Prognostic value of left atrial mechanics in cardiac light-chain amyloidosis with preserved ejection fraction: a cohort study.

BACKGROUND: Light-chain amyloidosis is a plasma cell disorder associated with poor outcomes, especially when the heart is involved. The characteristics of left atrial (LA) function and its prognostic implications in cardiac amyloidosis (CA) have not been fully investigated. METHODS: Between April 2014 and June 2019, 93 patients with a diagnosis of CA, normal left ventricular ejection fraction (LVEF) and sinus rhythm were included. Their clinical, baseline echocardiographic and follow-up data were investigated. LA function, including LA strain and strain rate, was assessed using 2D speckle tracking echocardiography in different LA functional phases. RESULTS: Among all patients, 38 (40.9%) died. Multivariate Cox regression analyses showed that LA mechanics regarding LA reservoir and booster pump functions were independent predictors for overall survival. Traditional echocardiographic parameters for LA structure like LA volume index and LA width were not associated with mortality. Moreover, LA strain and strain rate in reservoir and contractile phases improved the discrimination and goodness of fit of the conventional prognostic model, the Mayo criteria 2004 and 2012, in our study population. Decreased LA mechanics were associated with impaired left ventricular (LV) systolic and diastolic function, and LA reservoir and contractile functions were associated with LA structure. CONCLUSIONS: Assessment of LA reservoir and contractile functions via 2D speckle tracking echocardiographic LA mechanical indices provide clinical and prognostic insights into cardiac light-chain amyloidosis patients, especially those with preserved EF and sinus rhythm. Emphasizing the monitoring of LA function may be beneficial for the prognosis prediction of CA.

Subclinical left ventricular systolic dysfunction detected by two-dimensional speckle tracking echocardiography in patients with pheochromocytoma and paraganglioma and preserved ejection fraction.

BACKGROUND: Excessive catecholamine leads to pressure overload and left ventricular (LV) remodeling. The goal of this study was to explore subclinical LV systolic dysfunction and the mechanism of preserved left ventricular ejection fraction (LVEF) in patients with pheochromocytoma and paraganglioma using two-dimensional speckle tracking echocardiography. METHODS: A total of 48 patients with pheochromocytoma and paraganglioma and preserved LVEF and 38 age- and gender-matched volunteers were studied. Echocardiographic parameters including LVEF, and global peak longitudinal and circumferential strains were measured. The correlation between echocardiographic parameters and blood pressure as well as biochemical parameters was analyzed. RESULTS: LVEF was similar between patients with pheochromocytoma and paraganglioma and controls. The amplitude of LV longitudinal strain was decreased, and the amplitude of LV circumferential strain was increased in the pheochromocytoma and paraganglioma group (P = .003 and P = .009). LV mass index and blood pressure were positively correlated with 24-hour urinary norepinephrine (r = .696, P < .0001; r = .470, P = .0007). The amplitude of LV longitudinal strain reduced with increase in blood pressure, 24-hour urinary norepinephrine and LV mass index (r = -.305, P = .035; r = -.506, P = .0002; r = -.680, P < .0001). CONCLUSIONS: This study revealed that excessive norepinephrine in pheochromocytoma and paraganglioma was associated with increased blood pressure and LV mass. The LV longitudinal strain was decreasing with increase in blood pressure and LV mass index. The enhanced LV circumferential strain might be the mechanism of compensation to maintain the normal LVEF in these patients.

Increased Urokinase-Type Plasminogen Activator Receptor Expression on Circulating Monocytes Is Correlated with Clinical Instability and Long-Term Adverse Cardiac Events in Patients with Coronary Artery Disease.

OBJECTIVES: This study sought to investigate the clinical correlates and prognostic roles of urokinase-type plasminogen activator receptor (uPAR) on circulating monocytes in patients with coronary artery disease (CAD). METHODS: 263 angina patients were included in this study. The percentage of uPAR expressing monocytes (PUEM) and the mean fluorescence intensity (MFI) index of uPAR were measured using flow cytometry. Patient follow-up was on average 604 days. Major adverse cardiac events (MACE) were defined as a composite of cardiac death, reinfarction, acute heart failure and hospitalization for revascularization. RESULTS: The PUEM and MFI index levels were significantly more elevated in acute coronary syndrome patients than in stable ones. uPAR expressions on circulating monocytes at admission were correlated to inflammatory biomarkers and myocardial necrosis. Logistic regression analysis revealed that PUEM ≥15% (OR 21.96, 95% CI 7.31-65.98, p < 0.001) and uPAR MFI index ≥3.00 (OR 3.54, 95% CI 1.18-10.59, p = 0.024) were independent determinants of clinical instability in patients with CAD. When followed up, a high PUEM level at admission was an independent prognostic parameter for long-term MACE (HR 3.99, 95% CI 1.31-12.11, p = 0.015). CONCLUSIONS: uPAR expression on circulating monocytes is associated with clinical instability and myocardial necrosis and independently predicts the risk of MACE in patients with CAD.

Correlation between severity of adolescent idiopathic scoliosis and pulmonary artery systolic pressure: a study of 338 patients.

PURPOSE: Study of patients with adolescent idiopathic scoliosis. OBJECTIVE: To examine the correlation between pulmonary arterial pressure and coronal Cobb angle of idiopathic scoliosis. METHODS: A total of 338 patients (82.8 % female) with idiopathic scoliosis (average age 15.6 years; range 14-20 years) were included. Preoperatively, the coronal Cobb angle of curvature and the apex location and direction were determined from radiographic records. Tricuspid regurgitation velocity (TRV) and inferior vena cava diameter were also measured using Doppler echocardiography. Pulmonary arterial systolic pressure (sPAP) was calculated from the TRV according to the modified Bernoulli equation and correlations between sPAP and the features of scoliosis were identified by statistical analysis. RESULTS: Among the 338 patients, there were 305 thoracic curves, 276 (90.5 %) of which were right curves, and 265 thoracolumbar/lumbar curves. sPAP varied from 5.0 to 37.6 mmHg. Pulmonary hypertension could not be excluded in the case of one patient. A mild correlation (Spearman test, correlation coefficient = 0.187, P = 0.001) between sPAP and coronal Cobb angle of the main thoracic (MT) curves was identified. Correlations between sPAP and the degree of other curves were not significant. Patients with sPAP >20 mmHg also had larger thoracic curve angles (mean MT 42.16° vs. 52.45°; U test, P = 0.002). There were no differences in sPAP levels between patients with right and left thoracic curves. CONCLUSIONS: A mild positive correlation was identified between sPAP and the coronal Cobb angle of the MT curves. There was no relationship between sPAP and the direction of the curvature.

[Long-term efficacy and safety of adjuvant trastuzumab for HER2-positive early breast cancer].

OBJECTIVE: To explore the long-term efficacy and safety of adjuvant trastuzumab for HER2-positive early breast cancer. METHODS: The clinicopathological data were collected 31 HER2-positive early breast cancer patients on the 1/2-year adjuvant therapy of trastuzumab at our hospital from October 2001 to October 2003. And the disease-free survival, recurrence-free survival, overall survival and safety were respectively analyzed. SPSS 13.0 software was used for statistical analysis. RESULTS: During a median follow-up period of 9.3 years, 6 cases had recurrence, there were 5 cases of second primary cancer and 4 patients died. In total, the 5 and 10-year recurrence-free survival rates were 83.3% and 80.0%, the 5 and 10-year disease-free survival rates 80.6% and 57.3% and the 5 and 10-year overall survival rates 96.8% and 87.1% respectively. The 10-year recurrence-free survival rate of 13 patients with ER and/or PR positive and 18 ER/PR negative patients were 100.0% and 64.7% (χ² = 5.44, P = 0.019) and 10-year overall survival rate 100% and 77.8% respectively (χ² = 3.163, P = 0.075). Trastuzmab was well-tolerated when used as adjuvant treatment. There was no occurrence of cardiac events. CONCLUSION: Adjuvant trastuzmab has definite efficacies and excellent safety in the treatment of HER2-positive breast cancer patients. And there was no long-term cumulative cardiac toxicity.

[Long-term effects of arteriovenous fistula on cardiac structure and function in non-diabetic hemodialysis patients].

OBJECTIVE: To evaluate the long-term effects of arteriovenous fistula (AVF) on heamodynamic changes and cardiac structure and function in non-diabetic hemodialysis patients. METHODS: Data were collected from 50 non-diabetic hemodialysis patients (aged 18 to 60 years) who had used AVF as the vascular access. AVF flow (Qa), stoke volume (SV), cardiac output (CO), cardiac index (CI), central blood volume (CBV) and peripheral vascular resistance (PR) were measured using the ultrasound dilution technique. Echocardiography was performed in the second day after hemodialysis sessions to evaluate the influence of AVF on the cardiac structure and function. RESULTS: The cubic polynomial regression model best fit the relationships of Qa with SV, CO, and CI. CO and CI significantly increased and PR reduced when the Qa of AVF was more than 2.0 L/min(all P<0.05), and no statistical difference of CO, CI and PR in groups of Qa between 0.6-2.0 L/min and less than 0.6 L/min(all P>0.05). In different Qa groups, the grades of cardiac function (based on New York Heart Association classification) showed significant difference, among which the cardiac failure was significantly common when Qa >2.0 L/min(both P<0.05). Echocardiography showed the left atrium dimension, thickness of posterior wall and interventricular seprum of left ventricle, left ventricular end-systolic dimension (LVESD) and end-diastolic dimension (LVEDD), venae cava inferior, and pulmonary artery systolic pressure gradually increased when Qa increased, while the ejection fraction and fractional shortening reduced(all P<0.05). Notably, the changes of LVESD, LVEDD, and venae cava inferior with different Qa were statistically significant(all P<0.05). CONCLUSIONS: Long-term AVF remarkably affects the cardiovascular dynamics of non-diabetic hemodialysis patients. A cubic polynomial regression model best fits the relationship of AVF Qa with SV, CO, and CI. The cardiac adaptic changes after long-term AVF include the enlargement of left ventricle and the thickening of ventricular wall. The risk of cardiac failure significantly increases when the Qa of AVF is more than 2.0 L/min with much higher CO and lower PR.

[Ultrasonic characteristics of intravenous leiomyomatosis with intracardiac extension].

OBJECTIVE: To explore the sonographic characteristics of intraveous leiomyomatosis (IVL) with intracardiac extension and improve its diagnosis. METHODS: The clinical and sonographic data of 13 female patients with pathologically confirmed IVL with intracardiac extension who were treated in our hospital between 2002 and 2012 were retrospectively analyzed. These patients aged 44 years old (range: 38-49 years), and 10 of them were first-episode patients and the remaining 3 were recurrent patients. Eight patients had a history of hysterectomy for leiomyoma. RESULTS: The first-episode symptoms included exertional chest tightness and shortness of breath (n=5), abdominal distention and edema of low extermity (n=4), exertional palpitation of cardiac origin (n=3), and menorrhagia (n=1). Ultrasonography showed that all patients had isoechoic or hypoechoic tumors extended through the inferior vena cava into right heart chambers (62% in right atrium alone and 38% in right ventricle and atrium). Nine masses in right heart chamber (69.2%) were oval and 4 (30.8%) were serpentine, which were all with well-demarcated borders and most (80%) with heteroechogenic texture. Ten patients had hypoechoic or mixed echoic tumors in pelvic cavity or uterus, and 6 of them had abundant blood flow. CONCLUSIONS: IVL with intracardiac extension has certain sonographic characteristics. Ultrasonography is a valuable tool in the diagnosis of IVL with intracardiac extension.

Diagnosis, Genetics, and Management of 24 Patients With Cardiac Paragangliomas: Experience From a Single Center.

Context: Paragangliomas located within the pericardium represent a rare yet challenging clinical situation. Objective: The current analysis aimed to describe the clinical characteristics of cardiac paragangliomas, with emphasis on the diagnostic approach, genetic background, and multidisciplinary management. Methods: Twenty-four patients diagnosed with cardiac paraganglioma (PGL) in Peking Union Medical College Hospital, Beijing, China, between 2003 and 2021 were identified. Clinical data was collected from medical record. Genetic screening and succinate dehydrogenase subunit B immunohistochemistry were performed in 22 patients. Results: The median age at diagnosis was 38 years (range 11-51 years), 8 patients (33%) were females, and 4 (17%) had familial history. Hypertension and/or symptoms related to catecholamine secretion were present in 22 (92%) patients. Excess levels of catecholamines and/or metanephrines were detected in 22 (96%) of the 23 patients who have completed biochemical testing. Cardiac PGLs were localized with 131I-metaiodobenzylguanidine scintigraphy in 11/22 (50%), and 99mTc-hydrazinonicotinyl-tyr3-octreotide scintigraphy in 24/24 (100%) patients. Genetic testing identified germline SDHx mutations in 13/22 (59%) patients, while immunohistochemistry revealed succinate dehydrogenase (SDH) deficiency in tumors from 17/22 (77%) patients. All patients were managed by a multidisciplinary team through medical preparation, surgery, and follow-up. Twenty-three patients received surgical treatment and perioperative death occurred in 2 cases. Overall, 21 patients were alive at follow-up (median 7.0 years, range 0.6-18 years). Local recurrence or metastasis developed in 3 patients, all of whom had SDH-deficient tumors. Conclusion: Cardiac PGLs can be diagnosed based on clinical manifestations, biochemical tests, and appropriate imaging studies. Genetic screening, multidisciplinary approach, and long-term follow-up are crucial in the management of this disease.

[Association between cardiac function and intradialytic hypotension].

OBJECTIVE: To prospectively determinate the association of left ventricular systolic and diastolic function with intradialytic hypotension (IDH) in patients on maintenance hemodialysis. METHODS: Totally 115 patients with sinus rhythm were included in this study and divided into IDH group (n=29) and control group (n=86). The cardiac function was assessed by New York Heart Association (NYHA) classification before hemodialysis. Echocardiograms were performed in the next day after hemodialysis and the software of GE EchoPAC was used to estimate parameters of cardiac systolic and diastolic functions including ejection fraction, fractional shortening of left ventricular diameter, stroke volume (SV), cardiac output (CO), left ventricular mass index, Tei index, isovolumetric relaxation time, E-deceleration time, mitral inflow peak early diastolic velocity (E) to late diastolic velocities (A) ratio, and E to peak mitral annulus velocity (E/Em) ratio. RESULTS: The mean age (p=0.045) and the ratio of heart failure evaluated by the NYHA classification (p<0.01) were significantly higher in IDH group than those in control group. No difference was noted for gender, body mass index, duration of dialysis, body weight elevated rate and blood pressure between these two groups (all p>0.05). Left ventricular diameters at the end of diastolic phase, SV, and CO in IDH group were significantly lower than those in control group (all P<0.05), whereas no significant difference was found for EF, FS, Tei index, IVRT, EDT, E/A and E/Em ratio (all p>0.05). Multivariate logistic regression analysis showed that NYHA cardiac function was an independent predictor of IDH, and the risk of IDH increased by 1.134 times with incremental one grade of NYHA classification. CONCLUSIONS: IDH is a common complication of hemodialysis. Neither systolic dysfunction nor diastolic dysfunction of left ventricle evaluated in second day after hemodialysis affects its frequency, whereas the clinical cardiac function is an independent predictor of IDH.

[Infective endocarditis in patients with hypertrophic obstructive cardiomyopathy: five cases report].

OBJECTIVE: To analyze the clinical characteristics of infective endocarditis in patients with hypertrophic obstructive cardiomyopathy. METHODS: Clinical characteristics from 5 patients with infective endocarditis and hypertrophic obstructive cardiomyopathy hospitalized from January 2000 to December 2010 in our hospital were analyzed. RESULTS: Four patients were diagnosed with left ventricular outflow tract obstructive cardiomyopathy with outflow pressure gradient from 36 to 140 mm Hg (1 mm Hg = 0.133 kPa) and left atrial size 44 - 68 mm. Another patient was diagnosed as ventricular hypertrophic cardiomyopathy with significant right-ventricular outflow tract hypertrophy (30 mm), high pressure gradient (164 mm Hg) and enlarged right atrial (56 mm × 53 mm), there was a 17 mm × 8 mm vegetation on right-ventricular outflow tract in this patient. Blood cultures were positive for streptococcus viridans in all five patients, and enterococcus faecium was revealed in one aortic valve vegetation culture. Transthoracic echocardiogram was performed 2 - 4 times for each patient, the vegetations of two patients was detected only by transesophageal echocardiography. The mitral valve vegetation was detected in two patients, the aortic and mitral valve vegetations were detected in one patients, mitral and tricuspid vegetations in one patient and right ventricular outflow tract vegetation in one patient. The four hemodynamically stable patients were successfully treated with antibiotic therapy, one patient received urgent surgery (replacement of the aortic and mitral valve as well as septal myectomy). All patients recovered and follow-up (1 - 6 years) was available in 4 patients and no complication was observed. CONCLUSION: The risk of infective endocarditis complicating hypertrophic obstructive cardiomyopathy is the highest in patients with both outflow obstruction and marked valve insufficiency, these patients should receive prophylactic antibiotic therapy during procedures that predispose to infective endocarditis.

[Clinical and echocardiographic features of cardiac myxomas in 64 patients].

OBJECTIVE: To summarize the clinical and echocardiographic features of cardiac myxomas. METHODS: The medical records of patients with diagnosis of cardiac myxomas who hospitalized in our department from October 1985 to February 2011 were analyzed. RESULTS: A total of 64 patients were enrolled [40 female, the mean age was 2 - 77 (47 ± 17) years]. The main complaints were palpitation (n = 24, 38%), short breath (n = 23, 36%), fever (n = 13, 20%), chest tightness (n = 11, 17%), dizziness (n = 10, 16%), fatigue (n = 10, 16%), weight loss (n = 10, 16%), syncope (n = 9, 14%), edema (n = 8, 13%); and thrombus embolisms (n = 13, 20%), including stroke (n = 7, 11%) and periphery artery embolism (n = 6, 9%). The interval from symptoms onset to diagnosis (surgical removal) ranged from 1 day to 9 years (median: 3 months). Single myxoma was detected in 62 (97%) patients (58 in left atria, 2 in right atria and 2 in right ventricle) and multiple myxomas were found in 2 (3%) patients and one patient was diagnosed as Carney syndrome. The mean size of tumor assessed by echocardiography was (5.0 ± 1.8) cm × (2.9 ± 1.0) cm. All myxomas were surgically removed (54 patients received operation in our hospital and 10 patients were operated in other hospitals) and diagnosis was confirmed during operation and the mean myxoma size obtained from operation was (5.4 ± 1.6) cm × (3.6 ± 1.3) cm × (2.6 ± 1.2) cm (P > 0.05 vs. tumor size assessed by echocardiography). The locations of tumor stalks found by echocardiography were confirmed during surgery in most cases (97%). Incidence of NYHA class III diagnosis was more often in patients with right heart myxomas [3 cases (3/4)] than in patients with left atrium myxomas [17% (10/58), P < 0.05]. CONCLUSIONS: Clinical manifestations of cardiac myxomas were various and non-specific. Echocardiography remains the most valuable diagnosis tool for patients with cardiac myxomas.

Initiating ivabradine during hospitalization in patients with acute heart failure: A real-world experience in China.

BACKGROUND: Initiating ivabradine in acute heart failure (HF) is still controversial. HYPOTHESIS: Ivabradine might be effective to be added in acute but hemodynamically stable HF. METHODS: A retrospective cohort of hemodynamically stable acute HF patients was enrolled from January 2018 to January 2020 and followed until July 2020. The primary endpoints were all-cause mortality and rehospitalization for HF. Secondary endpoints included heart rate (HR), cardiac function measured by New York Heart Association (NYHA) class, and left ventricular ejection fraction (LVEF) and adverse events, which were compared between patients with or without ivabradine. RESULTS: A total of 126 patients were enrolled (50 males, median age 54 years, 81% with decompensated HF, median follow-up of 9 months). In patients treated with ivabradine, although baseline HRs were higher than the reference group (96 vs. 80 bpm), they were comparable after 3 months; more patients tolerated high doses of ß-blockers (27% vs. 7.9%), improved to NYHA class I function (55.6% vs. 23.8%) and exhibited normal LVEFs (37.8% vs. 14.3%) than the reference group (all p < .05). Ivabradine was associated with a significant reduction of rehospitalization for HF than the reference group (25.4% vs.61.9%), with longer event-free survival times (hazard ratio: 0.45, 95% confidence interval [CI]: 0.25-0.79), and was related with primary endpoints negatively (hazard ratio 0.51, 95% CI: 0.28-0.91) (all p < .05). CONCLUSION: In patients with acute but hemodynamically stable HF, ivabradine may significantly reduce HR, improve cardiac function, and reduce HF rehospitalization.

[Value of cardiac magnetic resonance imaging for the diagnosis of cardiac amyloidosis].

OBJECTIVE: To observe the clinical features and cardiac magnetic resonance imaging (CMR) characteristics of patients with endomyocardial biopsy (EMB)-proven cardiac amyloidosis (CA). METHODS: EMB proven CA patients underwent CMR examination from September 2006 to December 2010 were included. The findings of clinical manifestation, electrocardiogram, echocardiography and CMR were analyzed. RESULTS: Among the 18 patients with EMB verified CA, 5 patients underwent CMR. All 5 patients had heart failure symptoms and electrocardiogram was abnormal. Echocardiogram showed concentric left ventricular hypertrophy, granular appearance of the myocardium, left atrial enlargement and moderate to severe left ventricular diastolic dysfunction. CMR revealed increased thickness of the left ventricular wall (especially at the inter-ventricular septum), enlarged bilateral auricle, restricted left ventricular filling with normal or mild to moderate reduced systolic function. Pleural and pericardial effusions were observed in 2 patients. Abnormal late gadolinium enhancement (LGE) was detected in all 5 patients. CMR revealed different patterns of LGE. Left ventricular global subendocardial delayed gadolinium enhancement or transmural delayed gadolinium enhancement were found, and patients also showed line-, granular- or patchy-like enhancement. The degree and range of LGE paralleled the disease course and were consistent with electrocardiogram changes. CONCLUSIONS: As a noninvasive diagnostic tool, CMR is valuable in the diagnosis of CA. For patients with clinical suspicion of CA, CMR could be a helpful diagnostic tool, especially in the hospitals where EMB is not available.

[A clinical analysis of 25 cases of prosthetic valve endocarditis].

OBJECTIVE: To report the clinical characteristics of prosthetic valve endocarditis (PVE). METHODS: All 25 cases of definite PVE (Duke criteria) diagnosed at our hospital between January 1992 to December 2008 were retrospectively analyzed. Among them, 7 cases were pathologically confirmed and the others were clinically confirmed with either 2 major criteria or 1 major and ≥ 3 minor criteria. Their clinical characteristics, underlying heart diseases, previous heart operations, presenting manifestations, causative microbes, echocardiographic findings and prognosis, were studied. RESULTS: (1) Although most cases underwent valve transplantations for underlying heart diseases of rheumatic heart diseases and congenital heart diseases, 10 patients were complicated with infectious endocarditis (IE) prior to the operations, 4 of them were PVE. (2) Eleven of them developed PVE within 2 months postoperatively. Fever (100%), major vessel embolism (48%), and anemia (36%) were the most frequently manifestations. Fourteen cases (56%) had positive culture results with 15 causative pathogens, including 5 coagulase-negative Staphylococcus (CNS, 3 were methicillin-resistant coagulase-negative Staphylococcus, MRSCoN), 4 fungi, 2 Enterococcus faecalis, 2 Burkholderia cepacia, 1 Stenotrophomonas maltophilia, and 1 Streptococcus. (3) Prosthetic valve vegetations, periannular leakage, regurgitation, were the main echocardiographic findings. Transesophageal echocardiography (TEE) revealed 13 PVE who had no positive findings on previous transthoracic echocardiography (TTE). (4) Eighteen PVE (72%) developed peri-annular complications (12 leakage, 3 dehiscence, 2 abscesses, 1 fistula), major vessel embolism, congestive heart failure (16%) were frequently observed, 9 of the 17 patients died in hospital, in spite of intensive managements. CONCLUSIONS: PVE has a high mortality and is a severe complication for patients who underwent heart surgery. Its causative pathogen spectrum is quite different from that of native valve endocarditis. TTE is not sensitive for some PVE cases.

[Cardiovascular manifestations in Chinese patients with hypereosinophilic syndrome].

OBJECTIVE: To evaluate the cardiovascular involvements in Chinese patients with hypereosinophilic syndrome. METHOD: We respectively reviewed 149 inpatients with hypereosinophilic syndrome admitted to Peking Union Medical College Hospital and analyzed the cardiovascular involvements in these patients. RESULTS: Cardiac abnormalities was evidenced in 32.9% patients (49/149). The ratio of male vs female was 34:15. The average age of the patients was (41.3 ± 16.9) years and course of disease was (26.4 ± 72.3) months. Cardiovascular involvements included ST segment and/or T wave (ST-T) ischemic changes, arrhythmia, myocardial injury, cardiac thrombosis, pericardial effusion, pulmonary hypertension, valve disorder, vein or artery thrombosis. After glucocorticoid and/or chemotherapeutic agents and treatment for symptoms, 11 (22.4%) patients achieved remission but have recurrent attacks and 3 (6.1%) patients died from failure in treatment. The prognosis in patients with echocardiogram abnormalities were poorer than those only with electrocardiogram abnormalities (P < 0.05). CONCLUSIONS: Cardiovascular involvements are common in patients with hypereosinophilic syndrome and the manifestation of these involvement is various. Cardiovascular complications of HES are a major source of morbidity and mortality in these disorders.

[Electrocardiographic and echocardiographic features of patients with primary cardiac amyloidosis].

OBJECTIVE: To summarize the electrocardiography and echocardiography features of patients with cardiac amyloidosis (CA) diagnosed by endo-myocardial biopsy (EMB). METHODS: A total of 20 consecutive patients [7 men, mean age (50 ± 12) years] referred for EMB because of clinical suspicion of CA from September 2006 to October 2009 were included in the study. Primary CA was diagnosed in 11 out of 20 patients (55%) by EMB and biomarkers examination. The electrocardiography and echocardiography features were analyzed. RESULTS: The voltage of all the limb leads were low in the 11 CA patients [mean values of (0.33 - 0.51) mV], the incidence of low voltage and pseudo-infarction patterns were 45% and 45%, respectively. Concentric hypertrophy and normal left ventricular diameters were evidenced in all CA patients on echocardiography, left atrial enlargement (n = 10, 91%), granular/sparking appearance of the myocardium (n = 9, 82%) and moderate to large pericardial effusion (n = 7, 64%) as well as left ventricular systolic dysfunction (n = 8, 73%) were often presented in CA patients. CONCLUSIONS: The diagnosis of primary CA should be considered in patients with unknown origin of heart failure, concentric hypertrophy and normal left ventricular diameters with granular/sparking appearance of the myocardium or pericardial effusion presented on echocardiography and low voltage of limb leads or pseudo-infarction pattern presented on electrocardiography. EMB and serum (urine) biomarkers examinations should be then performed to confirm or exclude the diagnosis of CA.

[Clinico-pathological evaluation of restrictive cardiomyopathy].

OBJECTIVE: restrictive cardiomyopathy (RCM) is characterized by impairment of ventricular filling during diastole with preserved systolic function. The clinical and histopathological profile on endomyocardial biopsy of 25 consecutive patients with RCM was analyzed in this study. METHODS: twenty-five patients with diagnosis of RCM and underwent endomyocardial biopsy (EMB) were enrolled in the study. The clinical characteristics, electrocardiogram, serum chemistry, right heart catheter and cardiac pathology results were obtained. RESULTS: heart failure symptom was present in all 25 patients and left ventricular size and function were normal or near normal while serum brain natriuretic peptide (577 pg/ml) was moderately elevated. Right atrial and ventricular end-diastolic as well as pulmonary capillary wedge pressures derived from right heart catheter examination were increased. Amyloid deposition were evidenced in 16 and eosinophilic myocarditis in 2 patients upon pathological examination of EMB. In the remaining 7 patients, 3 were diagnosed idiopathic RCM, 2 were diagnosed as amyloidosis by biopsy from non-cardiac tissue and etiology remained unknown in 2 patients. Thus, conclusive diagnosis was made on EMB samples in 84% (21/25) patients of RCM. CONCLUSION: RCM may result from various local and systemic disorders. EMB is helpful for identifying the underlying etiology.

Usefulness of Myocardial Strain and Twist for Early Detection of Myocardial Dysfunction in Patients With Autoimmune Diseases.

Cardiac involvement in autoimmune diseases (AD) is common but underdiagnosed due to a lack of sensitive imaging methods. We aim to evaluate the characteristics of left ventricular (LV) systolic dysfunction in patients with AD using deformational parameters from 2-dimensional speckle-tracking echocardiography (STE). We retrospectively enrolled 86 AD patients and 71 healthy controls. All subjects underwent transthoracic echocardiography and STE to analyze LV strain and twist. A twist-radial displacement loop was constructed to investigate the relation between LV contractility and dimension. In AD patients, 68 had preserved LV ejection fraction (EF ≥ 50%), and 18 had reduced LVEF (EF < 50%). The patients with preserved LVEF exhibited significantly lower values of global longitudinal, circumferential, and radial strain than controls (-19.11 ± 4.18 vs -21.49 ± 2.53%, -25.17 ± 5.04% vs -27.37 ± 2.87%, 17.68 ± 5.69% vs 21.17 ± 6.44%, respectively; all p <0.01) and a marked attenuation in peak twist (14.24 ± 5.57 vs 18.10 ± 5.97, p <0.01) attributed to impaired apical rotation (9.03 ± 5.17 vs 12.79 ± 5.99, p <0.01). AD patients were more likely to present with abnormal loop types with flat ascending slope and delayed peak twist time. In conclusion, abnormal strain and twist precede deterioration in LVEF, suggesting early myocardial involvement in AD. STE can be used as a good alternative for early detection of myocardial dysfunction in AD patients.

Use of reflectance ratios as a proxy for coastal water constituent monitoring in the pearl river estuary.

Spectra, salinity, total suspended solids (TSS, in mg/L) and colored dissolved organic matter (CDOM, ag(400) at 400 nm) sampled in stations in 44 different locations on December 18, 19 and 21, in 2006 were measured and analyzed. The studied field covered a large variety of optically different waters, the absorption coefficient of CDOM ([ag(400)] in m(-1)) varied between 0.488 and 1.41 m(-1), and the TSS concentrations (mg/L) varied between 7.0 and 241.1 mg/L. In order to detect salinity of the Pearl River Estuary, we analyzed the spectral properties of TSS and CDOM, and the relationships between field water reflectance spectra and water constituents' concentrations based on the synchronous in-situ and satellite hyper-spectral image analysis. A good correlation was discovered (the positive correlation by linear fit), between in-situ reflectance ratio R(680)/R(527) and TSS concentrations (R(2) = 0.65) for the salinity range of 1.74-22.12. However, the result also showed that the absorption coefficient of CDOM was not tightly correlated with reflectance. In addition, we also observed two significant relationships (R(2) > 0.77), one between TSS concentrations and surface salinity and the other between the absorption coefficient of CDOM and surface salinity. Finally, we develop a novel method to understand surface salinity distribution of estuarine waters from the calibrated EO-1 Hyperion reflectance data in the Pearl River Estuary, i.e. channels with high salinity and shoals with low salinity. The EO-1 Hyperion derived surface salinity and TSS concentrations were validated using in-situ data that were collected on December 21, 2006, synchronous with EO-1 Hyperion satellite imagery acquisition. The results showed that the semi-empirical relationships are capable of predicting salinity from EO-1 Hyperion imagery in the Pearl River Estuary (RMSE < 2‰).

[Cardiac manifestations of patients with mitochondrial disease].

OBJECTIVE: To analyze the cardiac manifestations of mitochondriopathy patients. METHODS: We retrospectively analyzed the clinical (Electrocardiogram, Holter monitoring, echocardiogram and laboratory examinations) and pathological data of 90 mitochondriopathy patients diagnosed within recent 20 years. The cardiac involvement data from these patients were summarized. RESULTS: Hypertrophic cardiomyopathy was found in 2 patients and dilated cardiomyopathy in 3 patients Mitochondriopathy diagnosis was made in 1 patient two years after heart transplantation due to heart failure resulting from previously diagnosed hypertrophic cardiomyopathy with noncompaction. The prevalence of cardiomyopathy is 5.6% (5/90). The prevalence of various arrhythmias was 22.2% (20/90). Four patients received permanent pacemaker because of Adams-Stokes attack or bradyarrhythmias (mitochondriopathy diagnosis was made 1-3 years post pacemaker implantation in 3 cases). History of syncope, respiratory failure, RBBB, atrial fibrillation and episodic ventricular tachyarrhythmias were presented in 1 patient with mitochondriopathy, another mitochondriopathy patient developed atrial tachyarrhythmias. Arrhythmia were present in 14 mitochondriopathy patients including RBBB, bifascicular block, intraventricular block, Wolff-Parkinson-White syndrome and short PR interval syndrome. The mtDNA 3243A-G mutation was detected in 8 patients. CONCLUSIONS: Incidence of cardiomyopathy, heart failure and severe arrhythmias is high in patients with mitochondriopathy. Therefore, young cardiomyopathy patients with severe conduction block disorders should undergo relevant etiologic and genetic screening for mitochondriopathy and patients with diagnosed mitochondriopathy should regularly receive electrocardiogram and echocardiography examinations for possible cardiac involvement.