牛丽莉
中国医学科学院阜外医院 超声科
Background:Evaluation of the tricuspid valve (TV) is crucial for clinical decision making and post-treatment follow-up in pulmonary hypertension (PH) patients. However, little is known about 4-dimensional (4D) TV geometric remodeling in patients with PH. The aim of this study was to examine the 4D geometry of the TV in PH and its correlation with PH severity.Methods:A total of 74 PH patients with mean pulmonary arterial pressure >25 mmHg and 15 age- and gender-matched healthy individuals were consecutively included from September 2017 to December 2018 in National Center for Cardiovascular Diseases, Fuwai Hospital. All participants underwent 2-dimensional (2D) and 4D transthoracic echocardiography and PH patients underwent right heart catheterization (RHC) within 48 hours of echocardiography. TV geometry was analyzed using a dedicated 4D echocardiography from the right ventricular-focused apical view.Results:Compared with controls, PH patients had significantly larger 4D tricuspid annular (TA) and TV tenting sizes except in the 2-chamber diameter. In high-quality image cases, maximal tenting height (MTH), coaptation point height, tenting volume and 4-chamber diameter had good or moderate correlation with PH severity graded according to RHC mean pulmonary artery pressure (r=0.705, r=0.644, r=0.602, r=0.472, respectively; P<0.001 for all). In multivariable linear regression analysis, PH severity was independently associated with coaptation point height (F=18.070, P<0.001 with an R2=0.647) and MTH (F=25.576, P<0.001 with an R2=0.378). Among all 4D TV parameters, MTH had the highest area under the receiver operating characteristic (ROC) curve (AUC) in high-quality image cases [AUC =0.857, 95% confidence interval (CI): 0.743-0.972; P<0.001], comparable to echocardiographic systolic pulmonary arterial pressure (AUC =0.847, 95% CI: 0.733-0.961; P<0.001).Conclusions:In PH, TV geometric remodeling occurs mainly in TA septal-lateral dimension and TV tenting height. Worsening PH is an independent determinant of TV coaptation point height and MTH, not TA size. MTH shows a great diagnostic potential to detect severe PH.
Quantitative imaging in medicine and surgery 2024
BACKGROUND:Sengers syndrome characterized by hypertrophic cardiomyopathy is an extremely rare genetic disorder. Sengers syndrome associated with left ventricular non-compaction (LVNC) has not been described.METHODS:Genetic testing was used to identify candidate AGK variants in the proband. The predicted molecular structures were constructed by protein modeling. Exon skipping caused by the identified splicing mutations was verified by in silico analyses and in vitro assays. The genotypic and phenotypic features of patients with AGK splicing mutations were extracted by a systematic review.RESULTS:The proband was characterized by Sengers syndrome and LVNC and caused by a novel compound heterozygous AGK splicing mutation. This compound mutation simultaneously perturbed the protein sequences and spatial conformation of the acylglycerol kinase protein. In silico and in vitro analyses demonstrated skipping of exons 7 and 8 and premature truncation as a result of exon 8 skipping. The systematic review indicated that patients with an AGK splicing mutation may have milder phenotypes of Sengers syndrome.CONCLUSIONS:The genotypic and phenotypic spectrums of Sengers syndrome have been expanded, which will provide essential information for genetic counseling. The molecular mechanism in AGK mutations can offer insights into the potential targets for treatment.IMPACT:First description of a child with Sengers syndrome and left ventricular non-compaction cardiomyopathy. A novel pathogenic compound heterozygous splicing mutation in AGK for Sengers syndrome was identified. The identified mutations led to exons skipping by in silico analyses and in vitro assays.
Pediatric research 2023
Background: Four-dimensional automatic right ventricular quantification technology (4D auto-RVQ) is a new method that can simultaneously measure right ventricular (RV) structure and strain. The role of 4D auto-RVQ in determining RV function and hemodynamics is not clear. The role of 4D auto-RVQ in determining RV function and hemodynamics is not clear. We assessed the 4D auto-RVQ to measure right heart structure, function, and hemodynamics in patients with pulmonary hypertension (PHTN) correlated with right heart catheterization (RHC). Methods: We enrolled a prospective cohort of 103 patients with PHTN and 25 healthy controls between September 2017 and December 2018. All patients with PHTN underwent echocardiography and RHC. Patients were included if they underwent two-dimensional (2D) and 4D auto-RVQ echocardiographic sequences on the same day as RHC. We analyzed RV functional indices using 2D and 4D auto-RVQ analyses. We divided patients with PHTN into three groups according to echocardiographic image quality as follows: high (n = 24), average (n = 48), and poor (n = 4). Hemodynamic parameters were measured using RHC, including mean right atrial pressure, mean pulmonary arterial pressure, RV cardiac index (RV-CI), and pulmonary vascular resistance. Results: There were significant differences in most 2D and 4D auto-RVQ parameters between patients with PHTN and healthy controls. Interobserver variability showed significant agreement with 4D auto-RVQ for most measurements except for 4D end-diastolic volume. Indices measured by auto 4D-RVQ in the high-quality image group had a good correlation with RHC but not in the average- and poor-quality image group. Mid-RV diameter showed the best predictive power for the right RV-CI [area under the curve (AUC) 0.935; 95% confidence interval (CI), 0.714-0.997; p < 0.001]. RV end-systolic volume >121.50 mL had a 71.43% sensitivity and a 100% specificity to predict right RV-CI (AUC, 0.890; 95% CI, 0.654-0.986; p < 0.001). Conclusions: 4D auto-RVQ may be used to estimate RV function and some hemodynamic changes compared with RHC in PHTN patients with high image quality. Furthermore, a large sample of the study is needed to evaluate RV function by 4D auto-RVQ in PHTN patients with average image quality.
Frontiers in cardiovascular medicine 2021
BACKGROUND:Myocardial layer-specific strain can identify myocardial ischemia. Global myocardial work efficiency (GWE) based on non-invasive left ventricular (LV) pressure-strain loops is a novel parameter to determine LV function considering afterload. The study aimed to compare the diagnostic value of GWE and myocardial layer-specific strain during treadmill exercise stress testing to detect significant coronary artery disease (CAD) with normal baseline wall motion.METHODS:Eighty-nine patients who referred for coronary angiography due to suspected of CAD were included. Forty patients with severe coronary artery stenosis were diagnosed with significant CAD, and 49 were defined as non-significant CAD. Stress echocardiography was performed 24 h before angiography. Layer-specific longitudinal strains were assessed from the endocardium, mid-myocardium, and epicardium by 2D speckle-tracking echocardiography. Binary logistic regression analyses were performed to evaluate the association between significant CAD and echocardiographic parameters. A receiver operating characteristic curve was used to assess the capability of layer-specific strain and GWE to diagnose significant CAD.RESULTS:Patients with significant CAD had the worse function in all three myocardial layers at peak exercise compared with those with non-significant CAD when assessed with global longitudinal strain (GLS). At the peak exercise and recovery periods, GWE was lower in patients with significant CAD than in patients with non-significant CAD. In multivariable binary logistic regression analysis, peak endocardial GLS (OR: 1.35, p = 0.006) and peak GWE (OR: 0.76, p = 0.001) were associated with significant CAD. Receiver operating characteristic curves showed peak GWE to be superior to mid-myocardial, epicardial, and endocardial GLS in identifying significant CAD. Further, adding peak GWE to endocardial GLS could improve diagnostic capabilities.CONCLUSIONS:Both GWE and endocardial GLS contribute to improving the diagnostic performance of exercise stress echocardiography. Furthermore, adding peak GWE to peak endocardial GLS provides incremental diagnostic value during a non-invasive screening of significant CAD before radioactive or invasive examinations.
Frontiers in cardiovascular medicine 2021
To explore the value of right ventricular (RV) parameters detected by three-dimensional echocardiography (3DE) in risk stratification in pulmonary arterial hypertension (PAH) patients. We prospectively recruited 130 pulmonary hypertension patients from National Center for Cardiovascular Diseases, Fuwai Hospital. Each participant was performed a transthoracic echocardiography and 3DE parameters were measured using an off-line software (4D RV Function 2.0, TomTec). Patients were classified into low, intermediate-high risk group based on 2015 ESC Guidelines. A total of 91 PAH patients (34 ± 12 years old, 25 males) were enrolled, among which, 42 were classified into low risk group, while 49 were intermediate-high risk group. Compared with low-risk patients, those with intermediate-high risk had significantly larger 3DE-RV volumes, worse ejection fraction (EF) and tricuspid annular plane systolic excursion, and decreased longitudinal strain (LS). Receive operating characteristic curves illustrated all the 3DE parameters were able to predict intermediate-high risk stratification, especially 3D-RVEF (area under curve, 0.82, 95% CI 0.73-0.91, P < 0.001). And 3D-RVEF < 26.39% had a 81.6% sensibility and 73.8% specificity to predict intermediate-high risk stratification. Univariate and multivariate Logistic regression analyses identified 3D-RV end-diastolic (OR 1.02, 95% CI 1.01-1.03, P = 0.002) and end-systolic (OR 1.03, 95% CI 1.01-1.04, P < 0.001) volumes, 3D-RVEF (OR 0.82, 95% CI 0.75-0.90, P < 0.001) and LS of free wall (OR 1.17, 95% CI 1.05-1.31, P = 0.005) as independent predictors of intermediate-high risk stratification. In conclusion, RV volumes, EF and free wall strain detected by 3DE were independent predictors of intermediate-high risk stratification in PAH patients, among which, RVEF showed the best predictive capacity.
The international journal of cardiovascular imaging 2020
BACKGROUND:Right ventricular (RV) intraventricular mechanical dyssynchrony detected by two-dimensional speckle tracking echocardiography (2D-STE) has been reported to be correlated with a decrease in RV contractile efficiency in pulmonary hypertension (PH) patients, while little attention has been paid to biventricular dysfunction. Therefore, we aimed to evaluate the predictive value of 2D-STE detected interventricular dyssynchrony for exercise capacity and disease severity in patients with pre-capillary PH (PcPH).METHODS:Conventional transthoracic echocardiography, 2D-STE and cardiopulmonary exercise tests (CPETs) were performed in all participants. Intra- and interventricular dyssynchrony were calculated as the standard deviation (SD) of the time intervals corrected for heart rate between QRS onset and peak longitudinal strain. Multivariate linear regression analyses were performed to identify independent predictors of peak oxygen consumption (PVO2) during the CPET. Multivariable logistical regression modeling was used to analyze the associations between interventricular dyssynchrony and risk assessment.RESULTS:Sixty-six PcPH patients were consecutively recruited (19 male and 47 female, average 35 years old). WHO functional class, N-terminal pro-brain natriuretic peptide (BNP) and body mass index were included as independent predictors in the first multivariate regression analysis of clinical data without echocardiographic parameters (Model-1, r2=0.423, P<0.001). We subsequently added conventional echocardiographic parameters and 2D-STE parameters to the clinical data, RV fractional area change (Model-2, r2=0.417, P<0.001), RV global longitudinal strain (Model-3, r2=0.454, P=0.001), RV intraventricular dyssynchrony (Model-4: r2=0.474, P<0.001) and interventricular dyssynchrony (Model-5, r2=0.483, P<0.001) were identified as independent predictors of PVO2. Interventricular dyssynchrony, calculated as the SD of the time intervals of nine segments, was independently associated with risk assessment (odd ratio 1.027, 95% CI: 1.003-1.052, P=0.03). The area under the receiver-operating characteristic curve (AUC) was 0.73 (P<0.001).CONCLUSIONS:Interventricular dyssynchrony detected by 2D-STE contributed to a better evaluation of exercise capacity and disease severity in PcPH patients.
Annals of translational medicine 2020
PURPOSE:Imaging artifacts are frequently encountered when performing clinical echocardiography. Based on our review of the literature, two-dimensional linear artifacts are mainly reported in the ascending aorta in patients with suspected aortic dissections. However, pulmonary artery artifacts that mimic pulmonary artery dissection have not been discussed. We herein report our experience with children and adults with preexisting heart conditions and pulmonary artery imaging artifacts.METHODS:The study population comprised 10 patients with heart disease who were treated at our hospital from March 2015 to September 2017. Nine patients were children with congenital heart disease, mainly patent ductus arteriosus (n = 8), and one patient was an adult with pulmonary artery hypertension. Transthoracic echocardiography was performed in all patients.RESULTS:We confirmed the diagnosis in six patients during a surgical operation for other indications and in four patients by computed tomographic pulmonary angiography. The most common pulmonary imaging artifact was observed from the left high parasternal view (9/10, 90%). Most of the artifacts were diagonally oriented (8/10, 80%), and a few were horizontally oriented. Half of the artifacts were located in the main pulmonary arteries with mild pulmonary artery dilatation. Pulmonary hypertension was seen only in the adult patient. The thymus gland was clearly seen in young patients.CONCLUSION:Pulmonary artery imaging artifacts in patients with preexisting heart disease during echocardiographic examination can mimic pulmonary artery dissection. Understanding the types and origins of these ultrasound artifacts is important to avoid a false-positive diagnosis.
BioMed research international 2019
Resting two-dimensional speckle tracking echocardiography (2D-STE) identified right ventricular (RV) systolic function were reported to predict exercise capacity in pulmonary hypertension (PH) patients, but little attention had been payed to 2D-STE detected RV diastolic function. Therefore, we aim to elucidate and compare the relations between 2D-STE identified RV diastolic/systolic functions and peak oxygen consumption (PVO2) determined by cardiopulmonary exercise testing (CPET) in pre-capillary PH. 2D-STE was performed in 66 pre-capillary PH patients and 28 healthy controls. Linear correlation and multivariate regression analyses were performed to evaluate and compare the relations between RV 2D-STE parameters and PVO2. Receiver operating characteristic curves were used to compare the predictive value of 2D-STE parameters in predicting the cut-off-PVO2 < 11 ml/min/kg. There were significant differences of all the 2D-STE parameters between PH patients and healthy controls. In patients, RV-peak global longitudinal strain (GLS, rs = - 0.498, P < 0.001), RV- peak systolic strain rate (GSRs, rs = - 0.537, P < 0.001) and RV- peak early diastolic strain rate (GSRe, rs = 0.527, P < 0.001) significantly correlated with PVO2, but no significant correlation was observed between RV- peak late diastolic strain rate (GSRa, rs = 0.208, P = 0.093) and PVO2. The first multivariate regression analysis of clinical data without echocardiographic parameters identified WHO functional class, NT-proBNP and BMI as independent predictors of PVO2 (Model-1, adjusted r2 = 0.421, P < 0.001); Then we added conventional echocardiographic parameters and 2D-STE parameters to the clinical data, identified S,(Model-2,adjusted r2 = 0.502, P < 0.001), RV-GLS (Model-3, adjusted r2 = 0.491, P < 0.001), RV-GSRe (Model-4, adjusted r2 = 0.500, P < 0.001) and RV-GSRs (Model-5, adjusted r2 = 0.519, P < 0.001) as independent predictors of PVO2, respectively. The predictive power was increased, and Model-5 including RV-GSRs showed the highest predictive capability. ROC curves found RV-GSRs expressed the strongest predictive value (AUC = 0.88, P < 0.001), and RV-GSRs > - 0.65/s had a 88.2% sensibility and 82.2% specificity to predict PVO2 < 11 ml/min/kg. 2D-STE assessed RV function improves the prediction of exercise capacity represented by PVO2 in pre-capillary PH.
The international journal of cardiovascular imaging 2019
To investigate and compare the value of right ventricular longitudinal strain detected by two-dimensional and three-dimensional speckle-tracking echocardiography in risk stratification evaluation in pre-capillary pulmonary hypertension. We consecutively screened 66 patients diagnosed with pre-capillary pulmonary hypertension in our center. According to the risk assessment recommended by 2015 European Society of Cardiology Guidelines, all participants were classified into low- and intermediate-high-risk group. Two-dimensional and three-dimensional strains were measured using off-line softwares (GE EchoPAC version 201 and TomTec, 4D RV Function 2.0). Fifty-seven pre-capillary pulmonary hypertension patients (average 35 years old, 18 males and 39 females) were finally enrolled in our study, 32 (56.1%) were classified in low-risk group, while 25 (43.9%) were in the intermediate-high-risk group. Clinical data associated with disease severity, such as N-terminal pro-brain natriuretic peptide (r = 0.574, P < 0.001), peak oxygen consumption (r = -0.484, P < 0.001), and 6-min walking distance (r = -0.356, P = 0.008) were significantly correlated with two-dimensional right ventricular longitudinal strain; while the correlations with three-dimensional right ventricular longitudinal strain were weaker. Receiver operating characteristic curves for the detection of intermediate-high risk stratification showed two-dimensional right ventricular longitudinal strain had the best predictive capacity (area under curve, 0.82, 95% CI: 0.71-0.93, P < 0.001). Univariate and Multivariate Logistic regression analyses identified two-dimensional right ventricular longitudinal strain as an independent predictor (OR: 1.42, 95% CI: 1.18-1.71, P < 0.001) of intermediate-high risk stratification in this cohort of pre-capillary pulmonary hypertension patients, the predictive capacity retained (OR: 1.45, 95% CI: 1.18-1.78, P < 0.001) after adjusted by age, gender, and body mass index, while three-dimensional speckle-tracking echocardiography parameters were not. In conclusion, when used for the detection of intermediate-high risk stratification in pre-capillary pulmonary hypertension, two-dimensional right ventricular longitudinal strain was better than three-dimensional right ventricular longitudinal strain.
Pulmonary circulation 2019
We report a very rare case of mitral valve dissection and aortic-left ventricular tunnel caused by possible autoimmune vasculitis. We suspected Behcet's disease in this patient. There was no obvious clinical evidence of infective endocarditis. Echocardiography is the diagnostic tool of choice to recognize valvular dysfunction, related pathology, and possible complications. The patient may require immunosuppressive therapy due to the high likelihood of recurrence in the perioperation period.
Echocardiography (Mount Kisco, N.Y.) 2018
