Ke Wan

Distribution pattern of left-ventricular myocardial strain analyzed by a cine MRI based deformation registration algorithm in healthy Chinese volunteers

The cine magnetic resonance imaging based technique feature tracking-cardiac magnetic resonance (FT-CMR) is emerging as a novel, simple and robust method to evaluate myocardial strain. We investigated the distribution characteristics of left-ventricular myocardial strain using a novel cine MRI based deformation registration algorithm (DRA) in a cohort of healthy Chinese subjects. A total of 130 healthy Chinese subjects were enrolled. Three components of orthogonal strain (radial, circumferential, longitudinal) of the left ventricle were analyzed using DRA on steady-state free precession cine sequence images. A distinct transmural circumferential strain gradient was observed in the left ventricle that showed universal increment from the epicardial to endocardial myocardial wall (epiwall: −15.4u2009±u20091.9%; midwall: −18.8u2009±u20092.0%; endowall: −22.3u2009±u20092.3%, Pu2009<u20090.001). Longitudinal strain showed a similar trend from epicardial to endocardial layers (epiwall: −16.0u2009±u20092.9%; midwall: −15.6u2009±u20092.7%; endowall: −14.8u2009±u20092.4%, Pu2009<u20090.001), but radial strain had a very heterogeneous distribution and variation. In the longitudinal direction from the base to the apex of the left ventricle, there was a trend of decreasing peak systolic longitudinal strain (basal: −23.3u2009±u20094.6%; mid: −13.7u2009±u20097.3%; apical: −13.2u2009±u20095.5%; Pu2009<u20090.001). In conclusion, there are distinct distribution patterns of circumferential and longitudinal strain within the left ventricle in healthy Chinese subjects. These distribution patterns of strain may provide unique profiles for further study in different types of myocardial disease.

Reference value of left and right atrial size and phasic function by SSFP CMR at 3.0 T in healthy Chinese adults

The size and function of the left atrium (LA) and right atrium (RA) are related closely with the prognosis of cardiovascular diseases. However, their normal reference values, as measured by cardiac magnetic resonance (CMR), are not well established in Chinese populations. Healthy Chinese subjects (nu2009=u2009135, 66 males, age 23–83 years) without cardiovascular risk factors were recruited. We imaged the LA and RA of all subjects using short axis and long axis slices by steady-state free precession (SSFP) sequences using a 3.0T scanner. The size and functional parameters were measured. Age and gender differences in LA were further explored. The normal reference values of atrial dimensions, volumes, and empty fractions (EFs) were provided by short axis (SAX) and area-length methods. Volumes and EFs derived by the area-length method showed correlated well with those derived by the by SAX method, but significantly underestimated the volumes (all Pu2009<u20090.001) and overestimated the LA EFs (all Pu2009<u20090.001). Atrial dimensions and volumes were generally larger in males. Conduit EFs and total EFs showed gender differences. Most atrial parameters correlated with age. In general, our results showed that gender and age have considerable impact on LA and RA size and function.

Right ventricular involvement evaluated by cardiac magnetic resonance imaging predicts mortality in patients with light chain amyloidosis

Few studies have focused on right ventricular (RV) involvement in cardiac amyloidosis (CA). We investigated the prognostic value of RV assessment by cardiovascular magnetic resonance (CMR) in CA. In 2011–2014, consecutive patients with suspected CA referred for CMR were retrospectively evaluated. Demographic and baseline clinical characteristics were collected. Healthy volunteers were matched for sex and age and served as controls. All subjects underwent a contrast-enhanced CMR examination. RV size, function, and late gadolinium enhancement (LGE) were analyzed. All deaths during follow-up were recorded. Sixty-one patients [37 males (60.7%), age 60xa0±xa011xa0years] were included; CA was diagnosed in 47 (77.0%) patients. CA patients displayed decreased biventricular ejection fraction, elevated left ventricular mass index, and increased biventricular end-systolic volume index (ESVi) compared with controls. A total of 27 deaths (57.4%) occurred in the CA group at 21-month median follow-up. Multivariable analysis demonstrated that RVESVi (HR 1.033, 95% CI 1.004–1.063, Pxa0=xa00.026) and RV-LGE (HR 2.814, 95% CI 1.063–7.450, Pxa0=xa00.037) were independent predictors of mortality in CA. For all amyloid patients, log NT-proBNP (HR 3.412; 95% CI 1.484–7.845; Pxa0=xa00.004) and RV-LGE (HR 4.149; 95% CI 1.623–10.607; Pxa0=xa00.003) were identified as independent predictors. RVESVi and RV-LGE are independent predictors of survival and evaluation of RV by CMR enables risk stratification in patients with CA.

Phenotypic diversity identified by cardiac magnetic resonance in a large hypertrophic cardiomyopathy family with a single MYH7 mutation

Limited data is available on phenotypic variations with the same genotype in hypertrophic cardiomyopathy (HCM). The present study aims to explore the relationship between genotype and phenotype characterized by cardiovascular magnetic resonance (CMR) in a large Chinese family. A proband diagnosed with HCM from a multigenerational family underwent next-generation sequencing based on a custom sureSelect panel, including 117 candidate pathogenic genes associated with cardiomyopathies. All genetic results were confirmed by the Sanger sequencing method. All confirmed mutation carriers underwent CMR exam and myocardial tissue characterization using T1 mapping and late gadolinium enhancement (LGE) on a 3T scanner (Siemens Trio, Gemany). After clinical and genetic screening of 36 (including the proband) members of a large Chinese family, nineteen family members are determined to carry the single p.T1377M (c.4130C>T) mutation in the MYH7 gene. Of these 19 mutation carriers, eight are diagnosed with HCM, one was considered as borderline affected and ten are not clinically or phenotypically affected. Different HCM phenotypes are present in the nine affected individuals in this family. In addition, we have found different tissue characteristics assessed by T1 mapping and LGE in these individuals. We describe a family that demonstrates the diverse HCM phenotypes associated with a single MYH7 mutation.

Left Ventricular Myocardial Deformation on Cine MR Images: Relationship to Severity of Disease and Prognosis in Amyloid Light-Chain Amyloidosis.

Purpose To measure left ventricular (LV) myocardial strain with cine magnetic resonance (MR) imaging and a deformable registration algorithm (DRA) and to assess the prognostic value of myocardial strain in patients with light-chain (AL) amyloidosis. Materials and Methods In this prospective study, 78 consecutive patients with AL amyloidosis who underwent contrast material-enhanced cardiac MR imaging were enrolled at West China Hospital. LV myocardial strains and late gadolinium enhancement (LGE) were evaluated. Association between myocardial strain and all-cause mortality was analyzed with the stepwise Cox regression model. Results Global longitudinal strain (GLS) and global circumferential strain (GCS) were significantly lower in the no or nonspecific LGE group compared with the subendocardial LGE and transmural LGE groups (mean GLS, -10% ± 3 [standard deviation] vs -7% ± 3 vs -4% ± 1; P < .001) (mean GCS, -13% ± 3 vs -11% ± 3 vs -7% ± 2; P < .001). GLS and GCS were reduced in patients without clinical cardiac amyloidosis (mean GLS, -13% ± 3 vs -16% ± 2; P = .005) (mean GCS, -16% ± 1 vs -19% ± 2; P = .02). Circumferential and radial strains were impaired in basal segments in accordance with the distribution of LGE. Multivariate Cox analysis revealed that GCS (hazard ratio [HR] = 1.16 per 1% absolute decrease; 95% confidence interval [CI]: 1.03, 1.31; P = .02) and the presence of transmural LGE (HR = 1.75; 95% CI: 1.10, 2.80; P = .02) were independent predictors of all-cause mortality after adjustment for LV ejection fraction, right ventricular ejection fraction, LV mass index, GLS, and global radial strain. Conclusion Strain parameters derived with cine MR imaging-based DRA may be a new noninvasive imaging marker with which to evaluate the extent of cardiac amyloid infiltration and may offer independent prognostic information for all-cause mortality in patients with AL amyloidosis.

Cardiovascular magnetic resonance evidence of myocardial fibrosis and its clinical significance in adolescent and adult patients with Ebstein’s anomaly

BackgroundMyocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease. However, the presence, characteristics, and clinical significance of myocardial fibrosis in Ebstein’s anomaly have not been fully investigated. This study aimed to evaluate myocardial fibrosis using cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and T1 mapping techniques, and to explore the significance of myocardial fibrosis in adolescent and adult patients with Ebstein’s anomaly.MethodsForty-four consecutive patients with unrepaired Ebstein’s anomaly (34.0u2009±u200916.2xa0years; 18 males), and an equal number of age- and gender-matched controls, were included. A comprehensive CMR protocol consisted of cine, LGE, and T1 mapping by modified Look-Locker inversion recovery (MOLLI) sequences were performed. Ventricular functional parameters, native T1, extracellular volume (ECV), and LGE were analyzed. Associations between myocardial fibrosis and disease severity, ventricular function, and NYHA classification were analyzed.ResultsLGE was found in 10 (22.7%) patients. Typical LGE in Ebstein’s anomaly was located in the endocardium of the septum within the right ventricle (RV). The LV ECV of Ebstein’s anomaly were significantly higher than those of the controls (30.0u2009±u20093.8% vs. 25.3u2009±u20092.3%, Pu2009<xa00.001). An increased ECV was found to be independent of the existence of LGE. Positive LGE or higher ECV (≥30%) was associated with larger fRV volume, aRV volume, increased disease severity, and worse NYHA functional class. In addition, ECV was significantly correlated with the LV ejection fraction (Pu2009<u2009xa00.001).ConclusionsBoth focal and diffuse myocardial fibrosis were observed in adolescent and adult patients with Ebstein’s anomaly. Increased diffuse fibrosis is associated with worse LV function, increased Ebstein’s severity, and worse clinical status.

Right ventricular septomarginal trabeculation hypertrophy is associated with disease severity in patients with pulmonary arterial hypertension

To characterize the morphological change in the right ventricle (RV) of patients with pulmonary artery hypertension (PAH) and further explore the correlation between septomarginal trabeculation (SMT) and right ventricular (RV) function, myocardial fibrosis, and exercise capacity in patients with PAH. Sixty untreated PAH patients were prospectively included from May 2016 to April 2017. All patients underwent comprehensive clinical evaluation and cardiac magnetic resonance (CMR). The area and diameter of the basal segment of SMT, and the mass of SMT were measured on cine SSFP images. Relationship between parameters of SMT and RV ejection fraction (RVEF), 6xa0min walking distance (6MWD), myocardial fibrosis and pulmonary vascular resistance (PVR) were evaluated by Pearson’s correlation and logistic regression. Predictive performance of SMT parameters for reduced RVEF or impaired 6MWD was evaluated by receiver operating characteristics (ROC) analysis. Compared with SMT diameter index and mass index, SMT area index (SMT Ai) in basal segment was the best parameter to show correlation with RVEF (ru2009=u2009−u20090.496, Pu2009<u20090.001), 6MWD (ru2009=u2009−u20090.619, Pu2009<u20090.001), and inferior insertion point (I IP) extracelluar volume (ECV) (ru2009=u20090.365, Pu2009=u20090.008). ROC showed that SMT Ai had the strongest predictive value for reduced RVEF (AUCu2009=u20090.756), and impaired 6MWD (AUCu2009=u20090.813). SMT parameters were closely correlated with RV systolic function and 6MWD in patients with PAH. SMT Ai is a simple imaging indicator for the severity of PAH.

Age and Gender Impact the Measurement of Myocardial Interstitial Fibrosis in a Healthy Adult Chinese Population: A Cardiac Magnetic Resonance Study

Background: Diffuse myocardial fibrosis is a common pathological process in many cardiovascular diseases. In order to determine disease, we must have standard normal imaging values. We investigated myocardial interstitial fibrosis of the left ventricle (LV) in a healthy population of Chinese adults and explored the impact of gender, age, and other physiological factors using a T1 mapping technique of cardiac magnetic resonance imaging (CMR). Materials and Methods: We recruited 69 healthy adult Chinese subjects (35 males; age 18–76). LV function and global strain were obtained from functional imaging. T1 mapping was performed using a modified look-locker sequence. Global and segmental native T1 and extracellular volume (ECV) were calculated using dedicated software. Gender, age, and segmental variation of both native myocardial T1 and ECV of the LV were analyzed. Results: The global myocardial native T1 and ECV of the LV in this Chinese adult healthy population was 1,202 ± 45 ms and 27 ± 3% at 3T field strength, respectively. Females had a higher myocardial native T1 and ECV of the LV compared to males [1,210 (1,188–1,264) ms vs. 1,182 (1,150–1,211) ms, P < 0.001; 28 ± 3 vs. 26 ± 3%, P = 0.027, respectively]. ECV in older group was higher than younger group [27 (26–29)% vs. 25 (24–29), P = 0.019]. The multi-variate linear regression analysis showed that only gender (Beta = −0.512, P < 0.001) was independently related with global native T1 of LV while gender (Beta = −0.278, P = 0.017) and age (Beta = 0.303, P = 0.010) were independently related with global ECV of LV. From the base to apex of the LV, myocardial native T1 (P = 0.020) and ECV (P < 0.001) significantly increased. Within the same slice of the LV, there were significant segmental variations of both myocardial native T1 (P < 0.001) and ECV (P < 0.001) values. Conclusion: Gender and age have significant impacts on the imaging markers of myocardial interstitial fibrosis in healthy adult Chinese volunteers. Segmental variation of myocardial interstitial fibrosis was also observed.

A successful permanent pacemaker implantation in a challenging case with giant heart

A 56-year-old woman suffering from dizziness and pre-syncope was admitted for implantation of pacemaker. She was diagnosed with rheumatic mitral disease and received mitral valve replacement 25 years ago. Twenty-four hour Holter monitoring found maximum R–R interval of 6.4 s during day time. Chest X-ray showed a giant cardiac silhouette with a cardiothoracic ratio of 0.93 (Panel A). Transthoracic echocardiography demonstrated right and left atria was extremely enlarged (left atrium, 105 mm 120 mm 147 mm; right atrium, 46 mm 82 mm; left ventricle, 59 mm; right ventricle, 24 mm 42 mm; maximum tricuspid regurgitation velocity, 2.7 m/s) (Panel B). However, the lead failed to be firmly fixed after we removed the guidewire. After repeated attempts, we tried to gradually retrieve the

Increased Prognostic Value of Query Amyloid Late Enhancement Score in Light-Chain Cardiac Amyloidosis

BACKGROUNDnLate gadolinium enhancement (LGE) pattern is a powerful imaging biomarker for prognosis of cardiac amyloidosis. It is unknown if the query amyloid late enhancement (QALE) score in light-chain (AL) amyloidosis could provide increased prognostic value compared with LGE pattern.Methodsu2004andu2004Results:Seventy-eight consecutive patients with AL amyloidosis underwent contrast-enhanced cardiovascular magnetic resonance imaging. Patients with cardiac involvement were grouped by LGE pattern and analyzed using QALE score. Receiver operating characteristic curve was used to identify the optimal cut-off for QALE score in predicting all-cause mortality. Survival of these patients was analyzed with the Kaplan-Meier method and multivariate Cox regression. During a median follow-up of 34 months, 53 of 78 patients died. The optimal cut-off for QALE score to predict mortality at 12-month follow-up was 9.0. On multivariate Cox analysis, QALE score ≥9 (HR, 5.997; 95% CI: 2.665-13.497; P<0.001) and log N-terminal pro-brain natriuretic peptide (HR, 1.525; 95% CI: 1.112-2.092; P=0.009) were the only 2 independent predictors of all-cause mortality. On Kaplan-Meier analysis, patients with subendocardial LGE can be further risk stratified using QALE score ≥9.nnnCONCLUSIONSnThe QALE scoring system provides powerful independent prognostic value in AL cardiac amyloidosis. QALE score ≥9 has added value to differentiate prognosis in AL amyloidosis patients with a subendocardial LGE pattern.