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Murine Echocardiography of Left Atrium, Aorta, and Pulmonary Artery
Published on: February 20, 2017
Changes in left ventricular diastolic function of asymptomatic hereditary hemochromatosis subjects during five years
Yukitaka Shizukuda1, Dorothy J Tripodi, Vandana Sachdev
1Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland, USA. shizukya@uc.edu
Insights
Cardiac asymptomatic hereditary hemochromatosis (HH) patients show stable left ventricular (LV) diastolic function over five years with phlebotomy. This study confirms no significant decline in LV diastolic function for HH patients undergoing treatment.
Area of Science:
- Cardiology
- Hematology
- Genetics
Background:
- Previous studies indicated preserved left ventricular (LV) diastolic function in asymptomatic hereditary hemochromatosis (HH) patients, despite augmented left atrial function.
- Concerns remain regarding potential progressive LV diastolic dysfunction in HH patients despite conventional phlebotomy treatment.
Purpose of the Study:
- To prospectively monitor and assess the long-term (5-year) impact of conventional phlebotomy on LV diastolic function in cardiac asymptomatic hereditary hemochromatosis (HH) patients.
Main Methods:
- Prospective 5-year follow-up study involving 14 newly diagnosed HH, 20 chronic HH, and 18 volunteer control (VC) subjects.
- Evaluation of LV diastolic function using tissue Doppler imaging and strain rate analysis with color-coded tissue Doppler at baseline and 5-year follow-up.
- All HH patients were C282Y homozygotes, New York Heart Association functional class I, and received conventional phlebotomy.
Main Results:
- No statistically significant differences in LV diastolic function measures were observed among the HH and VC groups at the 5-year follow-up.
- Echocardiographic measures of active left atrial contraction showed a tendency to decrease in HH groups over the 5-year period compared to baseline.
- LV diastolic function remained comparable across all groups after 5 years, irrespective of HH diagnosis or treatment history.
Conclusions:
- Conventional phlebotomy treatment appears effective in maintaining stable left ventricular diastolic function in cardiac asymptomatic hereditary hemochromatosis patients over a 5-year period.
- The study provides reassurance that LV diastolic function does not significantly deteriorate in this patient population under standard care.
Abstract:
We have previously reported that left ventricular (LV) diastolic function in those with cardiac asymptomatic hereditary hemochromatosis (HH) is similar to that of volunteer control (VC) subjects, despite a presence of augmented left atrial contractile function. However, concern still exists that those with HH might gradually develop LV diastolic dysfunction despite receiving conventional phlebotomy treatment. To address this concern, we prospectively monitored the LV diastolic function of those with HH and VCs during a 5-year period. A total of 14 subjects with newly diagnosed HH (age 51 ± 12 years, 4 women, group A), 20 with chronic HH (age 51 ± 9 years, 7 women, group B), and 18 VCs (age 50 ± 8 years, 6 women, group C) successfully completed both the baseline evaluation of LV diastolic function, including tissue Doppler imaging, strain rate analysis with color-coded tissue Doppler, and the same studies repeated at 5 years of follow-up. All those with HH were New York Heart Association functional class I, were positive for the C282Y homozygote, and received conventional phlebotomy therapy. No VC had HH genetic mutations. The measures of LV diastolic function were comparable among the groups at 5 years of follow-up by analysis of variance. The echocardiographic measures of active left atrial contraction tended to decrease in the HH groups at 5 years of follow-up from baseline. In conclusion, LV diastolic function does not significantly deteriorate statistically during a 5-year period in subjects with cardiac asymptomatic HH after conventional phlebotomy treatment, regardless of their treatment history.
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