Primary hemochromatosis: anatomic and physiologic characteristics of the cardiac ventricles and their response to phlebotomy

Journal Article · Sun Jul 01 04:00:00 EDT 1984 · Am. J. Cardiol.; (United States)

DOI:https://doi.org/10.1016/0002-9149(84)90321-7· OSTI ID:6306984

Dabestani, A; Child, J S; Henze, E; Perloff, J K; Schon, H; Figueroa, W G; Schelbert, H R; Thessomboon, S

M-mode and 2-dimensional echocardiography and gated equilibrium blood pool imaging (rest and exercise) were used in 10 patients with primary hemochromatosis to characterize the spectrum of pathophysiologic abnormalities of the cardiac ventricles and to determine the response to chronic therapeutic phlebotomy. Dilated and restrictive cardiomyopathic patterns were identified in 1 patient each, but our data do not permit conclusions on when in the natural history a given pattern becomes overt. On entry into study, 3 patients had normal ventricles and 7 had ventricular abnormalities on echocardiography and blood pool angiography. In 2 of the latter patients, biventricular dysfunction and increased left ventricular (LV) mass normalized after phlebotomy; 1 patient achieved a normal LV response to exercise. Of the 4 patients with isolated abnormal LV ejection fraction responses to exercise, the EF normalized in 2 after phlebotomy. In 1 patient, isolated right ventricular enlargement and dysfunction (echocardiographic and radionuclide imaging) normalized after phlebotomy. Thus, primary hemochromatosis can effect LV and RV size and function; clinically occult cardiac involvement can be identified by echocardiography and equilibrium blood pool imaging; therapeutic phlebotomy can ameliorate or reverse the deleterious effects of excess cardiac iron deposition which appears to exert its harm, at least in part, by a mechanism other than irreversible connective tissue replacement.

• Research Organization: Univ. of California, Los Angeles
• OSTI ID: 6306984
• Journal Information: Am. J. Cardiol.; (United States), Journal Name: Am. J. Cardiol.; (United States) Vol. 54:1; ISSN AJCDA
• Country of Publication: United States
• Language: English

Related Subjects

550601* -- Medicine-- Unsealed Radionuclides in Diagnostics
62 RADIOLOGY AND NUCLEAR MEDICINE
BETA DECAY RADIOISOTOPES
BETA-MINUS DECAY RADIOISOTOPES
BODY
CARDIOVASCULAR SYSTEM
COUNTING TECHNIQUES
DIAGNOSIS
DIAGNOSTIC TECHNIQUES
DISEASES
DYNAMIC FUNCTION STUDIES
ELEMENTS
HEART
HOURS LIVING RADIOISOTOPES
INTERMEDIATE MASS NUCLEI
IRON
ISOMERIC NUCLEI
ISOMERIC TRANSITION ISOTOPES
ISOTOPES
METABOLIC DISEASES
METALS
MORPHOLOGICAL CHANGES
NUCLEI
ODD-EVEN NUCLEI
ORGANS
PATIENTS
RADIOISOTOPE SCANNING
RADIOISOTOPES
SCINTISCANNING
TECHNETIUM 99
TECHNETIUM ISOTOPES
TRANSITION ELEMENTS
ULTRASONOGRAPHY
YEARS LIVING RADIOISOTOPES