Myocardial Disorders

Benson, Lee; Wilson, Gregory J.; Freedom, Robert M.

doi:10.1007/978-1-4471-1814-5_41

Cited by 2 publications

(3 citation statements)

References 137 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…After birth, CM is diagnosed in 3% of newborns with cardiovascular disease. 11 The high intrauterine loss associated with fetal CM, occurring in one third of prenatally diagnosed cases, likely accounts at least in part for these differences. In addition, the lack of significant clinical manifestations after birth for certain forms of fetal CM, particularly those associated with an abnormal intrauterine milieu, may contribute to the discrepancy in incidence as well as differences in the distribution of lesions.…”

Section: Discussionmentioning

confidence: 99%

Fetal Cardiomyopathies

et al. 2002

View full text Add to dashboard Cite

Background-Although the prenatal diagnosis of most fetal structural heart defects and dysrhythmias has been described, there is a paucity of information about cardiomyopathies (CMs) in prenatal life. Methods and Results--To determine the pathogenic mechanisms, hemodynamic findings, and outcome of fetal CM, we reviewed the fetal echocardiograms and perinatal histories of 55 affected fetuses. Dilated CM was diagnosed in 22 cases, including 2 with congenital infections, 5 familial cases, 6 with endocardial fibroelastosis related to maternal anti-Ro/La antibodies, and 9 idiopathic cases. Thirty-three had hypertrophic CM, 7 associated with maternal diabetes, 2 with Noonan's syndrome, 2 with ␣-thalassemia, 18 with twin-twin transfusion syndrome, 1 with familial hypertrophy, and 3 with idiopathic hypertrophy. Systolic dysfunction was present in all cases of dilated CM and 15 cases of hypertrophic CM. Diastolic dysfunction was present in 19 of 30 fetuses with assessment of diastolic function parameters. Significant mitral or tricuspid valve regurgitation was seen in 32 cases. Eight fetuses were hydropic and 23 had signs of early hydrops. Seven pregnancies were terminated. Of 46 continued pregnancies with follow-up, 29 (63%) died perinatally. The presence of systolic dysfunction, diastolic dysfunction, and significant atrioventricular valve regurgitation were identified as risk factors for mortality. By multiple logistic regression, diastolic dysfunction was associated with an 8-fold increased risk relative to the other parameters. Conclusions-Fetal CM has a broad spectrum of intrinsic and extrinsic causes. A poor outcome is observed in many affected fetuses. Diastolic dysfunction in fetal CM is associated with the highest risk of mortality. (Circulation. 2002; 106:585-591.)

show abstract

Section: Discussionmentioning

confidence: 99%

Fetal Cardiomyopathies

et al. 2002

View full text Add to dashboard Cite

show abstract

“…1,28,31,[33][34][35] At present, EFE is most commonly thought to be a reaction to heart chamber stresses of variable aetiology and not a primary disease entity, a view first advocated by Black-Schaffer 44 and strongly supported by Lurie 18,23 among others. 12,45,52,53 Despite this, the concept of primary EFE as a specific diagnostic entity distinct from DCM persists. 22 We concur with Seki and colleagues 22 that further investigative attention to this pathological phenomenon is warranted to promote a greater understanding of its natural history and aetiology.…”

Section: Efementioning

confidence: 99%

“…Whether a primary entity or not, left ventricular EFE is an important distinctive pathological feature commonly found in paediatric DCM, the presence of which deserves to be terminologically highlighted. Thus, we advocate for the descriptive designation, "DCM with EFE", as previously proposed by Benson et al 53 to differentiate these cases from those without significant EFE and to facilitate future scientific investigation.…”

Section: Paediatric Dcm With Efementioning

confidence: 99%

Paediatric dilated cardiomyopathy with and without endocardial fibroelastosis – a pathological analysis of 89 explants

2021

View full text Add to dashboard Cite

Heart failure due to dilated cardiomyopathy is a major indication for paediatric cardiac transplantation. Endocardial fibroelastosis is a recognised pathological finding of unknown prognostic significance in paediatric dilated cardiomyopathy. To evaluate the nature of the association between left ventricular endocardial fibroelastosis and paediatric dilated cardiomyopathy, we reviewed surgical pathology reports of dilated cardiomyopathy explants (1986–2016) in order to characterise the pathological findings and to compare and contrast their frequency among four age groups: less than 1 year; 1–5 years; 6–10 years; and greater than 11 years. The 89 explants (47 males and 42 females) were all characterised by increased weight and left ventricular chamber dilatation without increased wall thickness. Ninety-five per cent of the specimens in the two youngest subsets had left ventricular endocardial fibroelastosis. Compared to the oldest age group, recipients aged 1–5 years had a 6-fold increase and those younger than 1 year a 19-fold increase in the odds of observing left ventricular endocardial fibroelastosis. Explants with and without endocardial fibroelastosis were otherwise phenotypically similar. In paediatric dilated cardiomyopathy endocardial fibroelastosis is a very common pathological finding, especially in infants and young children. We propose that the descriptive, clinico-pathological designation “Dilated Cardiomyopathy with Endocardial Fibroelastosis” should be adopted to facilitate future investigation into the potential prognostic/therapeutic significance of left ventricular endocardial fibroelastosis.

show abstract

Myocardial Disorders

Cited by 2 publications

References 137 publications

Fetal Cardiomyopathies

Fetal Cardiomyopathies

Paediatric dilated cardiomyopathy with and without endocardial fibroelastosis – a pathological analysis of 89 explants

Contact Info

Product

Resources

About