scholarly journals Left Ventricular Magnetic Resonance Imaging Strain Predicts the Onset of Duchenne Muscular Dystrophy–Associated Cardiomyopathy

2020 ◽  
Vol 13 (11) ◽  
Author(s):  
Saira Siddiqui ◽  
Tarek Alsaied ◽  
Sarah E. Henson ◽  
Janvi Gandhi ◽  
Priyal Patel ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Duchenne Muscular Dystrophy ◽  
Magnetic Resonance ◽  
Muscular Dystrophy ◽  
Ejection Fraction ◽  
Radial Strain ◽  
Left Ventricular ◽  
3 Dimensional ◽  
Lv Dysfunction ◽  
Lv Ejection Fraction

Background: Early detection of left ventricular (LV) dysfunction before the onset of overt Duchenne muscular dystrophy–associated cardiomyopathy (DMDAC) may direct clinical management to slow onset of dysfunction. We aimed to assess whether LV strain will predict those who develop DMDAC. Methods: We performed a single center retrospective case control study of patients with Duchenne muscular dystrophy who underwent serial cardiac magnetic resonance between 2006 and 2019. Patients with Duchenne muscular dystrophy with an LV ejection fraction ≥55% on ≥1 cardiac magnetic resonance were identified and grouped into age-matched +DMDAC and –DMDAC. Within 3 years, +DMDAC had a subsequent cardiac magnetic resonance with a decline in LV ejection fraction ≥10% and absolute LV ejection fraction ≤50%. −DMDAC maintained an LV ejection fraction ≥55% on serial cardiac magnetic resonances. Two-dimensional and 3-dimensional global radial strain, global circumferential strain (GCS), and global longitudinal strain were measured using tissue tracking software and their ability to predict DMDAC onset was assessed. Multivariable analysis adjusted for late gadolinium enhancement. Results: Thirty +DMDAC and 30 age-matched −DMDAC patients were included with a total of 164 studies analyzed. Before DMDAC onset, 2-dimensional global radial strain and GCS were significantly worse in +DMDAC compared with −DMDAC (25.1±6.0 versus 29.0±6.3, P =0.011; −15.4%±2.4 versus −17.3%±2.6, P =0.003). Three-dimensional GCS and global radial strain had similar findings. Among strain measures, 3-dimensional GCS had the highest area under the curve to predict DMDAC in our cohort. These findings persisted after adjusting for the presence of late gadolinium enhancement. Conclusions: Reduced global radial strain and GCS may predict those at risk for developing DMDAC before onset of LV dysfunction and its clinical utility warrants further exploration.

scholarly journals Left Ventricular Deformation in Patients with Connective Tissue Disease: Evaluated by 3.0T Cardiac Magnetic Resonance Tissue Tracking

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Jin Wang ◽  
Ke Shi ◽  
Hua-yan Xu ◽  
Qin Zhao ◽  
Xi Liu ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Connective Tissue ◽  
Ejection Fraction ◽  
Connective Tissue Disease ◽  
Left Ventricular ◽  
Lv Function ◽  
Peak Strain ◽  
Apical Slice ◽  
Lv Ejection Fraction

AbstractThe aim of this study was to assess left ventricular (LV) myocardial strain in patients with connective tissue disease (CTD) and compare LV deformation between subgroups of idiopathic inflammatory myopathy (IIM) and non-IIM. Ninety-eight patients with CTD, comprising 56 with IIM and 42 with non-IIM, and 30 healthy subjects were enrolled and underwent 3.0T cardiac magnetic resonance imaging (MRI) scanning. The LV function and strain parameters were measured and assessed. Our result revealed that CTD patients had preserved LV ejection fraction (60.85%) and had significantly decreased global and regional peak strain (PS) in radial, circumferential, and longitudinal directions (all p < 0.05). IIM patients showed significantly reduced global longitudinal PS (GLPS) and longitudinal PS at apical slice, whereas all strain parameters decreased in non-IIM patients. Except GLPS and longitudinal PS at apical slice, all strain parameters in non-IIM patients were lower than those in IIM patients. By Pearson’s correlation analysis, the LV global radial and circumferential PS were correlated to N-terminal pro-brain natriuretic peptide level and LV ejection fraction in both IIM and non-IIM patients. This study indicated that CTD patients showed abnormal LV deformation despite with preserved LVEF. The impairment of LV deformation differed between IIM and non-IIM patients.

Abstract 14890: Diagnosing Duchenne Muscular Dystrophy-Associated Cardiomyopathy: Echocardiogram Shoots, Cardiac Magnetic Resonance Imaging Scores

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Rahul Sheth ◽  
Claudio Ramaciotti ◽  
Faris G Araj ◽  
Daniel Cheeran ◽  
Alpesh A Amin ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cardiac Magnetic Resonance ◽  
Duchenne Muscular Dystrophy ◽  
Magnetic Resonance ◽  
Muscular Dystrophy ◽  
Cardiac Function ◽  
Left Ventricular ◽  
Resonance Imaging ◽  
Normal Lvef

Background: Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular disorder characterized by progressive muscle degeneration. The leading cause of death is complications from the development of DMD-associated cardiomyopathy. The majority of DMD patients have skeletal abnormalities that limit the quality of echocardiograms (ECHO). Although cardiac magnetic resonance imaging (cMRI) is the gold standard cardiac imaging tool, the vast majority of DMD patients are evaluated by ECHO rather than cMRI. We hypothesized that when compared to ECHO, cMRI more accurately assesses cardiac function and provides more valuable data for the diagnosis of DMD-associated cardiomyopathy. Methods: We undertook a retrospective study of 20 DMD patients referred to the UT Southwestern Adult Neuromuscular Cardiomyopathy Clinic who had undergone both an ECHO and cMRI within 15 months of each other. We assessed the proportion of studies demonstrating reduced left ventricular (LV) function [cMRI LVEF<62%, ECHO fractional shortening (FS)<25%] and that were technically difficult by both techniques. In addition, we assessed the proportion of cMRIs with late gadolinium enhancement (LGE), a marker of myocardial fibrosis and associated with increased mortality. Results: Amongst these patients, 10% of cMRIs and 85% of ECHOs were technically difficult. By cMRI, 30% of the studies were normal (LVEF 64±2 %), while 70% showed reduced function (LVEF: 44±12%). By ECHO, 75% of the studies were normal (FS 32±5), while 25% showed reduced function (FS: 15±8). Of the patients with truly reduced function by cMRI, 64% were considered normal by ECHO. Finally, 61% of cMRI studies showed LGE (50% with normal LVEF and 67% with reduced LVEF). Conclusions: In summary, cMRI is superior to ECHO in assessing cardiac function in DMD. ECHO underestimates left ventricular dysfunction, likely due to the poor quality of studies obtained in this patient population. In addition, cMRI identifies LGE, a marker associated with increased mortality, irrespective of cardiac function. Earlier utilization of cMRI will expedite the diagnosis of DMD-associated cardiomyopathy and enable the cardiologist to initiate aggressive guideline-directed medical therapy at an earlier age.

scholarly journals Effect of Myocardial Fibrosis on Left Ventricular Function in Rheumatic Mitral Stenosis: A Preliminary Study with Cardiac Magnetic Resonance

2018 ◽  
Vol 38 (4) ◽  
pp. 202-206
Author(s):  
Elen Elen ◽  
Celly A. Atmadikoesoemah ◽  
Manoefris Kasim
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Ejection Fraction ◽  
Myocardial Fibrosis ◽  
Mitral Stenosis ◽  
Left Ventricular ◽  
Lv Function ◽  
Fibrotic Tissue ◽  
Rheumatic Mitral Stenosis ◽  
Lv Dysfunction

Background: Left ventricular (LV) dysfunction was frequently found in rheumatic mitral stenosis. Myocardial fibrosis had been revealed in rheumatic heart disease and could be associated with LV dysfunction. We evaluate myocardial fibrosis profile related to LV function in rheumatic mitral stenosis with cardiac magnetic resonance (CMR). Methods: Eighteen patients with severe rheumatic mitral stenosis without history of coronary artery disease or its risk factors underwent 1.5T CMR examination. LV ejection fraction (LVEF), right ventricular ejection fraction (RVEF), myocardial fibrotic tissue were evaluated with CMR. Other hemodynamic data was derived from echocar­diography results. Results: These patients (40.4±10.5 years old, 72.2% female, 66.7% atrial fibrillation) had LVEF of 50.9±15.9% and RVEF of 37.7±13.9%. Volume of fibrotic tissue in these patients were 16.6 (5.5-55.8)%. In multivariate analysis, volume of fibrotic tissue was a significant predictor of LVEF that myocardial fibrotic tissue of 1% was associated with LVEF reduction of 0.87% (95% CI 0.51%-1.24%). Conclusion: LV function was determined by the extent of myocardial fibrosis in rheu­matic mitral stenosis.   Abstrak Latar Belakang: Disfungsi ventrikel kiri (LV) sering ditemukan pada mitral stenosis rematik. Fibrosis miokardium ditemukan pada penyakit jantung rematik. Fibrosis miokardium pada penyakit jantung rematik juga dihubungkan dengan disfungsi LV. Kami mengevaluasi profil fibrosis miokardium yang berhubungan dengan fungsi LV pada mitral stenosis rematik dengan cardiac magnetic resonance (CMR). Metode: Dilakukan pemeriksaan 1.5T CMR pada delapanbelas pasien dengan mitral stenosis rematik berat tanpa riwayat penyakit jantung koroner atau faktor resikonya. Fraksi ejeksi LV (LVEF), fraksi ejeksi RV (RVEF), dan jaringan fibrotik miokardium dievaluasi menggunakan CMR. Data hemodinamik lainnya didapatkan dari pemeriksaan ekokardiografi. Hasil: Pasien tersebut (40.4±10.5 tahun, 72.2% perempuan, 66.7% fibrilasi atrium) memiliki LVEF 50.9±15.9% dan RVEF 37.7±13.9%. Vol­ume jaringan fibrotic pada pasien tersebut adalah 16.6 (5.5-55.8)%. Dalam analisis multivariat, volume jaringan fibrotic adalah prediktor LVEF yang signifikan yaitu 1% jaringan fibrotic miokardium dihubungkan dengan menurunan LVEF sebesar 0.87% (95% CI 0.51%-1.24%). Kesimpulan: Fungsi LV dipengaruhi seberapa besar fibrosis miokardium pada mitral stenosis rematik

scholarly journals Fully penetrant genetic mutation results in wide familial variability: a cardiac magnetic resonance focused report

2021 ◽  
Vol 31 (1) ◽  
pp. 129-135
Author(s):  
Maria Livia Iovanescu ◽  
Andreea Sorina Marcu ◽  
Cristian Militaru ◽  
Octavian Istratoaie ◽  
Ioana Gheonea ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Genetic Testing ◽  
Magnetic Resonance ◽  
Ejection Fraction ◽  
Systolic Dysfunction ◽  
Interventricular Septum ◽  
Genetic Mutation ◽  
Left Ventricular ◽  
Cine Imaging ◽  
Lv Ejection Fraction

Dilated cardiomyopathy (DCM) is a myocardial disorder characterized by ventricular dilation (LV or biventricular) and systolic dysfunction, with a broad etiological spectrum, comprising numerous genetic and non-genetic causes. Cardiac magnetic resonance (CMR) has become an important tool in guiding the etiological diagnosis in DCM. We present the case of a 37-years old man admitted to our department in order to investigate the diagnosis of DCM using CMR. Cine imaging showed mild left ventricular (LV) dilatation and moderate systolic (LV ejection fraction = 42%) dysfunction, but also apical hypertrabeculation meeting the criteria for non-compaction and late gadolinium enhancement (LGE) images revealed mid-wall fibrosis in the basal and midventricular segments of the inferior interventricular septum (IVS) – typical of non-ischemic DCM. Complete family history revealed the diagnosis of DCM in the mother of the patient and in the maternal grandfather, who had died at 87. After genetic testing of the index patient showed a pathogenic mutation in the TTN (titin) gene (c.79273A>T), cascade genetic testing followed, for his mother, sister, uncle and two cousins who all came back positive for the same mutation. CMR examination of the mother done 6 years prior demonstrated severe LV dilatation and systolic dysfunction (LV ejection fraction = 23%), LV non-compaction and mid-wall IVS fi brosis. CMR examination was performed for the other members of the family and discovered pathological findings in the uncle (normal LV volume and function, but focal mid-wall fi brosis in the inferior IVS) and the male cousin (LV non- compaction), while the female cousin had a normal exam. Using CMR and genetic testing, this case report proves the phenotypic heterogenicity of a completely penetrant titin mutation in the same family. Moreover, CMR is shown to be essential in DCM evaluation, having the ability to guide etiologic diagnosis and to detect alterations such as fibrosis and non-compaction in the absence of LV dilation or dysfunction.

Association between Serum N-Terminal Pro-Brain Natriuretic Peptide Concentration and Left Ventricular Dysfunction and Extracellular Water in Continuous Ambulatory Peritoneal Dialysis Patients

2006 ◽  
Vol 26 (3) ◽  
pp. 360-365 ◽  
Author(s):  
Jung-Ahn Lee ◽  
Do-Hyoung Kim ◽  
Soo-Jeong Yoo ◽  
Dong-Jin Oh ◽  
Suk-Hee Yu ◽  
...  
Keyword(s):  
Peritoneal Dialysis ◽  
Ejection Fraction ◽  
Brain Natriuretic Peptide ◽  
Natriuretic Peptide ◽  
Continuous Ambulatory Peritoneal Dialysis ◽  
Left Ventricular ◽  
Extracellular Water ◽  
Volume Load ◽  
Lv Dysfunction ◽  
Lv Ejection Fraction

Background This study investigated the association between serum N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels and extracellular water (ECW%) and left ventricular (LV) dysfunction in continuous ambulatory peritoneal dialysis (CAPD) patients. Methods The study involved 30 stable CAPD patients: 14 males, 16 females; mean age 52 ± 14 years; mean CAPD duration 34 ± 12 months; 12 with diabetes mellitus (DM) and 18 non-DM. Serum NT-pro-BNP levels were determined using electrochemiluminescence immunoassay. Baseline echocardiography was performed using a Hewlett-Packard Sonos 1000 (Andover, Massachusetts, USA) device equipped with a 2.25-MHz probe, allowing M-mode, two-dimensional, and pulsed Doppler measurements. Left ventricular mass index (LVMI) was calculated according to the Penn formula. A multifrequency bioimpedance analyzer was used; ECW% was calculated as a percentage of total body water and was considered the index of volume load. Results ( 1 ) Serum NT-pro-BNP level, ECW%, LVMI, and LV ejection fraction in CAPD patients were 3924 (240 – 74460) pg/mL, 36.7% ± 2.2%, 158 ± 48 g/m2, and 60.5% ± 11.2%, respectively. ( 2 ) Patients were divided into three tertiles (10 patients each) according to their serum NT-pro-BNP concentration [1st tertile 1168 (240 – 2096), 2nd tertile 4856 (2295 – 20088), 3rd tertile 35012 (20539 – 74460) pg/mL]. The tertiles did not differ significantly in terms of age, sex, presence of DM, body mass index, or PD duration. Patients in the 3rd tertile (highest serum NT-pro-BNP concentration) had the highest LVMI (126 ± 45 vs 160 ± 41 vs 200 ± 23 g/m2 for 1st, 2nd, 3rd tertiles, respectively) and the lowest LV ejection fraction (66% ± 11% vs 62% ± 6% vs 55% ± 9%). ECW% did not differ significantly between tertiles (35.5% ± 2.0% vs 37.5% ± 2.0% vs 36.5% ± 2.0%). ( 3 ) In CAPD patients, serum NT-pro-BNP levels correlated positively with LVMI ( r = 0.628, p = 0.003) and negatively with LV ejection fraction ( r = –0.479, p = 0.033). Serum NT-pro-BNP levels did not correlate with ECW% ( r = 0.227, p = 0.25). ( 4 ) Stepwise regression analysis showed that LV ejection fraction (b = -0.610, p = 0.015) and LVMI (b = 0.415, p = 0.007) were independently associated with the serum NT-pro-BNP concentration. Conclusions There was no link between ECW% and serum NT-pro-BNP concentration. Thus, serum NT-pro-BNP levels may not provide objective information with respect to pure hydration status in CAPD patients. In contrast, serum NT-pro-BNP levels were linked to LVMI and LV ejection fraction in CAPD patients. Therefore, while the serum NT-pro-BNP concentration might not be a useful clinical marker for extracellular fluid volume load, it appears useful for evaluating LV hypertrophy and LV dysfunction in CAPD patients.

Sign in / Sign up

Export Citation Format

Share Document