Changing concepts of endocardial fibroelastosis

Lurie, Paul R.

doi:10.1017/s1047951110000181

Cited by 48 publications

(68 citation statements)

References 48 publications

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“…Endocardial fibrosis has been described in NS 8 and it has also been demonstrated in patients with endomyocardial fibrosis, M A N U S C R I P T A C C E P T E D ACCEPTED MANUSCRIPT 10 Loeffler syndrome, and endocardial fibroelastosis -all diagnoses that exhitbit restrictive physiology. [20][21][22][23][24][25] We speculate that the persistent left atrial dilation noted in NS patients postmyectomy may be a reflection of residual restrictive physiology. Histopathologic examination of such specimens may yield important clues as to the differences in the pathobiology between NS and HC.…”

Section: Discussionmentioning

confidence: 99%

Surgical Ventricular Septal Myectomy for Patients With Noonan Syndrome and Symptomatic Left Ventricular Outflow Tract Obstruction

Poterucha

Johnson

O’Leary

et al. 2015

The American Journal of Cardiology

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Section: Discussionmentioning

confidence: 99%

Surgical Ventricular Septal Myectomy for Patients With Noonan Syndrome and Symptomatic Left Ventricular Outflow Tract Obstruction

Poterucha

Johnson

O’Leary

et al. 2015

The American Journal of Cardiology

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“…Fibrotic changes in the fetal myocardium and endocardium likely occur early in utero because of the flow aberrations leading to altered loading conditions and subendocardial ischemia. 21,26,27 Extent of myocardial fibrosis and its clinical importance and prognostic value in patients who have undergone FAV and with congenital AS have not been well defined. Cardiac magnetic resonance imaging may be helpful in quantifying degree of myocardial fibrosis and play a role in determining which patients are most likely to progress successfully to biventricular circulation.…”

Section: Discussionmentioning

confidence: 99%

Postnatal Left Ventricular Diastolic Function After Fetal Aortic Valvuloplasty

Friedman

Margossian

Harrild

et al. 2011

The American Journal of Cardiology

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Fetal aortic balloon valvuloplasty (FAV) has shown promise in altering in utero progression of aortic stenosis to hypoplastic left heart syndrome. In patients who achieve a biventricular circulation after FAV, left ventricular (LV) compliance may be impaired. Echocardio-graphic indexes of diastolic function were compared between patients with biventricular circulation after FAV, congenital aortic stenosis (AS), and age-matched controls. In the neonatal period, patients with FAV had similar LV, aortic, and mitral valve dimensions but more evidence of endocardial fibroelastosis than patients with AS. Patients with FAV underwent more postnatal cardiac interventions than patients with AS (p = 0.007). Mitral annular early diastolic tissue velocity (E′) was lower in patients with FAV and those with AS and controls in the neonatal period and over follow-up (p <0.001). Septal E′ was similar among all 3 groups in the neonatal period. In follow-up patients, with FAV had lower septal E′ than patients with AS or controls (p <0.001). Early mitral inflow velocity/E′ was higher in patients with FAV as neonates and at follow-up (p <0.001). Mitral inflow pulse-wave Doppler-derived indexes of diastolic function were similar between groups. In conclusion, echocardiographic evidence of LV diastolic dysfunction is common in patients with biventricular circulation after FAV and persists in short-term follow-up. LV diastolic dysfunction in this unique population may have important implications on long-term risk of left atrial and subsequent pulmonary hypertension.

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“…On gross examination, these changes appear as a pearly or opaque white inner lining of the heart, predominately in the ventricles [1, 2]. The thickening caused by the abnormal deposition of collagen and elastin within the smooth muscle layer of the endocardium is thought to limit ventricular diastolic compliance and might also restrict ventricular growth [3, 4].…”

Section: Introduction1mentioning

confidence: 99%

“…EFE is reported in conjunction with a broad spectrum of diseases (Table 1), including hypoplastic left heart syndrome (HLHS) and viral endocarditis [1], which have been comprehensively reviewed in the literature. Little is known about the pathogenesis of this condition, in part due to the wide variety of circumstances in which it occurs.…”

Section: Introduction1mentioning

confidence: 99%

“…Little is known about the pathogenesis of this condition, in part due to the wide variety of circumstances in which it occurs. Some authors have suggested that an inflammatory response within the heart leads to a proliferation and migration of mesenchymal cells such as fibroblasts, myofibroblasts, or smooth muscle cells that deposit extracellular matrix constituents such as collagen and elastin within the endocardium [1, 4, 5]. …”

Section: Introduction1mentioning

confidence: 99%

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A mouse model of endocardial fibroelastosis

Clark

Pepper

Best

et al. 2015

Cardiovascular Pathology

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Background Endocardial Fibroelastosis (EFE) is a pathologic condition of abnormal deposition of collagen and elastin within the endocardium of the heart. It is seen in conjunction with a variety of diseases including hypoplastic left heart syndrome and viral endocarditis. While an experimental model using heterotopic heart transplant in rats has been described, we sought to fully describe a mouse model that can be used to further elucidate the potential mechanisms of and treatments for EFE. Materials and Methods The hearts of 2-day-old C57BL/6 mice were transplanted into the abdomen of 7-week-old C57BL/6 mice. At 2 weeks, the hearts were harvested and histologic analysis performed using hematoxylin and eosin, Masson’s Trichrome, Russell-Movat’s Pentachrome, Picrosirius Red, Hart’s, Verhoeff-Van Gieson, and Weigert’s Resorcin-Fuschin stains. Additionally, one heart was analysed using transmission electron microscopy (TEM). Results Specimens demonstrated abnormal accumulation of both collagen and elastin within the endocardium with occasional expansion in to the myocardium. Heterogeneity in extracellular matrix deposition was noted in the histologic specimens. In addition, TEM demonstrated the presence of excess collagen within the endocardium. Conclusions The heterotopic transplantation of an immature heart into a mouse results in changes consistent with EFE. This model is appropriate to investigate the etiology and treatment of endocardial fibroelastosis.

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Changing concepts of endocardial fibroelastosis

Cited by 48 publications

References 48 publications

Surgical Ventricular Septal Myectomy for Patients With Noonan Syndrome and Symptomatic Left Ventricular Outflow Tract Obstruction

Surgical Ventricular Septal Myectomy for Patients With Noonan Syndrome and Symptomatic Left Ventricular Outflow Tract Obstruction

Postnatal Left Ventricular Diastolic Function After Fetal Aortic Valvuloplasty

A mouse model of endocardial fibroelastosis

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