[Characteristics of the left heart structure and function in 86 term neonates with intrauterine growth restriction]. / 86ä¾‹å®«å† ç”Ÿé•¿å—é™è¶³æœˆæ–°ç”Ÿå„¿çš„å·¦å¿ƒç»“æž„åŠåŠŸèƒ½åˆ†æž.

OBJECTIVES: To study the left heart structure and functional characteristics of term neonates with intrauterine growth restriction (IUGR). METHODS: This study included 86 term neonates with IUGR admitted to the Neonatal Ward of Beijing Friendship Hospital, Capital Medical University from January 2019 to January 2022 as the IUGR group, as well as randomly selected 86 term neonates without IUGR born during the same period as the non-IUGR group. The clinical data and echocardiographic data were compared between the two groups. RESULTS: The analysis of left heart structure and function showed that compared with the non-IUGR group, the IUGR group had significantly lower left ventricular mass, left ventricular end-diastolic diameter, left ventricular end-systolic diameter, left atrial diameter, end-diastolic interventricular septal thickness, left ventricular posterior wall thickness, left ventricular end-diastolic volume, left ventricular end-systolic volume, and stroke volume (P<0.05) and significantly higher ratio of end-diastolic interventricular septal thickness to left ventricular posterior wall thickness, proportion of neonates with a mitral peak E/A ratio of ≥1, and cardiac index (P<0.05). The Spearman correlation analysis suggested that stroke volume was positively correlated with birth weight and body surface area (rs=0.241 and 0.241 respectively; P<0.05) and that the ratio of end-diastolic interventricular septal thickness to left ventricular posterior wall thickness was negatively correlated with birth weight and body surface area (rs=-0.229 and -0.225 respectively; P<0.05). CONCLUSIONS: The left ventricular systolic function of neonates with IUGR is not significantly different from that of neonates without IUGR. However, the ventricular septum is thicker in neonates with IUGR. This change is negatively correlated with birth weight and body surface area. The left ventricular diastolic function may be impaired in neonates with IUGR.

Compare image quality of T2-weighted imaging with different phase acceleration factors.

Previous studies demonstrated that adjusting the phase acceleration (PA) factors could influence image quality. To improve image quality and decrease respiratory artifacts of lesions in the liver on T2-weighted image by adjusting PA factor and number of excitation (NEX). Sixty consecutive patients with hepatic lesions were enrolled in this prospective research between May 2020 and June 2020. All patients had 3.0T magnetic resonance imaging with 4 sequences (combining PA factors and NEXs, the former was 2 and 3, the latter were 1.5 and 2, respectively, with the same other scanning parameters). Two readers used 5-point quality scales to assess image quality. The signal intensity was measured by drawing regions of interest in the liver, spleen, and background on the T2-weighted imaging. Artifacts, overall image impression, and vascular conspicuity were better when the PA factor was 3 than 2. Artifacts and vascular conspicuity were better when NEX was 2 than 1.5. PA factor 3 and NEX 2 got a higher score in 5-point quality scales and less scan time than the other 3 sequences. Meanwhile, the signal-to-noise ratio of PA factor 3 and NEX 2 was best among these 4 sequences. PA factor and NEX could influence the imaging quality and lesion-to-hepatic contrast in detecting hepatic lesions on T2-weighted images. PA factor 3 and NEX 2 may have a positive effect in the clinic, especially for those with irregular respiration, as it decreased artifacts and reduced scan time.

Clinical Characteristics of Congenital Atresia of the Left Main Coronary Artery in 12 Children.

Background: Left main coronary artery atresia (LMCAA) is an extremely rare abnormality and only <100 cases have been reported worldwide. We describe the clinical manifestations, imaging features, prognosis, and treatments of LMCAA who were admitted in our department, which aimed to improve the clinical diagnosis and treatments of LMCAA in children. Methods: A retrospective study identified 12 patients diagnosed with congenital left coronary artery atresia at Pediatric Heart Center of Beijing Anzhen Hospital from June 2010 to June 2019. The clinical characteristics, imaging data, and treatment follow-up were analyzed. Results: Among the 12 cases, 8 were boys and 4 were girls; the age of onset was 2 months to 2 years old (median age 7 months); the age of diagnosis was 7 months to 6 years old (median age 2 years and 11 months). At the initial diagnosis, there were 4 cases of respiratory tract infection with cardiac murmur, 3 cases of cardiac shadow enlargement, 1 case of recurrent syncope, 2 cases of feeding difficulty with cardiac enlargement, and 2 cases of simple cardiac murmur. In 12 cases of electrocardiogram examination, 7 cases showed pathological Q waves of lead I, AVL and v4-v6; in 12 cases of chest X-ray examination, 8 cases showed cardiac shadow enlargement; in 12 cases of our hospital's first cardiac ultrasound examination, 4 cases were definitely diagnosed, and 8 cases showed the possibility of left coronary artery abnormality; in 5 cases of cardiac coronary CT angiography examination, 2 cases were confirmed, 2 cases reported suspected left coronary artery abnormality, and 1 case did not report abnormality; All cases were definitely diagnosed in 8 cases of angiography. Follow-up was performed from 1 to 8 years; one case died suddenly, one case of syncope after activity was treated by oral medication, 3 cases received open coronary angioplasty and mitral valvuloplasty, recovered well after operation, the rest of the children were treated by oral medication, and the symptoms are stable at present. Conclusions: Left main coronary artery atresia is difficult to diagnose and can result in heart failure early in life. Timely diagnosis and reasonable treatment are the keys to improve the prognosis.

Pulmonary valve annular and right ventricular outflow tract size as predictions values for moderate to severe pulmonary regurgitation after repaired Tetralogy of Fallot.

OBJECTIVE: This study sought to investigate the variation of right heart structure pre- and post-operation as risk factors for moderate to severe pulmonary regurgitation (PR) after repaired Tetralogy of Fallot and the best "cutoff" values for the transannular patch (TAP). METHODS: We collected surgical, echocardiographic, and computed tomographic data of Teralogy of Fallot (TOF) patients over two years and calculated z-score values based on the echocardiographic data. Based on the PR level after follow-up, the patients were divided into two groups, trivial to mild PR and moderate to severe PR. A multivariate logistic regression analysis was performed, and the receiver operating characteristic curve analysis was used to find the best "cutoff" value for risk factors. RESULTS: A total of 104 TOF patients were included in our cohort study. From the multivariate analysis, correction strategy (P = .002), difference in zRVOT (OR 1.974, 95% CI 1.354 to 2.878, P < .0001), and zPVA (OR 3.605, 95% CI 1.980 to 6.562, P < .0001) were the significant risk factors for moderate to severe PR. The "cutoff" value for the difference in zPVA that could predict moderate to severe PR in the TAP group was 3, and the optimal "cutoff" value for TAP was -1.4. CONCLUSIONS: The TAP is a risk factor for significant PR after surgery. We recommend the optimal "cutoff" value for TAP is -1.4 calculated using Shan-Shan Wang's data set. During the procedure, to limit the RVOT resection and restrict the enlargement of pulmonary annulus within a variation of z-score as 3 would reduce significant PR.

HF-SENSE: an improved partially parallel imaging using a high-pass filter.

BACKGROUND: One of the major limitations of MRI is its slow acquisition speed. To accelerate data acquisition, partially parallel imaging (PPI) methods have been widely used in clinical applications such as sensitivity encoding (SENSE) and generalized autocalibrating partially parallel acquisitions (GRAPPA). SENSE is a popular image-domain partially parallel imaging method, which suffers from residual aliasing artifacts when the reduction factor goes higher. Undersampling the k-space data and then reconstruct images with artificial sparsity is an efficient way to accelerate data acquisition. By exploiting artificial sparsity with a high-pass filter, an improved SENSE method is proposed in this work, termed high-pass filtered SENSE (HF-SENSE). METHODS: First, a high-pass filter was applied to the raw k-space data, the result of which was used as the inputs of sensitivity estimation and undersampling process. Second, the adaptive array coil combination method was adopted to calculate sensitivity maps on a block-by-block basis. Third, Tikhonov regularized SENSE was then used to reconstruct magnetic resonance images. Fourth, the reconstructed images were transformed into k-space data, which was filtered with the corresponding inverse filter. RESULTS: Both simulation and in vivo experiments demonstrate that HF-SENSE method significantly reduces noise level of the reconstructed images compared with SENSE. Furthermore, it is found that HF-SENSE can achieve lower normalized root-mean-square error value than SENSE. CONCLUSIONS: The proposed method explores artificial sparsity with a high-pass filter. Experiments demonstrate that the proposed HF-SENSE method can improve the image quality of SENSE reconstruction. The high-pass filter parameters can be predefined. With this image reconstruction method, high acceleration factors can be achieved, which will improve the clinical applicability of SENSE. This retrospective study (HF-SENSE: an improved partially parallel imaging using a high-pass filter) was approved by Institute Review Board of 2nd Affiliated Hospital of Zhejiang University (ethical approval number 2018-314). Participant for all images have informed consent that he knew the risks and agreed to participate in the research.

TPO-Ab plays a role in arterial remodeling in patients with intracranial stenosis.

BACKGROUND AND AIMS: Intracranial stenosis (ICS), the common cause of ischemic stroke worldwide, is associated with a high risk of recurrent stroke. We aimed to investigate the relationship between arterial remodeling and antithyroid peroxidase-antibody (TPO-Ab) level in ICS and the effect of TPO-Ab level on the migration of vascular smooth muscle cells (VSMCs). METHODS: We analyzed data of mild-to-severe ICS patients with normal thyroid function who underwent high-resolution magnetic resonance imaging in our center. Vessel area (VA), lumen area, wall area and plaque size were assessed at the most narrowed lumen (MNL) and reference site, respectively. The remodeling index (RI) was defined as VAMNL/VAreference. Negative remodeling (NR) or non-NR was defined as RI ≤ 0.95 or > 0.95. A scratch-wound healing assay was also designed to analyze the impact of TPO-Ab level on migration of VSMCs, which were isolated from thoracic aorta segments of Sprague Dawley rats. RESULTS: A total of 88 patients were included. Patients with elevated TPO-Ab had smaller VA, wall area, plaque size and RI than those with normal level (p < 0.05). Elevated TPO-Ab was significantly associated with NR after adjusting for demographic and vascular risks (odds ratio 10.629, 95% confidence interval, 1.842-61.327, p = 0.008). The rate of VSMCs migration was significantly increased after culture with TPO-Ab (TPO-Ab 1 µg/ml vs. Mock, 29.8% vs. 12.0%, p < 0.01). CONCLUSIONS: Elevated TPO-Ab in ICS patients was related to NR. TPO-Ab could promote VSMCs migration, which might be involved in the NR of intracranial artery.

Role of Whole-exome Sequencing in Phenotype Classification and Clinical Treatment of Pediatric Restrictive Cardiomyopathy.

BACKGROUND: Restrictive cardiomyopathy (RCM) is the least common cardiomyopathy in which the walls are rigid and the heart is restricted from stretching and filling properly. Cardiac troponin I (cTnI) mutation-caused myofibril Ca2+ hypersensitivity has been shown to be associated with impaired diastolic function. This study aimed to investigate the linkage between the genotype and clinical therapy of RCM. METHODS: Five sporadic pediatric RCM patients confirmed by echocardiography were enrolled in this study. Whole-exome sequencing (WES) was performed for the cohort to find out candidate causative gene variants. Sanger sequencing confirmed the WES-identified variants. RESULTS: TNNI3 variants were found in all of the five patients. R192H mutation was shared in four patients while R204H mutation was found only in one patient. Structure investigation showed that the C terminus of TNNI3 was flexible and mutation on the C terminus was possible to cause the RCM. Catechins were prescribed for the five patients once genotype was confirmed. Ventricular diastolic function was improved in three patients during the follow-up. CONCLUSIONS: Our data demonstrated that TNNI3 mutation-induced RCM1 is the most common type of pediatric RCM in this study. In addition, WES is a reliable approach to identify likely pathogenic genes of RCM and might be useful for the guidance of clinical treatment scheme.

Balloon Angioplasty as a Modality to Treat Children with Pulmonary Stenosis Secondary to Complex Congenital Heart Diseases.

BACKGROUND: Pulmonary stenosis is common in children with complex congenital heart diseases. Proper management of this problem, especially postoperatively, is still controversial. This study was designed to assess the rate and determinants of success or failure of balloon angioplasty for such lesions. METHODS: Clinical and hemodynamic data from 40 pediatric patients (24 boys and 16 girls) with complex congenital heart diseases who underwent balloon angioplasty were reviewed retrospectively from January 2012 to December 2016. Patients were divided into four groups according to the site of stenosis, which included pulmonary valve stenosis (PVS), valved conduit stenosis, pulmonary artery stenosis (PAS), and supravalvular pulmonary stenosis (SVPS). Success rates were calculated according to defined criteria for initial success and favorable clinical impacts, and comparison between the successful subgroup and the unsuccessful subgroups was analyzed. RESULTS: Grouped by the site of stenosis, initial success rates varied from 40.0% to 52.4% with the greatest success being seen in the PVS group, followed by the PAS group and SVPS group. In the PVS group and the PAS group, there was no statistical difference among age at dilation, postoperative interval, balloon/stenosis ratio, or pressure gradient predilation between the successful and the unsuccessful subgroups. Favorable clinical impacts included success rates of balloon angioplasty in the SVPS group, which was best (100%), followed by the PVS group (90.9%) and the PAS group (85.7%). There were a total of two transient complications (5.0%). CONCLUSIONS: Balloon angioplasty was proven to be a safe and useful modality in children with complex congenital heart diseases and postoperative pulmonary stenosis, which should be the initial therapeutic modality in selected patients.

Cerebral blood flow evaluation of intensive rosuvastatin therapy in stroke/transient ischemic attack patients with intracranial arterial atherosclerotic stenosis study: Rationale and design.

INTRODUCTION: The risk of recurrent stroke is high in patients with intracranial atherosclerotic stenosis (ICAS). Statin use has been demonstrated to decrease the incidence of stroke by reducing atherosclerotic plaque burden. However, its effect on the hemodynamic situation and cerebral perfusion status has not yet been validated. With the use of computed tomography perfusion (CTP), we aim to evaluate the impact of Rosuvastatin on cerebral hemodynamic changes, as well as the downstream perfusion. METHOD: Cerebral bood flow evaluation of intensive rosuvastatin therapy in patients with intracranial arterial atherosclerotic stenosis (CEIRIS) is a single-center, prospective, randomized, parallel-group, and open-label trial, and it will include 50 participants as estimated. Patients with moderate to severe (50%-99%) ICAS are randomized 1:1 to 10 mg/day or 20 mg/day Rosuvastatin and followed every 13 weeks until 52 weeks. The primary outcome for the trial is the change in the relative regional cerebral blood flow evaluated by CTP after 52 weeks of Rosuvastatin treatment. The secondary outcomes are cerebral blood volume, change in the degree of stenosis of the target vessel and lipid parameters. CONCLUSION: The CEIRIS trial about the effects of statin on the temporal hemodynamic progression of ICAS may extend our understanding of the basic pathophysiology and mechanisms of stroke in ICAS patients.

Value of the Ratio of Occluder Versus Atrial Septal Length for Predicting Arrhythmia Occurrence after Transcatheter Closure in Children with Ostium Secundum Atrial Septal Defect.

BACKGROUND: Transcatheter occlusion has been applied to treat ostium secundum atrial septal defect (OS ASD) since 1997. During the clinical practice, several postoperative complications including arrhythmia have been reported. This study aimed to evaluate the value of the ratio of atrial septal occluder (ASO) versus atrial septal length (ASL) for predicting arrhythmia occurrence after transcatheter closure in children with OS ASD. METHODS: Six hundred and fifty-one children diagnosed with OS ASD underwent occlusion procedures after completing routine examinations. The onsets and types of arrhythmia both during and after the occlusion procedures were monitored. Treatments were given based on the individual types of arrhythmia. The binary logistic regression analysis and receiver-operating characteristic (ROC) curve were used in the analysis of value of the ratio of ASO/ASL for predicting postoperative arrhythmia occurrence. RESULTS: Transcather occlusions were conducted in 651 children, among whom 7 children had different types and degrees of arrhythmia, with an incidence of 1.1%. The types of arrhythmia included sinus bradycardia, atrial premature beats, bundle branch block, and different degrees of atrioventricular block. Normal electrocardiograph findings were resumed in these 7 patients following active therapies such as corticoids, nutrition, and surgeries. The binary logistic regression and ROC analysis suggested that the ratio of ASO/ASL exhibited an intermediate predictive value for predicting arrhythmia occurrence after occlusion procedures. A cut-off value of 0.576 in the ratio provided a sensitivity of 87.5% and a specificity of 76.2% with an area under the ROC curve of 0.791 (95% confidence intervals, 0.655-0.926; P < 0.05) in predicting arrhythmia occurrence after the closure procedures. CONCLUSIONS: The ratio of ASO/ASL might be a useful index for predicting arrhythmia occurrence after closure procedures in children with OS ASD.

Two congenital coronary abnormalities affecting heart function: anomalous origin of the left coronary artery from the pulmonary artery and congenital left main coronary artery atresia.

BACKGROUND: The anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) and congenital left main coronary artery atresia (CLMCA-A) are two kinds of very rare coronary heart diseases which affect heart function profoundly. This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients. METHODS: From April 1984 to July 2012, in Beijing Anzhen Hospital, 23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A. We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation, diagnosis, and treatments of these two kinds of congenital coronary abnormalities. RESULTS: The 23 patients (13 males and 10 females, aged ranging from 2.5 months to 65 years) identified with ALCAPA were classified into infantile type (age of onset younger than 12 months, 16 cases) and adult type (age of onset older than 12 months, 7 cases). Four patients were diagnosed with CLMCA-A (three males and one female, aged ranging from 3 months to 2 years). The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection, heart failure, dyspnea, feeding intolerance, diaphoresis, and failure to thrive. And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis, dilated cardiomyopathy, and acute myocardial infarction. As for the adult-type ALCAPA, cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease, myocarditis, or patent ductus arteriosus. In ECG examination: Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6, especially in lead avL. However, ECG of adult-type ALCAPA lacked distinct features. In chest radiography: pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A, while pulmonary artery segment dilation was more common in adult type. In echocardiography, the common features of infantile-type ALCAPA and CLMCA-A included left ventricular enlargement, left ventricular systolic function normal or mildly reduced in CLMCA-A or significantly reduced in ALCAPA, and moderate to large mitral valve. It was performed in 9 of 23 cases of ALCAPA and showed the origin of the dilated right coronary artery (RCA) from the right sinus of the aortic root and absence of LCA origin in angiography. After opacification of RCA, reverse flow in the LCA and pulmonary artery was visualized through coronary artery collateral circulation. Angio was performed in three of the four cases of CLMCA-A and showed left main coronary artery was a blind end, with diameter of only 1.1-2.0 mm. Treatment and prognosis: 21 patients with ALCAPA had cardiac surgery and 6 of them died postoperatively. Fifteen postoperative patients survived without overt symptoms within the follow-up period of 6-166 months (median 17 months). As for treatment of CLMCA-A, four patients took digoxin and diuretics without undergoing cardiac surgery. Their clinical symptoms improved during the close follow-ups. CONCLUSIONS: ALCAPA and CLMCA-A are two rare coronary artery abnormalities that affect cardiac function in infants and children. In younger patients with cardiomegaly and heart dysfunction these two congenital coronary diseases should be noticed.

[Congenital atresia of left main coronary artery in 4 children: case report and literature review].

OBJECTIVE: To investigate the clinical manifestations and treatment of congenital atresia of the left main coronary artery (CLMCA-A). METHOD: Four patients were diagnosed to have CLMCA-A from June 2010 to June 2012 in Beijing Anzhen Hospital. Clinical manifestations, ultrasound, ECG and angiographic characteristics were analyzed and summarized. RESULT: Of the 4 cases, age of onset was 3 months to 2 yrs. Three cases were diagnosed by angiography, and 1 case by CTA . All 4 cases had chronic heart failure symptoms and signs, such as sweating, shortness of breath, easily choked by milk, predispose to pneumonia, activity intolerance. ECG showed abnormal Q wave and other ischemic signs such as ST-T segment depression. Ultrasonography showed left ventricular enlargement, left ventricular systolic function was normal or slightly reduced, and there was moderate to large amount of mitral valve regurgitation. Left ventricular trabeculations increased. Coronary collateral circulation increased. Left coronary artery appeared to be slender and disconnected with left coronary artery sinus. Aortic root angiography was the golden diagnostic standard. Angiography was performed in 3 patients and showed that left main coronary artery had a blind end, diameter 1.1-2.0 mm. The right coronary artery was found rising from the right coronary sinus and visible on coronary collateral circulation. Contrast agent developing sequence: right coronary artery-collateral vessels-left coronary artery distal branches-left main coronary artery. CTA exam was performed in 2 cases and in 1 case the diagnoses was confirmed. All the 4 patients are currently in the close follow-up, digoxin and diuretics were taken everyday and clinical symptoms were improved. CONCLUSION: CLMCA-A is not rare, its clinical manifestations should be differentiated from those of cardiomyopathy, endocardial fibroelastosis, congenital valvular disease and abnormal left coronary artery originating from pulmonary artery etc. For pediatric patients with cardiac enlargement, abnormal heart function, mitral valve regurgitation etc, attention must be paid to consider the developmental abnormality of coronary artery, particularly the CLMCA-A diagnosis.

Prolonged total isovolumic time is related to reduced long-axis functional recovery following valve replacement surgery for severe aortic stenosis.

BACKGROUND: The left ventricular (LV) long axis (Lax) function is very sensitive in documenting myocardial abnormalities in aortic stenosis (AS). We hypothesized that Lax recovery after aortic valve replacement (AVR) is related to the extent of cavity dyssynchrony measured by total isovolumic time (t-IVT). METHODS: A consecutive 107 patients (aged 70 ± 7 years, 70 male) with severe AS and Lax impairment were studied. T-IVT was measured before and after AVR. Reduced Lax function and its post-operative recovery were defined as mitral annular plane systolic excursion (MAPSE) ≦10 mm and an increase of MAPSE >10%, respectively. RESULTS: LV function improved (EF: 43 ± 8 to 48 ± 10%; MAPSE: 7.9 ± 1.0 to 11.0 ± 2.4 mm) and t-IVT shortened (9.7 ± 3.7 to 7.0 ± 2.8s/min, p<0.01 for all) after AVR. Sixty-five (61%) patients had Lax recovery after a median of 32-month follow-up. Univariate predictors were LV size, LA dimensions, the presence of restrictive LV filling and prolonged t-IVT. Only LV end-systolic dimension, restrictive filling and t-IVT (OR 0.61, 95% CI 0.47-0.79, p<0.01) were independent predictors. A pre-operative t-IVT ≦ 9.3s/min was 81% sensitive and 63% specific in predicting Lax recovery (AUC 0.81, p<0.001). The prevalence of CAD or concomitant CABG were similar in 2 patient groups with different t-IVT. CONCLUSIONS: Lax recovery was evident in the majority of AS patients after AVR. The lower prevalence of Lax recovery seen in patients with prolonged t-IVT suggests that dyssynchrony may play an important role in the process of adverse LV remodeling.

Anomalous origin of the left coronary artery from the pulmonary artery in children: 15 years experience.

This study aimed to illustrate the experience of treating children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). The clinical data for 19 children with ALCAPA admitted to Beijing Anzhen Hospital from August 1993 to June 2009 were reviewed. According to the data, 47.4% (9/19) of the patients had a misdiagnosis of endocardial fibroelastosis, and 15.8% (3/19) had a misdiagnosis of dilated cardiomyopathy. Electrocardiography showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6 of 18 patients, especially in lead avL. The ratio of proximal right coronary artery diameter to aotic root diameter exceeded 0.20 for 15 of 16 patients. Apical ventricular aneurysm or aneurysmal dilation (52.6%,10/19), enhanced echogenicity of papillary muscles (84.2%, 16/19), and increased coronary collaterals (78.9%, 15/19) were detected frequently during echocardiography. A total of 18 patients underwent cardiac surgery including left coronary artery (LCA) ligation for 1 patient (5.6%), LCA ligation plus coronary artery bypass grafting for 1 patient (5.6%), Takeuchi operation for 7 patients (38.9%), and LCA reimplantation for 9 patients (50.0%). Five patients died in the hospital, and the remainder were asymptomatic during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed, and left ventricular systolic function and size returned to normal with alleviation of mitral insufficiency. The clinical features of ALCAPA are helpful for determining an accurate diagnosis. This anomaly can be treated successfully by several types of operations with good prognosis.

Clinical features and long-term prognosis of patients with anomalous origin of the left coronary artery from the pulmonary artery.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart anomaly. We aimed to illustrate the clinical features and long-term prognosis of patients with ALCAPA. METHODS: Twenty three patients (13 males and 10 females, ages ranging from 2.5 months to 65 years) identified as ALCAPA in Beijing Anzhen Hospital from April 1984 to June 2009 were divided into two groups, based on the age of onset: group 1 (≤ 12 months, n = 16) and group 2 (> 12 months, n = 7). RESULTS: Fifty six point three percent of patients in group 1 had been misdiagnosed as endocardial fibroelastosis (9/16), 18.8% as dilated cardiomyopathy (3/16) and 6.3% as myocardial infarction (1/16). Patients in group 2 were usually diagnosed as coronary heart disease, myocarditis, or patent ductus arteriosus. Electrocardiography in group 1 revealed abnormal Q waves with T wave inversion in leads I, avL, V(4)-V(6), especially in lead avL (deep and wide Q wave); but no specific manifestations in group 2. A higher percentage of patients in group 1 had cardiomegaly on chest radiograph (86.7% vs. 33.3%, P = 0.031), while pulmonary artery protrusion was more common in group 2 (26.7% vs. 83.3%, P = 0.046). Lower left ventricular ejection fraction (LVEF) was present in group 1 than in group 2 ((48.5 ± 11.5)% vs. (65.0 ± 6.1)%, P < 0.001). Apical ventricular aneurysm (62.5% vs. 0%, P = 0.007), enhanced echogenicity of papillary muscles (87.5% vs. 28.6%, P = 0.011) and endocardial thickening (93.8% vs. 14.3%, P < 0.001) were more frequent in group 1 than in group 2. The ratio of the proximal right coronary artery (RCA) diameter to the aortic root diameter exceeded 0.14 in all cases, more prominent in group 2 (0.26 ± 0.05 vs. 0.33 ± 0.03, P = 0.009). Increased coronary artery collaterals within the interventricular septum were detected in 18 patients (78.3%) by Doppler imaging. Twenty one patients underwent cardiac surgery, including left coronary artery (LCA) ligation (1/21), LCA ligation plus coronary artery bypass grafting (1/21), Takeuchi operation (7/21), and LCA reimplantation surgery (12/21). Four patients underwent concomitant mitral valve repair and one received mitral valve replacement. Aneurysm resection was performed in 3 cases. Six patients died in hospital after surgery, and the rest of the cohort had no overt symptoms during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed or disappeared, and the LVEF and left ventricle size returned to normal range with alleviation of mitral insufficiency. CONCLUSIONS: The accurate diagnosis of ALCAPA can be made with serial diagnostic methods. ALCAPA can be successfully treated with several types of cardiac surgery, and surgeries of establishing two-coronary-artery circulation are the preferred operations nowadays, with good long-term prognosis.

Early and long-term survival after aortic valve replacement in septuagenarians and octogenarians with severe aortic stenosis.

OBJECTIVE: To evaluate the predictors for mortality following aortic valve replacement (AVR) in elderly patients with aortic stenosis (AS). METHODS: 112 consecutive elderly AS patients (aged 77+/-2 years) with AVR between 1998 and 2003 were studied. Clinical and echocardiographic data of LV function were recorded before and 46 months after AVR. Results were compared with 72 younger patients (aged 60+/-1 years). Outcome measures were 30-day and long-term all cause mortalities. RESULTS: Elderly patients had higher NYHA class, more frequent atrial fibrillation, coronary artery disease, emergency operation and use of bioprosthetic valves. They also had shorter E-wave deceleration time (DT) and larger left atria (p<0.05 for all). 30-day mortality was 12% vs 4% (Log Rank x(2)=3.02, p=0.08) and long term mortality was 18% vs 7% (Log Rank x(2)=4.38, p=0.04) in two groups respectively. Age was not related to mortality after adjustment for other variables. Among all variables, anemia (OR 4.20, CI: 1.02-6.86, p=0.04), cardiopulmonary bypass (CPB) time (OR 1.02, CI 1.01-1.04, p<0.01), significant prosthesis patient mismatch (PPM) (OR 5.43, CI 1.04-18.40, p<0.05) were associated with 30-day mortality in elderly patients. Their long-term mortality was related to CBP time (OR 1.02, CI 1.00-1.05, p=0.04), PPM (OR 4.64, CI 1.33-16.11, p=0.02) and raised left atrial pressure: DT (OR 0.94, CI 0.84-0.99, p=0.03) and pulmonary arterial systolic pressure (OR 1.12, CI 1.03-1.19, p<0.001). CONCLUSION: Peri-operative AVR survival is encouraging. While pre-operative anemia and a longer CBP time determine early mortality, long term mortality is related to PPM, LV diastolic dysfunction and secondary pulmonary hypertension.

Predictors of survival after aortic valve replacement in patients with low-flow and high-gradient aortic stenosis.

AIMS: To identify predictors of survival following aortic valve replacement (AVR) in patients with low-flow and high-gradient aortic stenosis (AS). METHODS AND RESULTS: Eighty-six patients (aged 71 +/- 10 years) with severe AS [aortic valve mean pressure gradient >40 mmHg or valve area <1.0 cm(2)] and left ventricular (LV) dysfunction [ejection fraction (EF) <50%] underwent AVR. Cox proportional hazards were used to identify independent clinical and echocardiographic predictors of mortality. Operative (30-day) mortality was 10%. Peri-operative mortality was associated with lower mean LVEF, higher mitral E:A ratio, peak systolic pulmonary artery pressure (PSPAP), and serum creatinine (by 12%, 2.3, 28 mmHg, and 74 mmol/L, respectively, all P < 0.001), NYHA class III-IV (100 vs. 65%), concomitant CABG (89 vs. 55%), urgent surgery (78 vs. 35%), and longer bypass-time (by 28 min, all P < 0.05). Mortality at 4 years was 17%. Univariate predictors [hazard ratio (HR)] of 4-year mortality were: lower EF (HR 0.68 per % increase, P < 0.001), presence of restrictive LV filling (HR: 3.52, P < 0.001), raised PSPAP (HR: 1.07, P < 0.001), and CABG (HR: 4.93, P = 0.037). However, only low EF (<40%, HR 0.74, P = 0.030), the presence of restrictive filling (HR 1.77, P = 0.033), and raised PSPAP (>45 mmHg, HR 2.71, P = 0.010) remained as independent predictors after multivariate analysis. CONCLUSION: The severity of pre-operative systolic and diastolic LV dysfunction is the major predictor of mortality following AVR for low-flow and high-gradient AS.

Biochemical metabolic changes assessed by 31P magnetic resonance spectroscopy after radiation-induced hepatic injury in rabbits.

AIM: To compare the features of biochemical metabolic changes detected by hepatic phosphorus-31 magnetic resonance spectroscopy ((31)P MRS) with the liver damage score (LDS) and pathologic changes in rabbits and to investigate the diagnostic value of (31)P MRS in acute hepatic radiation injury. METHODS: A total of 30 rabbits received different radiation doses (ranging 5-20 Gy) to establish acute hepatic injury models. Blood biochemical tests, (31)P MRS and pathological examinations were carried out 24 h after irradiation. The degree of injury was evaluated according to LDS and pathology. Ten healthy rabbits served as controls. The MR examination was performed on a 1.5 T imager using a (1)H/(31)P surface coil by the 2D chemical shift imaging technique. The relative quantities of phosphomonoesters (PME), phosphodiesters (PDE), inorganic phosphate (Pi) and adenosine triphosphate (ATP) were measured. The data were statistically analyzed. RESULTS: (1) Relative quantification of phosphorus metabolites: (a) ATP: there were significant differences (P < 0.05) (LDS-groups: control group vs mild group vs moderate group vs severe group, 1.83 +/- 0.33 vs 1.55 +/- 0.24 vs 1.27 +/- 0.09 vs 0.98 +/- 0.18; pathological groups: control group vs mild group vs moderate group vs severe group, 1.83 +/- 0.33 vs 1.58 +/- 0.25 vs 1.32 +/- 0.07 vs 1.02 +/- 0.18) of ATP relative quantification among control group, mild injured group, moderate injured group, and severe injured group according to both LDS grading and pathological grading, respectively, and it decreased progressively with the increased degree of injury (r = -0.723, P = 0.000). (b) PME and Pi; the relative quantification of PME and Pi decreased significantly in the severe injured group, and the difference between the control group and severe injured group was significant (P < 0.05) (PME: LDS-control group vs LDS-severe group, 0.86 +/- 0.23 vs 0.58 +/- 0.22, P = 0.031; pathological control group vs pathological severe group, 0.86 +/- 0.23 vs 0.60 +/- 0.21, P = 0.037; Pi: LDS-control group vs LDS-severe group, 0.74 +/- 0.18 vs 0.43 +/- 0.14, P = 0.013; pathological control group vs pathological severe group, 0.74 +/- 0.18 vs 0.43 +/- 0.14, P = 0.005) according to LDS grading and pathological grading, respectively. (c) PDE; there were no significant differences among groups according to LDS grading, and no significant differences between the control group and experimental groups according to pathological grading. (2) The ratio of relative quantification of phosphorus metabolites: significant differences (P < 0.05) (LDS-moderate group and LDS-severe group vs LDS-control group and LDS-mild group, 1.94 +/- 0.50 and 1.96 +/- 0.72 vs 1.43 +/- 0.31 and 1.40 +/- 0.38) were only found in PDE/ATP between the moderate injured group, the severe injured group and the control group, the mild injured group. No significant difference was found in other ratios of relative quantification of phosphorus metabolites. CONCLUSION: (31)P MRS is a useful method to evaluate early acute hepatic radiation injury. The relative quantification of hepatic ATP levels, which can reflect the pathological severity of acute hepatic radiation injury, is correlated with LDS.

A case of primary malignant fibrous histiocytoma of the pancreas: CT and MRI findings.

Primary malignant fibrous histiocytoma (MFH) of the pancreas is rare and a distinct clinical entity. We report a case of recurrence of pancreatic MFH with computed tomography (CT) and magnetic resonance imaging (MRI) findings. A 67-year-old man presented with a history of decreased body weight over the past 6 mo. Abdominal CT revealed a large, multilocular cystic mass in the head of the pancreas with obvious atrophy in the body and tail of the pancreas. After 6 mo postoperatively, MRI demonstrated a recurrent large mass in the primary area of the head of the pancreas. The lesion was heterogeneous, hypointense to the liver on T1-weighted imaging, and heterogeneously hyperintense to the liver with a hypointense area in the central part of the tumor on fat-saturated T2-weighted imaging. Contrast-enhanced T1-weighted imaging demonstrated a large multilocular cystic mass with a cystic wall, fibrous septa and enhancement of solid components. To the best of our knowledge, this is the first report on recurrence of primary MFH of the pancreas, and the first with MRI findings.