Left ventricular non-compaction in patients with single ventricle heart disease

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.

1995 ◽  
Vol 12 (2) ◽  
pp. 121-127 ◽  
Author(s):  
ZIYAD M. HIJAZI ◽  
QI-LING CAO ◽  
ROBERT GEGGEL ◽  
STEVEN L. SCHWARTZ ◽  
GERALD R. MARX ◽  
...  

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.


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.


2019 ◽  
Vol 100 (3) ◽  
pp. 381-385
Author(s):  
E I Myasoedova ◽  
O S. Polunina ◽  
L P Voronina ◽  
G A Mukhambetova

Aim. To assess the level of pro-adrenomedullin in patients with chronic forms of coronary heart disease and to identify possible relationship with the indicators of left ventricular systolic function. Methods. 110 patients with ischemic heart disease and myocardial infarction with preserved left ventricular ejection fraction [average age 54.9 (42; 64) years] and 130 patients with ischemic cardiomyopathy [average age 55.2 (42; 64) years] were observed. All patients underwent transthoracic echocardiography by a standard technique and pulsed-wave tissue Doppler, left ventricular ejection fraction and myocardial performance index (Tei index) were calculated, maximum systolic velocity of the lateral part of the fibrous ring of the mitral valve was estimated. Pro-adrenomedullin level was measured in serum. Results. In both groups the level of pro-adrenomedullin was statistically significantly higher than the control va­lues — 0.49 (0.18; 0.58] nmol/L (p <0.017 and p <0.001, respectively). At the same time, in the group of patients with ischemic cardiomyopathy, the level of pro-adrenomedullin was statistically significantly higher than in the group of patients with ischemic heart disease and myocardial infarction with preserved left ventricular ejection fraction [1.72 (1.56; 1.98) nmol/l and 0.89 (0.51; 1.35) nmol/l, p <0.001]. Correlation analysis in both groups revealed statistically significant associations of the pro-adrenomedullin level with the left ventricular ejection fraction (r=–0.45, p=0.039, r=–0.51, p=0.034), maximum longitudinal velocity of the left ventricle (r=0.50, p=0.027, r=0.59, p=0.019), Tei-index (r=0.50, p=0.027, r=0.59, p=0.019). Conclusion. The data obtained demonstrate the potential of determining the level of pro-adrenomedullin as a biochemical marker of left ventricular dysfunction.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Drabik ◽  
E Kwiecien ◽  
A Mazurek ◽  
M Urbanczyk ◽  
W Szot ◽  
...  

Abstract Introduction The three main techniques to evaluate myocardial function and volumes: two-dimensional echocardiography (TTE), gated single photon emission computed tomography (SPECT) and cardiac magnetic resonance imaging (cMRI), have important differences in accessibility and practicality. Purpose We aimed to evaluate: TTE, SPECT and cMRI in the measurement of left ventricular ejection fraction (LVEF), left ventricular end-diastolic (LVEDV) and left ventricular end-systolic volumes (LVESV) in patients with acute and chronic myocardial ischemic injury. Methods Consecutive patients with first large acute myocardial infarction (AMI) [LVEF ≤45% and/or cMRI infarct size ≥10% of left ventricle, 2–5 days after pPCI, n=10] and patients with chronic ischemic heart failure (CIHF) [LVEF ≤45% by SPECT, NYHA class II or III, n=10] were enrolled. Multimodality imaging using the study techniques was performed within 36 hours. Results LVEF measured with TTE, SPECT and cMRI did not differ between both groups (AMI: 41±2.5%, 37.9±2.7%, 41±2.7%; p=0.07, CHF: 29.6±3.2%, 29.7±3.0%, 32.9±4.9%; p=0.42). LVEDV and LVESV evaluated by SPECT were +33.6%, +38.2% higher in the AMI group and +40.7%, 40.4% higher in the CIHF group compared with TTE (p<0.001). When measured with cMRI they were +52.1%, +50.5%, +31.5%, +25.5% higher (AMI, CIHF group, respectively), compared with TTE (p<0.001). There was a strong positive correlation between LVEF derived from TTE and SPECT (r=0.81, AMI; r=0.92, CIHF), TTE and cMRI (r=0.89, AMI; r=0.75, CIHF), SPECT and cMRI (r=0.88, AMI; r=0.76, CIHF; all p<0.01). As well as for LVEDV (r=0.79, 0.88; 0.90, 0.85; 0.76, 0.87; all p<0.05) and LVESV measurements (r=0.90, 0.91; 0.94, 0.89; 0.85, 0.85, all p<0.05). Figure 1 Conclusions There is a strong correlations between TTE, SPECT, and cMRI in the assessment of left ventricular function in patients with AMI or CIHF. However, TTE appears to significantly underestimate left ventricular volumes in relation to SPECT and cMRI.


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.


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