Abstract 2938: Decrease in Endocardial Radial Strain Precedes Global Left Ventricular Dysfunction in Patients With Hypertension.

Background: Recent studies have reported that endocardial radial strain was approximately two fold greater than epicardial strain in normal subjects. Hypertension is a major cause of myocardial dysfunction and heart failure, but the difference between endocardial and epicardial radial strain in patients with hypertension is unknown. Two-dimensional speckle tracking echocardiography is a novel echocardiographic method that enables angle-independent assessment. Aim: The aim of this study was to evaluate the difference between radial strain in endocardial and epicardial segments in hypertensive patients. Method: This study enrolled 37 patients with hypertension (H group, age 63±12years, 24men) and 17 sex- and age-matched normal subjects (N group, age 61±7 years, 9 men). Conventional echocardiography was performed by a Vivid 7 dimension (GE). Endocardial and epicardial radial strains were calculated from the left ventricular (LV) short axis view at the papillary muscle level using custom software (EchoPAC (GE)). Results: There were no significant differences in LV diastolic dimension (47±5 mm vs. 48±4 mm), LV systolic dimension (29±4 mm vs. 29±4 mm), LV ejection fraction (65±9% vs. 67±6%) and fractional shortening (38±5% vs. 39±5%) between the H and N group. LV mass index (LVMI) in the H group was significantly greater than in the N group (109±33g/m2 vs. 90±22g/m2, P<0.05). Global radial strain including endocardium and epicardium were not significantly different between the H and N group (59±20% vs. 62±17%). However, endocardial radial strain in the H group was significantly less than in the N group (48±24% vs. 62±15%, P=0.03), whereas there was no significant difference in epicardial radial strain between the two groups (38±16% vs. 28±10%). The ratio of endocardial to epicardial radial strain (Endo/Epi ratio) in the H group was significantly smaller than in the N group (1.5±1.1 vs. 2.4±1.1, P<0.01). There were significant correlations between End/Epi ratio and LVMI (r=-0.33, P<0.05), and relative wall thickness (r=-0.43, P<0.01). Conclusion: A decrease in endocardial radial strain precedes global LV dysfunction in patients with hypertension.

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665 Longitudinal strain impairment in organic mitral regurgitation: look beyond the valve

European Heart Journal Supplements ◽

10.1093/eurheartj/suab147.007 ◽

2021 ◽

Vol 23 (Supplement_G) ◽

Author(s):

Giovanni Diana ◽

Laura Manfredonia ◽

Monica Filice ◽

Emanuele Ravenna ◽

Francesca Graziani ◽

...

Keyword(s):

Mitral Regurgitation ◽

Ejection Fraction ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

Left Ventricular ◽

Cardiac Damage ◽

Lv Dysfunction ◽

Lv Mass ◽

Significant Difference ◽

Lv Ejection Fraction

Abstract Aims Global longitudinal strain (GLS) is a hallmark of cardiac damage in mitral regurgitation (MR). GLS > −18% in patients with severe organic MR (OMR) and normal LV ejection fraction (LVEF) is an independent predictor of postoperative LV dysfunction. While it is known that GLS is impaired in less than severe functional ischaemic MR (FMR), the value of GLS in less than severe OMR is not known. We aimed to determine prevalence and determinants of any GLS impairment in OMR, in comparison to FMR. Methods We retrospectively evaluated 51 consecutive patients (33 OMR and 18 FMR) with mild-to-moderate, moderate and moderate-to-severe MR (Table*). Overall, GLS was higher in OMR than FMR (17.9±4.5 vs. 10.3±5.3, P<0.001), with rate of impairment of 45% in OMR and 89% in FMR (P= 0.0024). Results However, no significant difference was found in GLS between mild-to-moderate, moderate and moderate-to-severe MR patients within OMR (17.7±4.7 vs. 16.9±3.9 vs. 22.4±3, respectively, P>0.05), as well as FMR (9.8±6.6 vs. 10.7±5.3 vs. 10.4±5.3, respectively, P>0.05) groups. GLS correlated directly with left ventricular (LV) ejection fraction (EF) in both OMR (r=0.69, P<0.001) and FMR (r=0.90, P<0.001), and inversely with LV mass indexed for body surface area (LVMi) in both OMR (r = −0.50, P=0.005) and FMR (r = −0.48, P=0.042). While correlation with LVEF was better for FMR than OMR (Z − 1.95, P=0.026), correlation with LVMi was similar for OMR and FMR groups (Z − 0.082, P>0.05). Conclusions In patients with OMR, GLS may be reduced, despite normal LVEF, in less than severe MR. Prevalence and degree of GLS impairment in OMR is less than in FMR. In OMR, as well as in FMR, GLS impairment is independent of entity of MR, but rather correlates with LVMi, maybe reflecting impact of myocardial fibrosis derived by increased LVMi on GLS.

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Speckle tracking echocardiography in the assessment of mouse models of cardiac dysfunction

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.00385.2009 ◽

2009 ◽

Vol 297 (2) ◽

pp. H811-H820 ◽

Cited By ~ 53

Author(s):

Yu Peng ◽

Zoran B. Popović ◽

Nikolai Sopko ◽

Jeannie Drinko ◽

Zheng Zhang ◽

...

Keyword(s):

Heart Failure ◽

Mouse Models ◽

Speckle Tracking ◽

Speckle Tracking Echocardiography ◽

Radial Strain ◽

Left Ventricular ◽

Cross Sectional ◽

Isoflurane Anesthesia ◽

Lv Dysfunction ◽

Lv Mass

Two-dimensional (2-D) speckle tracking echocardiography (STE) accurately quantifies circumferential strain ( Scirc) and radial strain ( Srad) in humans and in large and small animals. This study was performed to assess sensitivity of Scirc and Srad to left ventricular (LV) dysfunction in mouse models. We performed 2-D and M-mode echocardiography 1) in 6 mice during superficial and profound isoflurane anesthesia, 2) serially in 12 mice to monitor the development of heart failure induced by transverse aortic constriction (TAC) and in 8 corresponding control mice, and 3) in 26 mice with varying degrees of TAC-induced heart failure and 12 corresponding control mice immediately before euthanasia. Fractional shortening (FS) and LV mass were measured from standard M-mode tracings, whereas Scirc and Srad were derived by STE. Percent fibrosis and myocyte diameters were assessed from whole heart cross-sectional specimens stained by Masson trichrome. Profound isoflurane anesthesia decreased Scirc ( P = 0.027) but not Srad ( P > 0.05). Mice subjected to TAC showed an immediate and sustained decrease in FS ( P = 0.035), Scirc ( P = 0.016), and Srad ( P = 0.012). Scirc showed better correlation with FS ( r = 0.56 and P < 0.0001) and LV mass ( r = 0.42 and P = 0.0003) than Srad ( r = 0.54 and P < 0.0001 for FS and r = 0.37 and P = 0.014 for LV mass, respectively). Percent fibrosis correlated better with Scirc ( r = 0.46 and P = 0.004) than with Srad ( r = −0.32 and P = 0.05), whereas myocyte diameter showed similar correlation with both strains ( r = 0.45 and r = −0.44, respectively, and P = 0.006 for both). STE correctly identifies LV dysfunction and histological changes in mice and can be used for the serial assessment of cardiac remodeling in murine models.

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Abstract 1585: Echocardiographic Left Ventricular Volumes are Optimally Indexed to Fat Free Mass to Adjust for Differences in Body Size

Circulation ◽

10.1161/circ.116.suppl_16.ii_330-c ◽

2007 ◽

Vol 116 (suppl_16) ◽

Author(s):

Robert N Doughty ◽

Helen J Walsh ◽

Greg D Gamble ◽

Philippa A Cross ◽

James Aoina ◽

...

Keyword(s):

Body Composition ◽

Body Size ◽

Normal Subjects ◽

Left Ventricular ◽

Fat Free Mass ◽

Left Ventricular Volumes ◽

Lv Mass ◽

Significant Difference ◽

Lv Volumes ◽

Relationship Of

Introduction Echocardiographic left ventricular (LV) size and structure are commonly indexed to body surface area (BSA) to adjust for body size. However, BSA may not be the optimal variable to adjust measurements to. While previous studies have demonstrated that the best determinant of LV mass is fat free mass (FFM), the relationship of LV volumes with body composition is uncertain. Objectives To determine the relationships between LV size and body composition. Methods Normal subjects (Caucasian, Maori, Samoan, Chinese and Indian ethnicity) free of cardiac disease underwent medical history, ECG, blood test (cholesterol/glucose), echocardiography for LV volumes (2D biplane methods of discs) and DEXA scans (body composition). Results 166 subjects were assessed: 96 (56%) were women, median age 26yrs, mean BMI 25.4 (SD 6.0), BSA 1.85m 2 (SD 0.28), LVEDV 88ml (SD 27), LVESV 33ml (SD 14). Significant correlations were observed between LV size and measures of body size in all ethnic groups. However, FFM was the best independent predictor of LV volumes (LVEDV partial R 2 0.48 – 0.60). Body size was smallest among Asian subjects and highest among Maori & Samoan subjects. A significant difference in unindexed LVEDV was observed across the groups which persisted when indexed to BSA or height (all P<0.01). However, when LVEDV was indexed to FFM the ethnic differences were reduced (figure ). Conclusions Among normal subjects LV size is strongly related to FFM. Indexing LV volumes to FFM largely eliminated the differences in LVEDV between subjects with varying body composition. Reference and partition values for normal LV size should be based on LV volumes indexed to fat free mass.

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Abstract 13245: Clinical Correlates and Heritability of Cardiac Mechanics: Insights from the HyperGEN Study

Circulation ◽

10.1161/circ.130.suppl_2.13245 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Sadiya S Khan ◽

Kwang-Youn A Kim ◽

Jie Peng ◽

Frank G Aguilar ◽

Senthil Selvaraj ◽

...

Keyword(s):

Speckle Tracking ◽

Radial Strain ◽

Cardiac Mechanics ◽

Population Based ◽

Left Ventricular ◽

Structural Factors ◽

Familial Predisposition ◽

Lv Dysfunction ◽

Genetic Heritability ◽

Lv Mass

Introduction: A genetic predisposition to abnormal cardiac mechanics may explain the familial predisposition to heart failure (HF). We hypothesized that indices of cardiac mechanics (speckle-tracking strain parameters and tissue velocities) are heritable traits. Methods: We performed speckle-tracking analysis on echocardiograms and measured global longitudinal, circumferential, and radial strain (GLS, GCS, GRS), early diastolic strain rate, and e’ velocities in the HyperGEN study, a family- and population-based study of hypertension (N=2058 [54% African American (AA)]). Additive genetic heritability estimates for cardiac mechanics were calculated by maximizing the likelihood variance components in SOLAR (ver.6) adjusted for age, sex, race, height, weight, systolic blood pressure (SBP), left ventricular (LV) mass, ejection fraction, reader, image quality, and institution. Results: Mean age was 51±14 years and HF risk factors were common: hypertension (56%), obesity (47%), and diabetes (16%). Clinical and conventional echocardiographic characteristics, including male gender, hypertension, elevated fasting glucose, higher body-mass index, and LV hypertrophy were all significantly associated with increased absolute GLS and septal e’ velocity (P<0.05). After adjustment for covariates (and correlation within the same families), genetic contributions remained significant in GLS and septal e’ velocity in all participants, a finding which persisted in race-stratified analyses (Table). Conclusions: Echocardiographic measures of cardiac mechanics, particularly GLS and e’ velocity, are heritable traits, even after adjustment for clinical and cardiac structural factors (e.g., SBP and LV mass, respectively) that are known to be heritable and associated with cardiac mechanics. These data support the exploration of genetics of cardiac mechanics, which may provide insight into the molecular pathogenesis of LV dysfunction and HF.

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Urine Albumin to Creatinine Ratio and Echocardiographic Left Ventricular Structure and Function in Patients with Essential Hypertension

The American Heart Hospital Journal ◽

10.15420/ahhj.2011.9.2.90 ◽

2011 ◽

Vol 9 (2) ◽

pp. 90 ◽

Cited By ~ 2

Author(s):

Rohola Hemmati ◽

Mojgan Gharipour ◽

Hasan Shemirani ◽

Alireza Khosravi ◽

Elham Khosravi ◽

...

Keyword(s):

Essential Hypertension ◽

Peak Velocity ◽

Left Ventricular ◽

Lv Function ◽

Creatinine Ratio ◽

Uncomplicated Hypertension ◽

Urine Albumin ◽

Lv Dysfunction ◽

Mitral Annulus Velocity ◽

Lv Mass

Background:Appearance of microalbuminuria, particularly in patients with hypertension, might be associated with a higher prevalence of left ventricular (LV) dysfunction and geometric abnormalities. This study was undertaken to determine whether high urine albumin to creatinine ratio (UACR) as a sensitive marker for microalbuminuria can be associated with LV hypertrophy (LVH) and systolic and diastolic LV dysfunction.Methods:The study population consisted of 125 consecutive patients with essential uncomplicated hypertension. Urine albumin and creatinine concentration was determined by standard methods. LVH was defined as a LV mass index >100 g/m2 of body surface area in women and >130 g/m2 in men. Echocardiographic LV systolic and diastolic parameters were measured.Results:The prevalence of microalbuminuria in patients with essential hypertension was 5.6 %. UACR was significantly no different in patients with LVH than in patients with normal LV geometry (21.26 ± 31.55 versus 17.80 ± 24.52 mg/mmol). No significant correlation was found between UACR measurement and systolic and diastolic function parameters, including early to late diastolic peak velocity (E/A) ratio (R=-0.192, p=0.038), early diastolic peak velocity to early mitral annulus velocity (E/E') ratio (R=-0.025, p=0.794), LV ejection fraction (R=0.008, p=0.929), and LV mass (R=-0.132, p=0.154). According to the receiver operator characteristic (ROC) curve analysis, UACR measurement was not an acceptable indicator of LVH with areas under the ROC curves 0.514 (95 % confidence interval 0.394–0.634). The optimal cut-off value for UACR for predicting LVH was identified at 9.4, yielding a sensitivity of 51.6 % and a specificity of 48.3 %.Conclusion:In patients with uncomplicated essential hypertension, abnormal systolic and diastolic LV function and geometry cannot be effectively predicted by the appearance of microalbuminuria.

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Myocardial deformation in patients with a single left ventricle using 2D cardiovascular magnetic resonance feature tracking: a case–control study

The International Journal of Cardiovascular Imaging ◽

10.1007/s10554-021-02230-2 ◽

2021 ◽

Author(s):

Fabian Strodka ◽

Jana Logoteta ◽

Roman Schuwerk ◽

Mona Salehi Ravesh ◽

Dominik Daniel Gabbert ◽

...

Keyword(s):

Cardiovascular Magnetic Resonance ◽

Magnetic Resonance ◽

Left Ventricle ◽

Feature Tracking ◽

Systolic Function ◽

Radial Strain ◽

Left Ventricular ◽

Lv Function ◽

Significant Difference ◽

Lv Volumes

AbstractVentricular dysfunction is a well-known complication in single ventricle patients in Fontan circulation. As studies exclusively examining patients with a single left ventricle (SLV) are sparse, we assessed left ventricular (LV) function in SLV patients by using 2D-cardiovascular magnetic resonance (CMR) feature tracking (2D-CMR-FT) and 2D-speckle tracking echocardiography (2D-STE). 54 SLV patients (11.4, 3.1–38.1 years) and 35 age-matched controls (12.3, 6.3–25.8 years) were included. LV global longitudinal, circumferential and radial strain (GLS, GCS, GRS) and strain rate (GLSR, GCSR, GRSR) were measured using 2D-CMR-FT. LV volumes, ejection fraction (LVEF) and mass were determined from short axis images. 2D-STE was applied in patients to measure peak systolic GLS and GLSR. In a subgroup analysis, we compared double inlet left ventricle (DILV) with tricuspid atresia (TA) patients. The population consisted of 19 DILV patients, 24 TA patients and 11 patients with diverse diagnoses. 52 patients were in NYHA class I and 2 patients were in class II. Most SLV patients had a normal systolic function but median LVEF in patients was lower compared to controls (55.6% vs. 61.2%, p = 0.0001). 2D-CMR-FT demonstrated reduced GLS, GCS and GCSR values in patients compared to controls. LVEF correlated with GS values in patients (p < 0.05). There was no significant difference between GLS values from 2D-CMR-FT and 2D-STE in the patient group. LVEF, LV volumes, GS and GSR (from 2D-CMR-FT) were not significantly different between DILV and TA patients. Although most SLV patients had a preserved EF derived by CMR, our results suggest that, LV deformation and function may behave differently in SLV patients compared to healthy subjects.

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Effect of heart rate on left ventricular longitudinal myocardial function in type 2 diabetes mellitus

Cardiovascular Diabetology ◽

10.1186/s12933-021-01278-7 ◽

2021 ◽

Vol 20 (1) ◽

Author(s):

Yuki Yamauchi ◽

Hidekazu Tanaka ◽

Shun Yokota ◽

Yasuhide Mochizuki ◽

Yuko Yoshigai ◽

...

Keyword(s):

Diabetes Mellitus ◽

Type 2 Diabetes ◽

Heart Rate ◽

Type 2 Diabetes Mellitus ◽

Myocardial Function ◽

Normal Subjects ◽

Left Ventricular ◽

High Heart Rate ◽

Lv Dysfunction

Abstract Background Left ventricular (LV) longitudinal myocardial dysfunction is considered a marker of preclinical LV dysfunction in patients with type 2 diabetes mellitus (T2DM). High heart rate (HR) is associated with cardiovascular outcomes, but the effect of HR on LV longitudinal myocardial function in T2DM patients is uncertain. Methods We studied 192 T2DM patients with preserved LV ejection fraction (LVEF), and 81 age-, sex-, and LVEF-matched healthy volunteers. HR was measured as the average HR during echocardiography, and high HR was defined as resting HR ≥ 70 beats/minute. LV longitudinal myocardial function was assessed as global longitudinal strain (GLS). The predefined cutoff for subclinical LV dysfunction was set at GLS < 18%. Results GLS in T2DM patients with high HR was significantly lower than that in T2DM patients with low HR (16.3% ± 4.2% vs. 17.8% ± 2.8%; P = 0.03), whereas GLS in normal subjects with high and low HR was similar (20.3 ± 1.7% vs. 20.3 ± 2.0%; P = 0.99). Multivariable logistic regression analysis showed that high HR (odds ratio: 1.04; 95% confidence interval: 1.01–1.07; P = 0.01) was independently associated with GLS < 18% in T2DM patients as well as HbA1c, T2DM duration, LVEF, body mass index, and mitral inflow E and mitral e’ annular velocity ratio. One sequential logistic model evaluating the associations between GLS < 18% and clinical variables in T2DM patients showed an improvement with the addition of LVEF and E/e’ (P < 0.001) and a further improvement with the addition of high HR (P < 0.001). Conclusion Compared with normal subjects, resting HR was associated with LV longitudinal myocardial function in asymptomatic T2DM patients with preserved LVEF. Our findings provide new insights on the management of T2DM patients.

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Effects of candesartan on left ventricular hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy

European Heart Journal ◽

10.1093/ehjci/ehaa946.3360 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

H Maqsood ◽

H.A Shakeel ◽

H.F Shoukat ◽

M.D Khan ◽

S.A.Y Shah ◽

...

Keyword(s):

Late Gadolinium Enhancement ◽

Hypertrophic Cardiomyopathy ◽

Interstitial Fibrosis ◽

Angiotensin Receptor Blockers ◽

Left Ventricular ◽

Funding Source ◽

Gadolinium Enhancement ◽

Percent Change ◽

Lv Mass ◽

Significant Difference

Abstract Introduction Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) hypertrophy in the absence of pressure overload. Manifestations of the disease include heart failure associated with diastolic dysfunction and atrial and ventricular tachyarrhythmias. Pathological features of HCM include myocyte hypertrophy, interstitial fibrosis, and myocyte disarray and are mediated by angiotensin II. Purpose This study aimed to evaluate the effects of candesartan on left ventricular (LV) hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy (HCM). Methods In double-blind fashion, 30 patients (6 women, 24 men; age: 55±11 years) with HCM were randomly assigned to receive placebo (n=13) or candesartan 50 mg twice a day (n=17) for 1 year. To measure LV mass and extent of fibrosis, cardiac magnetic resonance imaging was performed at baseline and 1 year as assessed by late gadolinium enhancement. Results There was a trend toward a significant difference in the percent change in LV mass (median: +5% with placebo vs. −5% with candesartan; p=0.06). There was a significant difference in the percent change in the extent of late gadolinium enhancement, with the placebo group experiencing a larger increase (+30±27% with placebo vs. −22±44% with candesartan; p=0.03). Conclusion Our study concludes reduction of the progression of myocardial hypertrophy and fibrosis with candesartan in patients with hypertrophic cardiomyopathy. Our study population was limited so we warrant larger trials to confirm a place for angiotensin receptor blockers in the management of patients with hypertrophic cardiomyopathy. Figure 1 Funding Acknowledgement Type of funding source: Other. Main funding source(s): Self funding

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Atrial and Ventricular Structural and Functional Alterations in Obese Children

Medicina ◽

10.3390/medicina57060562 ◽

2021 ◽

Vol 57 (6) ◽

pp. 562

Author(s):

Rima Šileikienė ◽

Karolina Adamonytė ◽

Aristida Ziutelienė ◽

Eglė Ramanauskienė ◽

Jolanta Justina Vaškelytė

Keyword(s):

Speckle Tracking ◽

Longitudinal Strain ◽

Speckle Tracking Echocardiography ◽

Myocardial Dysfunction ◽

Global Longitudinal Strain ◽

Radial Strain ◽

Left Ventricular ◽

Z Score ◽

Obese Children ◽

2D Speckle Tracking

Background and objectives: Childhood obesity has reached epidemic levels in the world. Obesity in children is defined as a body mass index (BMI) equal to or above the 95th percentile for age and sex. The aim of this study was to determine early changes in cardiac structure and function in obese children by comparing them with their nonobese peers, using echocardiography methods. Materials and methods: The study enrolled 35 obese and 37 age-matched nonobese children. Standardized 2-dimensional (2D), pulsed wave tissue Doppler, and 2D speckle tracking echocardiography were performed. The z-score BMI and lipid metabolism were assessed in all children. Results: Obese children (aged 13.51 ± 2.15 years; 20 boys; BMI z-score of 0.88 ± 0.63) were characterized by enlarged ventricular and atrial volumes, a thicker left ventricular posterior wall, and increased left ventricular mass. Decreased LV and RV systolic and diastolic function was found in obese children. Atrial peak negative (contraction) strain (−2.05% ± 2.17% vs. −4.87% ± 2.97%, p < 0.001), LV and RV global longitudinal strain (−13.3% ± 2.88% vs. −16.87% ± 3.39%; −12.51% ± 10.09% vs. −21.51% ± 7.42%, p < 0.001), and LV global circumferential strain (−17.0 ± 2.7% vs. −19.5 ± 2.9%, p < 0.001) were reduced in obese children. LV torsion (17.94° ± 2.07° vs. 12.45° ± 3.94°, p < 0.001) and normalized torsion (2.49 ± 0.4°/cm vs. 1.86 ± 0.61°/cm, p = 0.001) were greater in obese than nonobese children. A significant inverse correlation was found between LV and RV global longitudinal strain and BMI (r = −0.526, p < 0.01; r = −0.434, p < 0.01) and total cholesterol (r = −0.417, p < 0.01). Multivariate analysis revealed that the BMI z-score was independently related to LV and RV global longitudinal strain as well as LV circumferential and radial strain. Conclusion: 2D speckle tracking echocardiography is beneficial in the early detection of regional LV systolic and diastolic dysfunctions, with preserved ejection fraction as well as additional RV and atrial involvement, in obese children. Obesity may negatively influence atrial and ventricular function, as measured by 2D speckle tracking echocardiography. Obese children, though they are apparently healthy, may have subclinical myocardial dysfunction.

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Fatal "Iron Heart" in an Adolescent: Biochemical and Ultrastructural Aspects of the Heart

PEDIATRICS ◽

10.1542/peds.55.3.336 ◽

1975 ◽

Vol 55 (3) ◽

pp. 336-341

Author(s):

Shyamal K. Sanyal ◽

Warren Johnson ◽

B. Jayalakshmamma ◽

Alexander A. Green

Keyword(s):

Myocardial Dysfunction ◽

Ventricular Myocardium ◽

Left Ventricular ◽

Right Atrial ◽

Left Ventricular Myocardium ◽

Biochemical Basis ◽

Iron Storage ◽

Ultrastructural Studies ◽

Significant Difference ◽

Iron Deposits

Histochemical and ultrastructural aspects of the heart were investigated in an adolescent with fatal congestive heart failure resulting from exogenous hemochromatosis. Extensive iron deposits were found in all four chambers, papillary muscles, and the conduction system. These deposits were most prominent over the outer third of the left ventricular myocardium, with no significant difference between deposits in the middle and inner thirds. Quantitative analysis of iron from different chambers and all zones of the left ventricular myocardium confirmed the aforementioned pattern of iron distribution. Iron deposits in sinoauricular and atrioventricular nodes were similar to those in the right atrial myocardium. Degenerative changes and fibrosis were minimal. Ultrastructural studies showed that intracytoplasmic iron deposition followed a perinuclear, paranuclear, or diffuse pattern. In addition, some iron was consistently present in the nucleus and mitochondria. It is postulated that the presence of iron in the mitochondria may adversely affect the cellular enzyme system; this could provide a biochemical basis for myocardial dysfunction in patients with acquired iron-storage disease.

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