Cardiac involvement in systemic diseases and secondary cardiomyopathies

Author(s):  
Amit Patel ◽  
Tomasz Miszalski-Jamka ◽  
Sophie Mavrogeni ◽  
Jeanette Schulz-Menger

Cardiovascular magnetic resonance (CMR) is an important tool for the evaluation of patients with systemic diseases and secondary cardiomyopathies such as sarcoidosis, systemic lupus erythematosus, the vasculitides, rheumatoid arthritis, the muscular dystrophies, and several others. Although the clinical manifestation of these systemic disorders can be variable, it is increasingly evident that a significant amount of cardiovascular involvement can exist prior to the development of obvious functional abnormalities such as a decrease in left ventricular ejection fraction. Because CMR can evaluate many aspects of heart disease such as cardiac structure and function, including, but not limited to, myocardial perfusion, fibrosis, and inflammation, these previously difficult-to-identify cardiac abnormalities associated with systemic diseases and secondary cardiomyopathies can readily be identified, even in the absence of abnormalities on other non-invasive tests. The basic time-efficient protocol includes assessment of function and focal fibrosis applying late gadolinium enhancement. Reversible changes can be detected by oedema imaging. Recent developments allow quantification of subtle changes using parametric mapping. Improved detection of heart disease in these patients allows for earlier initiation of medical therapy and may identify those at highest risk for developing complications such as heart failure, significant arrhythmias, and other potentially life-threatening problems. This chapter reviews the role of CMR in the evaluation and management of these disorders.

2020 ◽  
Vol 30 (1) ◽  
pp. 12-18 ◽  
Author(s):  
Preeti Choudhary ◽  
Wendy Strugnell ◽  
Rajesh Puranik ◽  
Christian Hamilton-Craig ◽  
Shelby Kutty ◽  
...  

AbstractObjective:Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function.Methods:Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data.Results:The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71).Conclusions:Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.


Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Vikashsingh Rambhujun ◽  
Vijayapraveena Paruchuri ◽  
Abdul Moiz Hafiz ◽  
Catherine Kreatsoulas ◽  
Joshua DeLeon ◽  
...  

Introduction: Prominent left ventricular trabeculations or left ventricular non-compaction (LVNC) is observed now more often with cardiac magnetic resonance imaging (MRI). The significance of LVNC in patients with preserved ejection fraction remains unknown however it may be a precursor to cardiomyopathy. Hypothesis: This study is aimed to evaluate the clinical outcome of patients with LVNC and preserved LV function compared to patients with normal myocardial anatomy and function. Methods: This is a retrospective analysis of patients referred for cardiac MRI between October 2012 and July 2017. Patients with hypertrophic cardiomyopathy, ischemic heart disease and infiltrative heart disease were excluded. Patients with prominent trabeculations and a noncompacted to compacted myocardial ratio ≥ 2.3 with a preserved left ventricular ejection fraction of ≥ 50% were included. The primary outcome is defined as cardiac hospitalizations resulting from chest pain, arrhythmias, syncope, and congestive heart failure. These patients were compared to patients who underwent cardiac MRI with preserved function and normal myocardial anatomy. Results: There were a total of 39 patients who met criteria of LVNC with preserved function by MRI. These were compared to 59 patients with preserved function and normal myocardial anatomy on MRI. There was no significant difference in demographics and LV size and function between the groups. In comparison with the control group, the LVNC group had a mean age of 44 ± 14 versus 42 ± 16, 62% female sex versus 59%, LV EF of 59 ± 5% versus 62 ± 6%, LV end diastolic volume of 148 ± 26 ml versus 146 ± 40 ml. There were a total of 7 cardiac hospitalizations, of which 5 occurred in patients with LVNC. LVNC was associated with a greater than 4-fold increased risk of events (HR4.6, 95%CI 1.0-21.8) (figure) Conclusions: In patients with preserved EF, LVNC anatomy is significantly associated with increased cardiac hospitalizations.


1988 ◽  
Vol 27 (02) ◽  
pp. 57-62
Author(s):  
R. Standke ◽  
R. P. Baum ◽  
S. Tezak ◽  
D. Mildenberger ◽  
F. D. Maul ◽  
...  

21 patients with LAD-stenoses of at least 70% and 21 patients with LAD- stenoses and additional intramural anterior wall infarctions were studied. 20 patients without heart disease or after successful transluminal coronary angioplasty and 18 patients with intramural anterior wall infarction after successful transluminal dilatation of the LAD (remaining stenosis maximal 30%) served as controls. The normal range of global and regional left ventricular ejection fraction response to exercise was defined based on the data of 25 further patients without relevant coronary heart disease. Thus, a decrease in global ejection fraction and regional wall motion abnormalities were judged pathological. All patients were comparable with respect to age, ejection fraction at rest and work load. Myocardial ischemia could be detected by the exercise ECG in 81 % of all patients without infarction and in 71 % of patients with infarction. The corresponding values for global left ventricular ejection fraction were 76% and 81 %, respectively, and for regional ejection fraction 95% in both groups. No false-positive exercise ECGs were observed in the healthy controls and 2 (11 %) in the corresponding group with intramural infarction. The global ejection fraction was pathological in 1 (5%) healthy subject without infarction and in 3 (17%) corresponding patients with infarction. Sectorial analysis revealed 5 and 22%, respectively. Our findings suggest that the exercise ECG has a limited sensitivity to detect myocardial ischemia in patients with isolated LAD-stenoses and intramural myocardial infarction. Radionuclide ventriculography yields pathological values more often; however, false-positive results also occur more frequently.


2021 ◽  
Vol 10 (11) ◽  
pp. 2284
Author(s):  
Diana Gurzău ◽  
Alexandra Dădârlat-Pop ◽  
Bogdan Caloian ◽  
Gabriel Cismaru ◽  
Horaţiu Comşa ◽  
...  

Left bundle branch block is not a benign pathology, and its presence requires the identification of a pathological substrate, such as ischemic heart disease. Left bundle branch block appears to be more commonly associated with normal coronary arteries, especially in women. The objectives of our study were to describe the particularities of left bundle branch block in women compared to men with ischemic heart disease. Result: We included seventy patients with left bundle branch block and ischemic heart disease, with a mean age of 67.01 ± 8.89 years. There were no differences in the profile of risk factors, except for smoking and uric acid. The ventricular depolarization (QRS) duration was longer in men than women (136.86 ± 8.32 vs. 132.57 ± 9.19 msec; p = 0.018) and also men were observed to have larger left ventricular diameters. Left bundle branch block duration was directly associated with ventricular diameters and indirectly associated with left ventricular ejection fraction value, especially in women (R = −0.52, p = 0.0012 vs. R = −0.50, p = 0.002). In angiography, 80% of women had normal epicardial arteries compared with 65.7% of men; all these patients presented with microvascular dysfunction. Conclusion: The differences between the sexes were not so obvious in terms of the presence of risk factors; instead, there were differences in electrocardiographic, echocardiographic, and angiographic aspects. Left bundle branch block appears to be a marker of microvascular angina and systolic dysfunction, especially in women.


2016 ◽  
Vol 23 (4) ◽  
pp. 319-328 ◽  
Author(s):  
Fagen Xie ◽  
Chengyi Zheng ◽  
Albert Yuh-Jer Shen ◽  
Wansu Chen

The left ventricular ejection fraction value is an important prognostic indicator of cardiovascular outcomes including morbidity and mortality and is often used clinically to indicate severity of heart disease. However, it is usually reported in free-text echocardiography reports. We developed and validated a computerized algorithm to extract ejection fraction values from echocardiography reports and applied the algorithm to a large volume of unstructured echocardiography reports between 1995 and 2011 in a large health maintenance organization. A total of 621,856 echocardiography reports with a description of ejection fraction values or systolic functions were identified, of which 70 percent contained numeric ejection fraction values and the rest (30%) were text descriptions explicitly indicating the systolic left ventricular function. The 12.1 percent (16.0% for male and 8.4% for female) of these extracted ejection fraction values are <45 percent. Validation conducted based on a random sample of 200 reports yielded 95.0 percent sensitivity and 96.9 percent positive predictive value.


1977 ◽  
Vol 53 (1) ◽  
pp. 55-61 ◽  
Author(s):  
A. L. Muir ◽  
W. J. Hannan ◽  
H. M. Brash ◽  
V. Baldwa ◽  
H. C. Miller ◽  
...  

1. In 18 patients with ischaemic heart disease left ventricular ejection fraction, measured by two different nuclear angiographic methods, has been compared with ejection fraction measured by single-plane contrast angiography. 2. The first nuclear angiographic technique involves detection of variation in the radioactivity from the left ventricle during the initial passage of a bolus of 99Tcm-labelled human serum albumin injected intravenously; the second is our own modification of a ‘gated’ method, which accumulates the radioactivity detected during the continuing recirculation of the plasma bound radioisotope, so presenting an ‘averaged’ ventricular volume curve. 3. Ejection fraction, measured by the ‘bolus’ method, is lower than that measured either by contrast ventriculography or by the ‘gated’ method. This may be due to a damping effect. 4. Ejection fraction measured by the ‘gated’ method is well correlated with that measured by contrast ventriculography (r = 0·89). 5. Our modification of the ‘gated’ method, which presents the changes in ventricular volume throughout the cardiac cycle, without needing computer facilities, is a useful non-invasive means for assessment of left ventricular function.


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