Familial occurrence of the contracted form of endocardial fibroelastosis

David E Fixler, Roger B. Cole, Milton H. Paul, Maurice Lev, Donald A. Girod

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

A family is presented in which the contracted form of endocardial fibroelastosis occurred in 2, possibly 3 generations. The family pedigree suggested a sex-linked recessive mode of inheritance. The 2 infants personally studied presented with severe heart failure and generalized edema and marked cardiomegaly. The electrocardiograms exhibited isolated right ventricular hypertrophy. Cardiac catheterization revealed severe pulmonary venous obstruction. Cineangio-cardiograms demonstrated normal left ventricular volume changes. Unresponsive heart failure with the hemodynamic evidence of mitral valve disease prompted cardiac surgery that was unsuccessful. Pathologically diffuse endocardial fibroelastosis involved all heart chambers. In contrast to the commoner dilated form of endocardial fibroelastosis, the left ventricular cavities were normal or mildly enlarged. This lesion is differentiated from other forms of pulmonary venous obstruction by elevation of the left ventricular end diastolic pressure level and absence of a diastolic pressure gradient distal to the pulmonary veins in fibroelastosis. Such differentiation is important because surgical treatment of contracted endocardial fibroelastosis has been uniformly unsuccessful.

Original languageEnglish (US)
Pages (from-to)208-213
Number of pages6
JournalThe American Journal of Cardiology
Volume26
Issue number2
DOIs
StatePublished - 1970

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Endocardial Fibroelastosis
Heart Failure
Blood Pressure
Right Ventricular Hypertrophy
Lung
Pulmonary Veins
Cardiomegaly
Pedigree
Cardiac Catheterization
Mitral Valve
Thoracic Surgery
Edema
Electrocardiography
Hemodynamics

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Familial occurrence of the contracted form of endocardial fibroelastosis. / Fixler, David E; Cole, Roger B.; Paul, Milton H.; Lev, Maurice; Girod, Donald A.

In: The American Journal of Cardiology, Vol. 26, No. 2, 1970, p. 208-213.

Research output: Contribution to journalArticle

Fixler, David E ; Cole, Roger B. ; Paul, Milton H. ; Lev, Maurice ; Girod, Donald A. / Familial occurrence of the contracted form of endocardial fibroelastosis. In: The American Journal of Cardiology. 1970 ; Vol. 26, No. 2. pp. 208-213.
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