Cardiac Abnormalities in Primary Hemochromatosis

Dabestani, Ali; Child, John S.; Perloff, Joseph K.; Figueroa, William G.; Schelbert, H.R.; Engel, Toby R.

doi:10.1111/j.1749-6632.1988.tb55509.x

Cited by 43 publications

(16 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Other studies have demonstrated the deleterious effect of high glucose levels on myocardial function with microvascular dysfunction and progressive LV remodeling being implicated for such changes [30]. It has been suggested that the deposition of cholesterol in the myocardium may cause a cholesterol myopathy similar to other infiltrating diseases [31]. Alternatively, myocardial metabolism may shift ATP production from a glucose based to a free fatty acid state [32] and flood the Krebs cycle to create more free radicals that injure the myocardium [33,34].…”

Section: Discussionmentioning

confidence: 99%

Hypercholesterolemia and Myocardial function evaluated via Tissue Doppler Imaging

Rubinstein

Pelosi

Vedre

et al. 2009

Cardiovasc Ultrasound

View full text Add to dashboard Cite

ObjectiveTo establish a link between hypercholesterolemia and myocardial dysfunction.BackgroundHeart failure is a complex disease involving changes in systolic and diastolic function. Newer echocardiographic imaging modalities may be able to detect discreet changes in myocardial function associated with hypercholesterolemia. Therefore we sought to establish a link between hypercholesterolemia and myocardial dysfunction with tissue Doppler imaging (TDI).MethodsTwenty-seven rabbits were studied: 7 were fed normal chow (group 1) and 20 a high cholesterol diet (10 with ezetimibe, 1 mg/kg/day; group 2 and 10 without, group 3). Echocardiographic images were obtained under general anesthesia. Serum cholesterol levels were obtained at baseline, 3 and 6 months and myocardial cholesterol levels measured following euthanasia.ResultsDoppler measurements, including E/A, E'/A' and S' were significantly lower in group 3 compared to both groups 1 and 2 but no significant differences were noted in chamber sizes or ejection fraction among the groups. Average serum cholesterol was higher in group 3 compared to groups 1 and 2 respectively (495 ± 305 mg/dl vs. 114 ± 95 mg/dl and 87 ± 37 mg/dl; p < 0.01). Myocardial cholesterol content was also higher in group 3 compared to group 2 (0.10 ± 0.04 vs. 0.06 mg/dl ± 0.02; p = 0.05). There was significant correlation between S', E'/A', E/E' and serum cholesterol (r2 = 0.17 p = 0.04, r2 = 0.37 p = 0.001 and r2 = 0.24 p = 0.01).ConclusionCholesterol load in the serum and myocardium was significantly associated with decreased systolic and diastolic function by TDI. Moreover, lipid lowering was protective.

show abstract

Section: Discussionmentioning

confidence: 99%

Hypercholesterolemia and Myocardial function evaluated via Tissue Doppler Imaging

Rubinstein

Pelosi

Vedre

et al. 2009

Cardiovasc Ultrasound

View full text Add to dashboard Cite

show abstract

“…Abnormalities of myocardial electroconductivity and myocardial function in humans were correlated morphologically with increased deposition of iron in both myocardium and conduction tissues (4,9,31). Longterm studies in thalassemic patients related marked cardiac hypertrophy and dilation with increased iron deposition.…”

Section: Discussionmentioning

confidence: 99%

“…We conclude that 1) initial decreases in liver magnetic resonance-relaxation time occur in the same range of iron excess as the threshold of iron load that induces delay or blockade of cardiac conduction and 2) a high incidence of sudden death, presumably from cardiac arrhythmias, was observed with large doses of iron that did not decrease left ventricular contractility. arrhythmia; cardiomyopathy; sudden death; electrophysiology IRON OVERLOAD PRODUCES CARDIAC TOXICITY affecting both electrical conduction and muscle contractility (9,10,24). Decreased survival in patients with hereditary hemolytic anemias and iron overload secondary to multiple transfusions of red blood cells is due primarily to iron-induced cardiac disease (21,28).…”

mentioning

confidence: 99%

Earliest cardiac toxicity induced by iron overload selectively inhibits electrical conduction

Schwartz¹,

Zy²,

Schwartz³

et al. 2002

Journal of Applied Physiology

View full text Add to dashboard Cite

show abstract

“…26–28 Once systolic dysfunction is documented, aerobic exercise capacity becomes compromised. 26,27 We have previously demonstrated that cardiac asymptomatic HH subjects have a similar aerobic exercise capacity compared to apparently healthy control subjects. 3 However, it was not clear at that point whether this would be a consistent finding at long-term follow-up since we have found that these HH subjects continue to be exposed to elevated levels of oxidative stress even as iron levels are improved, 29 and oxidative stress may affect aerobic exercise capacity through modulation of left ventricular diastolic function.…”

Section: Discussionmentioning

confidence: 96%

Changes in Exercise Capacity in Subjects with Cardiac Asymptomatic Hereditary Hemochromatosis During a Follow-Up After 5 yrs

Shizukuda

Smith

Tripodi

et al. 2012

American Journal of Physical Medicine &Amp; Rehabilitation

View full text Add to dashboard Cite

Objective A long-term effect of hereditary hemochromatosis (HH) on aerobic exercise capacity (AEC) has not been well described. Design Forty-three HH and 21 volunteer control (VC) subjects who were asymptomatic underwent cardiopulmonary exercise testing using the Bruce protocol. AEC was assessed with minute ventilation (VE), oxygen uptake (VO2), and carbon dioxide production (VCO2) at baseline (BL) at a 5-year follow up (5Y) assessment. A paired t-test was used for analyses of normality data; otherwise, a Wilcoxon singed rank sum test was used. Results Thirty-three HH subjects and 18 VC subjects returned for a repeat CPX at 5Y (80% overall return rate). At 5Y, AEC was not different between the two groups. As compared with BL measurements, exercise time, peak VO2, and the VE/VCO2 slope did not differ statistically at 5Y between both groups. Iron depletion by phlebotomy for 5 years did not significantly affect AEC in newly diagnosed HH subjects at baseline (n=14) and cardiac arrhythmias during exercise tended to decrease after 5 years of therapy in this group. Conclusions The AEC of asymptomatic HH subjects treated with conventional therapy is not statistically affected by the disease over a 5-year period.

show abstract

Cardiac Abnormalities in Primary Hemochromatosis

Cited by 43 publications

References 16 publications

Hypercholesterolemia and Myocardial function evaluated via Tissue Doppler Imaging

Hypercholesterolemia and Myocardial function evaluated via Tissue Doppler Imaging

Earliest cardiac toxicity induced by iron overload selectively inhibits electrical conduction

Changes in Exercise Capacity in Subjects with Cardiac Asymptomatic Hereditary Hemochromatosis During a Follow-Up After 5 yrs

Contact Info

Product

Resources

About