Right and Left Heart Catheterization and Angiocardiographic Findings in Idiopathic Cardiac Hypertrophy with Endocardial Fibroelastosis

Lynfield, Joshua; Gasul, Benjamin M.; Luan, Lawrence L.; Dillon, Robert J.

doi:10.1161/01.cir.21.3.386

Cited by 37 publications

(13 citation statements)

References 6 publications

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“…They felt that improvement in the six sur¬ vivors compared favorably with an expected 90% to 100% medical mortality. 3,5,12,22 When compared with our medical mortality, with cure or improvement in most of the sur¬ vivors, such a procedure seemed less beneficial. There is a possibility that selection of our two surgical cases on the basis of a myo¬ cardial infarction pattern on the electrocardi¬ ogram may mean that these were sicker patients.…”

Section: Commentmentioning

confidence: 97%

Prognosis in Endocardial Fibroelastosis

Linde¹,

Adams²

1963

Arch Pediatr Adolesc Med

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Section: Commentmentioning

confidence: 97%

Prognosis in Endocardial Fibroelastosis

Linde¹,

Adams²

1963

Arch Pediatr Adolesc Med

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“…Primary endocardial fibroelastosis has been clinically classified into 2 types; a dilated form associated with marked left ventricular enlargement, low cardiac output and terminal heart failure, and a contracted form associated with normal or reduced ventricular volumes, markedly altered ventricular compliance and secondary pulmonary hypertension. 4) Pathophysiologic conditions observed in our patient were marked elevation of the right and left ventricular diastolic pressures showing dip and plateau pressure contours, left atrial enlargement, moderate pulmonary hypertension and mild mitral regurgitation. These findings are consistent with those of the contracted form of primary endocardial fibroelastosis.…”

Section: Discussimentioning

confidence: 54%

Contracted form of primary endocardial fibroelastosis in a young adult without congestive heart failure.

et al. 1988

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A case of a 24-year-old man with the contracted form of primary endocardial fibroelastosis diagnosed by left ventricular endomyocardial biopsy showing a markedly thickened endocardium with fibroelastic proliferation is reported.He had no evident symptoms of congestive heart failure except for shortness of breath on moderate exertion.Echocardiogram showed thickened and dense echoes from the left side of the septum and from the posterior left ventricular endocardium. Hemodynamic and angiographic studies revealed marked elevation of right and left ventricular end-diastolic pressures with dip and plateau pressure contours, moderate pulmonary hypertension, left atrial enlargement and mild mitral regurgitation.Further elevation of right and left ventricular diastolic pressures and pulmonary artery pressure was observed at the second evaluation after 5 years. Our patient suggests that primary endocardial fibroelastosis should be included in the differential diagnosis of adult patients with obscure types of cardiac disease.

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“…Although the present case, as well as some others,2), 13), 19) was associated with VSD, it is generally accepted that endocardial fibroelastosis does not usually occur if a ventricular septal defect is present.20)-22) It is quite possible that VSD prevents stagnation of blood in a chamber and/or increase of the intraventricular pressure which favors for the establishment of endocardial fibroelastosis.15), 23), 24) It has been reported that the longer survived infants with congenital heart diseases the more extensive was endocardial fibroelastosis .16) Besides, severe endocardial fibroelastosis is rarely, if any, seen in stillborn infants .13), 25) Some people have suggested that occurrence of endocardial fibroelastosis is secondary to the associated congenital cardiac diseases or to some other factors.3), 13), 26) Andersen et al16) also hypothesized the occurrence of endocardial fibroelastosis to be secondary even for cases such as maximal endocardial changes were present at or near birth . A number of factors must .be taken into account for establishment of endocardial fibroelastosis associated with other congenital heart diseases.13) It may be impossible to decide whether all those endocardial changes had independently occurred or the valvular changes occurred first and the thickening of the endocardium was merely secondary.…”

Section: Dissionmentioning

confidence: 54%

Fetal Cardiac Failure Resulting from Endocardial Fibroelastosis

et al. 1974

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An autopsy was performed on a female stillborn infant with generalized edema associated with ascites. On gross examination of the heart the pulmonary trunk was atretic from its origin of the heart to the bifurcation. The ductus arteriosus was abnormally wide. The right ventricle was hypoplastic and its endocardium was diffusely thickened. The inner surface of right ventricle appeared pale, obscuring the identification of trabeculae carneae and papillary muscles. At the posterior wall there was an extremely thin lesion where no apparent cardiac muscle was contained. The tricuspid orifice was stenotic and the valve was hypoplastic. The right atrium was markedly enlarged and its wall was thickened. The foramen ovale had patency of the valvular competent type. Atrial septal defect (ASD) was not present. The left ventricle was remarkably dilated with markedly thick wall. Ventricular septal defect (VSD) was present immediately beneath the right coronary cusp of the aortic valve.Light microscopic examination of the right ventricle revealed a marked increase of collagen and elastic fibers with extensive deposits of calcium not only in the endocardium but also in the deep portion of myocardium. Fibroelastosis appeared particularly prominent in the subendocardial layer. The muscle fibers surrounded by these penetrating fibers and deposits of calcium showed degeneration. The entire myocardial layer of the specific area in the right ventricle where the wall was paper-thin was fully replaced by fibers and calcium. Inflammatory cell infiltration was found only at some places of the pericardium. The left ventricular wall also showed fibroelastosis, although the degree of involvement was much less as compared to that seen in the right ventricle. There were no significant pathological changes in both atria.

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Right and Left Heart Catheterization and Angiocardiographic Findings in Idiopathic Cardiac Hypertrophy with Endocardial Fibroelastosis

Cited by 37 publications

References 6 publications

Prognosis in Endocardial Fibroelastosis

Prognosis in Endocardial Fibroelastosis

Contracted form of primary endocardial fibroelastosis in a young adult without congestive heart failure.

Fetal Cardiac Failure Resulting from Endocardial Fibroelastosis

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