatomy of TV in TTE, so sometimes 3D-TTE must be performed to clarify the mechanism and to measure orifice, but when transthoracic view is not enough, 3D transoesophageal echocardiogram (TOE) can be useful for this purpose. Case We report the case of a 15-year-old boy that was referred to our clinic because of shortness of breath and a systolic tricuspid murmur. TTE was performed and an image compatible with tricuspid valve prolapse with no apical displacement of any leaflets (Figure, A) causing severe TR (Figure, B) was noticed, as well as severely dilated right chambers, with good ejection fraction of both ventricles. It was not clear the mechanism so 2D TOE was done, showing a prolapse of a leaflet (Figure, C) causing severe TR (Figure, D). The mechanism was finally clarified by 3D TOE (figure E). This was a prolapse of lateral portion of posterior leaflet (asterisk) with restrictive movement of anterior (triangle) and septal (arrow) ones, causing a huge coaptation defect in systole leading to a very severe tricuspid insufficiency with signs of volume overload of right ventricle. There was no atrial septal defect and pulmonary drainage anomalies were ruled out by cardiac magnetic resonance. Patient was referred to surgery due to symptoms and great dilatation of right chambers. Conclusión: Due to anatomical complexity and limitations of echography, cross and multimodality cardiac imaging is usually needed in assessing congenital heart disease. Apart from Ebstein anomaly, other congenital entities of tricuspid valve such as prolapse and/or retraction can lead to severe tricuspid regurgitation. Due to limitations of 2D TTE in assessing tricuspid valve anatomy, 3D TTE has to be performed, but if it is not enough, 3D TOE can be an option to evaluate mechanism and directly see the orifice of regurgitation in congenital disease of tricuspid valve. Abstract P879 Figure
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