Endocardial Fibroelastosis as a Cause of Sudden Unexpected Death

Letter, Els A. De; Piette, M

doi:10.1097/00000433-199912000-00009

Cited by 11 publications

(3 citation statements)

References 34 publications

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“…Thus, although primary EFE had been previously labeled as a separate form of cardiomyopathy, in 2006 the new classification of the American Heart Association does not consider it anymore as an isolate disease and includes it in the spectrum of DCM ( 22 ). Primary EFE in the original description is characterized by the presence of ventricular dilatation, diffuse fibrous endocardial thickening, upward displacement of the papillary muscles, and valve leaflets thickening ( 20 , 23 , 24 ). In terms of “outcome,” EFE is characterized by a very poor prognosis in pediatric patients ( 25 , 26 ).…”

Section: Discussionmentioning

confidence: 99%

Results of Late Gadolinium Enhancement in Children Affected by Dilated Cardiomyopathy

et al. 2017

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BackgroundLittle is known about the clinical value of late gadolinium enhancement (LGE), in children affected by dilated cardiomyopathy (DCM).Materials and methodsWe retrospectively evaluated 15 patients (8 ± 6 years, 6 males) with diagnosis of DCM who underwent cardiac magnetic resonance since 2014. All scans were performed with a 1.5 T system (Aera, Siemens). Study protocol included cine steady-state free precession sequences, followed by administration of 0.2 mmol/kg of gadolinium-based contrast agent. Inversion recovery Turbo Flash sequences, in the same position of cine images, were acquired 10–15 min after the injection of contrast agent, in order to assess the presence of LGE. The latter was considered positive with a signal intensity >6 SD from normal myocardial tissue. Indexed end-diastolic volume (EDVi) and end-systolic volume (ESVi), and left ventricle (LV) ejection fraction (EF) were calculated by using dedicated software on off-line workstation. Global longitudinal strain and diastolic function were evaluated by echocardiography. Clinical follow-up, including death, transplant, and listing for heart transplant [major adverse cardiac events (MACE)], were evaluated. Patients were divided into two different subgroups: negative (Group A) and positive (Group B) for presence of LGE. Statistical analysis was performed by using Mann–Whitney U test (p < 0.05 considered as statistically significant).ResultsSeven patients (47%) showed LGE. A global diffuse subendocardial pattern was evident in all patients presenting LGE (7/7, 100%). The following main LV indexes were observed in the two subgroups. Group A: EDVi = 96 ± 33 ml, ESVi = 56 ± 29 ml, LV EF = 45 ± 10%, global longitudinal strain = −16 ± 5%, E/e′ ratio = 10 ± 3, MACE = 1. Group B: EDVi = 130 ± 60 ml, ESVi = 89 ± 43 ml, LV EF = 31 ± 6%, global longitudinal strain = −13 ± 4%, E/e′ ratio = 9 ± 3, MACE = 3. There was no statistically significant difference between the two groups, in terms of EDVi (p: 0.2), ESVi (p: 0.2), and E/e′ ratio (0.9), whereas a significant difference of LV EF, presence of significative mitral regurgitation, and global longitudinal strain were observed (respectively, p: 0.03, p: 0.009, and p: 0.03).ConclusionIn our population of children with DCM, LGE shows a global diffuse subendocardial pattern. Presence of LGE seems to play a role in these patients determining a worst global systolic function.

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Section: Discussionmentioning

confidence: 99%

Results of Late Gadolinium Enhancement in Children Affected by Dilated Cardiomyopathy

et al. 2017

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“…It can be defined as the death of an apparently healthy child or infant within 24 hours of the onset of symptoms and signs in the absence of a previous diagnosis of neoplasia [1]. The most common tumors were those affecting primarily the heart and brainstem with direct invasion of the cardiac conduction system or respiratory centers of the pons or subsequent compression of major vessels [2,3]. In a 20-year retrospective review of autopsy cases at the Division of Pathology, Hospital for Sick Children in Toronto, Ontario, Canada, 7 cases of SUDNIC were identified out of 4926 autopsies (0.14%) [1].…”

Section: Discussionmentioning

confidence: 99%

Sudden Unexpected Death in Infancy Associated with an Epithelial-Type Hepatoblastoma in a 6-Month-Old Infant

2010

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Sudden unexpected death in infancy and childhood attributable to undiagnosed neoplasia is rare. Malignant neoplasms are very uncommon in infancy, with an age-standardized incidence rate of 118.3 per million. Primary malignant liver tumors are rare, with hepatoblastoma accounting for up to two thirds of cases. Although hepatoblastoma is the most common malignant neoplasm of the liver in childhood, it only accounts for 3.1% of childhood cancers for infants less than 12 months of age. We describe the first case of sudden death in an apparently healthy 6-month-old infant whose autopsy revealed an epithelial type hepatoblastoma with mixed fetal and embryonal patterns.

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“…Clinical findings mainly include heart failure, pulmonary infection, exercise intolerance, shortness of breath, and prolonged breastfeeding time ( 4 ). Importantly, the onset of EFE is rapid and patients might have a poor prognosis or even die without timely treatment ( 5 , 6 ). Therefore, the diagnosis of EFE should be established early and accurately.…”

Section: Introductionmentioning

confidence: 99%

The value of cardiac magnetic resonance imaging in endocardial fibroelastosis

Xiao

Wang²,

Cheng³

et al. 2022

Front. Pediatr.

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IntroductionEndocardial fibroelastosis (EFE), an uncommon congenital heart disorder often occurring in infants, has a poor prognosis. It is of great significance to perform early diagnosis and accurately analyze cardiac function to enable further clinical treatment and prognosis decisions. This study aimed to explore the findings of cardiac magnetic resonance (CMR) in patients with EFE, including morphological changes and cardiac function analyses. Additionally, we compared the difference in the evaluation of the cardiac function between CMR and echocardiography (Echo).MethodsEleven patients with EFE (nine females and two males, aged between 0.3 and 1.9 years), treated in our hospital, were analyzed retrospectively. Left ventricular posterior wall thickness (LVPW), anterior wall thickness (LVAW), fractional shortening (FS), ejection fraction (EF), end-systolic diameter (ESD), end-diastolic diameter (EDD), end-systolic volume (ESV), and end-diastolic volume (EDV) were assessed using both Echo and CMR. The Original Ross classification and the New York Heart Association functional classification were used to grade the patients’ cardiac function. The correlations between clinical cardiac function classification and MRI- and Echo-derived imaging data were determined.ResultsAll patients showed a thickened endocardium and left ventricle globular dilatation on CMR. We observed significant systolic dysfunction and whole or segmental abnormal ventricular movement. Compared with those measured by Echo, the EF, FS, and EDV values were significantly lower when measured using CMR. Compared with Echo measurements, the ESV, ESD, LVAW, and LVPW values were significantly higher when measured using CMR. CMR-measured EF and FS correlated better with the clinical cardiac functional score than those derived from Echo (EF, r = 0.646 > 0.224; FS, r = 0.627 > 0.245, respectively).ConclusionIn patients with EFE, the characteristic morphological changes of the heart could be displayed accurately using CMR. The parameters measured by CMR were more accurate than those of Echo and correlated well with clinical cardiac function scores, mainly because it does not make invalid geometrical assumptions.

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Endocardial Fibroelastosis as a Cause of Sudden Unexpected Death

Abstract: We present a case of primary endocardial fibroelastosis (EFE) which had been diagnosed in a 16-year-old girl who died suddenly and unexpectedly. This exceptional cause of death in adolescence led to a short literature review comparing our findings with previous medicolegal reports.

Cited by 11 publications

References 34 publications

Results of Late Gadolinium Enhancement in Children Affected by Dilated Cardiomyopathy

Results of Late Gadolinium Enhancement in Children Affected by Dilated Cardiomyopathy

Sudden Unexpected Death in Infancy Associated with an Epithelial-Type Hepatoblastoma in a 6-Month-Old Infant

The value of cardiac magnetic resonance imaging in endocardial fibroelastosis

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