Noninvasive electrocardiography monitoring for very early recurrence predicts long-term outcome in patients after atrial fibrillation ablation.

BACKGROUND: Atrial fibrillation (AF) is the most common sustained arrhythmia, and catheter ablation has been shown to be a highly effective treatment for patients with symptomatic AF. Very early recurrence (VER) of AF within 7 days after catheter ablation is common, but the clinical significance of VER remains unclear. We have examined the usefulness of the noninvasive electrocardiography monitor for the detection of VER and the relationship between VER and late recurrence (LR). METHODS: Eighty-eight patients with paroxysmal or persistent atrial fibrillation were retrospectively included. All patients underwent primary catheter ablation at a large general hospital between March 2016 and August 2018. All patients were followed up in atrial fibrillation clinic at an interval of every 3 months for late recurrence of AF. VER was evaluated by one-lead continuous noninvasive electrocardiography monitoring device for 7 days after ablation. The association between VER and LR was analyzed by univariate and multivariate Cox regression model. RESULTS: Mean age was 62.9 ± 9.7 years, and 39.8% were female. Thirty-two patients (36.4%) experienced VER. After a mean follow-up of 539.36 ± 211.66 days, 17 patients (19.3%) experienced LR. Multivariate Cox regression analysis revealed VER was an independent predictor of LR: HR 3.6 (95% CI, 1.2-10.8), p = .020. In addition, diabetes was also associated with LR of atrial fibrillation. CONCLUSIONS: Noninvasive electrocardiography monitoring was a useful tool for detecting VER and VER after catheter ablation was associated with LR.

Evaluating heart function in patients with POEMS syndrome.

AIMS: Our aim is to investigate the characterized echocardiographic cardiac measurements of POEMS syndrome and determine its relationship with clinical manifestations. METHODS AND RESULTS: The cross-sectional study included 27 treatment-naïve patients with newly diagnosed POEMS syndrome and 26 age- and sex-matched healthy volunteers. Information of clinical manifestations, serological tests, pulmonary function tests, and both conventional echocardiograph and tissue Doppler imaging (TDI) were collected and analyzed. Pearson's correlation coefficient was used for determining the related clinical and echocardiographic parameters. Compared to healthy people, left ventricular (LV) mass index (LVMI) was elevated in patients with POEMS syndrome (41.3 ± 11.0 g/m2.7 , P < .05). LV systolic dysfunction was found by decreased mitral S' (9.0 ± 2.2 m/sec, P < .01), and diastolic dysfunction by mitral E'/A' (1.10 ± 0.42, P < .05), E/E' (8.69 ± 4.06, P < .001) on lateral, and E/E' (7.90 ± 3.28, P = .133) on septal mitral annulus. The presence of decreased tricuspid annular plane systolic excursion (TAPSE) (22.2 ± 3.5 mm, P < .01) and lateral tricuspid S' (11.1 ± 1.8 m/sec, P < .05) suggested deterioration of right ventricular (RV) systolic function. Parameters obtained from standard echocardiograph (tricuspid E/A ratio and DT) and TDI ((lateral tricuspid annulus E'/A' and E/E') indicated reduced RV diastolic function. Pulmonary hypertension (PH) was presented in six patients. Correlation analysis suggested that PH was related to total lung capacity (TLC) and diffusion capacity of carbon monoxide (DLCO). CONCLUSION: Echocardiographic measurements found that there was elevation of LVMI, pulmonary artery hypertension, and subclinical impairment of systolic and diastolic functions of both the right and left heart in patients with POEMS syndrome.

Telangiectasia as a potential clinical marker of microvascular lesions in systemic sclerosis patients from EUSTAR data in China.

OBJECTIVES: To investigate the prevalence and clinical relevance of telangiectasia in Chinese patients with systemic sclerosis (SSc). METHODS: Data from 230 SSc EUSTAR patients from Peking Union Medical College Hospital (2009-2011) that fulfilled the 1980 American College of Rheumatology SSc classification criteria were prospectively collected. Demographic, clinical, and laboratory data were calculated between groups with and without telangiectasia, and a six-minute walk test, pulmonary function test (PFT), transthoracic echocardiography (TTE), right heart catheterisation (RHC) and modified Rodnan skin score (mRSS) were performed. RESULTS: 96 patients (41.7%) were diagnosed with telangiectasia. There were no significant differences between patients with and without telangiectasia based on gender, age at onset, Raynaud's phenomenon (RP) duration, or SSc classification. Disease duration both from RP onset of patients and from first non-RP manifestation of patients with telangiectasia was significantly longer than patients without (p<0.05). RP (97.9% vs. 90.3%), finger/toe sclerosis (96.9% vs. 88.1%), facial sclerosis (68.8% vs. 53.7%), digital ulcers (DUs; 40.6% vs. 23.1%), digital pitting (49.0% vs. 33.8%), joint contracture (20.8% vs. 10.4%) and erythrocyte sedimentation rate elevation (26.7% vs. 14.8%) were significantly greater in telangiectasia patients (p<0.05). There were no differences in autoantibody development between patients with and without telangiectasia (p>0.05). PFT showed that forced vital capacity (77.0±17.26 vs. 83.05±16.53, p=0.005) and diffusion capacity for CO of the lung (58.9±19.4 vs. 65.7±19.7, p=0.030) were lower, while forced expiratory volume ratio (87.02±7.8 vs. 84.33±7.1, p=0.029) was higher in SSc with telangiectasia. Pulmonary artery hypertension (PAH) prevalence (25.0% vs. 14.2%) was significantly greater in patients with telangiectasia. CONCLUSIONS: Telangiectasia are common in Chinese SSc patients and usually associated with DUs, RP, and PAH. Telangiectasia could be a clinical marker of microvascular disease in SSc.

Pulmonary hypertension in POEMS syndrome.

POEMS syndrome is a rare clonal plasma cell disease. Patients with POEMS syndrome are at risk of developing pulmonary hypertension, but the data on its incidence and impact on outcome are limited. We reviewed records of 154 POEMS syndrome patients with complete duplex echocardiography data for estimation of pulmonary artery systolic pressure (sPAP) at the time of diagnosis. Forty-two (27%) of 154 patients with pulmonary hypertension (estimated sPAP ≥50 mmHg) were identified. Median age was 46 years (range 31-71 years). Patients with pulmonary hypertension were more likely to have peripheral edema (P=0.04), ascites (P=0.02), pleural effusion (P=0.005), and have longer time from onset to diagnosis (P=0.004) when compared with those without pulmonary hypertension. Restrictive abnormalities and decreased diffusion capacity of carbon monoxide were observed in 83% and 96% patients with pulmonary hypertension, compared with 50% and 72% in patients without pulmonary hypertension, respectively. Reversibility of pulmonary hypertension was observed after treatment of POEMS syndrome. After median follow of 32 months, survival of patients with pulmonary hypertension was worse than those without (median overall survival 54 months vs. median not reached, P=0.021). In conclusion, pulmonary hypertension is a common feature of POEMS syndrome, and is associated with signs of extravascular volume overload. Although active treatment of POEMS syndrome can reverse pulmonary hypertension, survival of these patients is worse than those without pulmonary hypertension.

The prognostic value of left ventricular systolic function measured by tissue Doppler imaging in septic shock.

INTRODUCTION: Left ventricular (LV) dysfunction is common in septic shock. Its association with the clinical outcome is still controversial. Tissue Doppler imaging (TDI) is a useful tool to quantify LV function; however, little knowledge is available about the prognostic value of these TDI variables in septic shock. Therefore, we performed this prospective study to determine the role of TDI variables in septic shock. METHODS: Patients with septic shock in a medical intensive care unit were studied with transthoracic echocardiography with TDI within 24 hours after the onset of septic shock. Baseline clinical, laboratory, and echocardiographic variables were prospectively collected. Independent predictors of 90-day mortality were analyzed with the Cox regression model. RESULTS: During a 20-month period, 61 patients were enrolled in the study. The 90-day mortality rate was 39%; the mean APACHE IV score was 84 (68 to 97). Compared with survivors, nonsurvivors exhibited significantly higher peak systolic velocity measured at the mitral annulus (Sa) (11.0 (9.1 to 12.5) versus 7.8 (5.5 to 9.0) cm/sec; P < 0.0001), lower PaO2/FiO2 (123 (83 to 187) versus 186 (142 to 269) mm Hg; P = 0.002], higher heart rate (120 (90 to 140) versus 103 (90 to 114) beats/min; P = 0.004], and a higher dose of norepinephrine (0.6 (0.2 to 1.0) versus 0.3 (0.2 to 0.5) µg/kg/min; P = 0.007]. In the multivariate analysis, Sa > 9 cm/sec (hazard ratio (HR), 5.559; 95% confidence interval (CI), 2.160 to 14.305; P < 0.0001), dose of norepinephrine (HR, 1.964; 95% CI, 1.338 to 2.883; P = 0.001), and PaO2/FiO2 (HR, 0.992; 95% CI, 0.984 to 0.999; P = 0.031) remain independent predictors of 90-day mortality in septic-shock patients. CONCLUSIONS: Our study demonstrated that LV systolic function as determined by TDI, in particular, Sa, might be associated with mortality in patients with septic shock.

Left atrial function assessed by tissue doppler imaging as a new predictor of cardiac events after non-ST-elevation acute coronary syndrome.

AIMS: Left ventricular (LV) function is a predictor for future cardiac events in patients with non-ST-elevation acute coronary syndrome (NSTE-ACS). The aim of this study was to assess whether left atrium (LA) function has additional predictive value for the prognosis of NSTE-ACS patients, especially when assessed by tissue Doppler imaging (TDI). METHODS AND RESULTS: This study prospectively recruited 164 patients with NSTE-ACS where clinical and echocardiographic parameters were collected within the first 72 hours of admission. Primary end point was assessed during the 6-month follow-up period which included cardiac mortality and/or rehospitalization for recurrent ACS or heart failure. Atrial function was assessed by conventional echocardiographic parameters and by TDI that measured the mean atrial contraction velocity at the midsegments of interatrial septum, anterior, inferior, and lateral wall of LA (mLA-V). The primary end point occurred in 33 (20.1%) patients who had lower mLV-A (5.4 ± 1.6 vs 6.5 ± 1.4 cm/sec, P < 0.01). Patients with mLA-V <6.3 cm/sec had more cardiac events (30.9% vs 9.6%, P < 0.01). By multivariate logistic regression analysis, mLA-V <6.3 cm/sec was an independent predictor for cardiac events (odds ratio: 2.79, 95% confidence interval: 1.07-7.30, P = 0.04). Furthermore, mLA-V<6.3 cm/sec had an incremental predictive value for cardiac events to clinical data, LV ejection fraction, and LV diastolic function (E/E') (P = 0.02). CONCLUSIONS: In patients with NSTE-ACS, early assessment of LA function by TDI appears useful to predict the midterm cardiac events, which adds prognostic information in addition to that of LV function.

[The value of Doppler echocardiography in assessment of pulmonary capillary wedge pressure in pulmonary hypertension].

OBJECTIVE: To explore the association between Doppler echocardiography parameters and pulmonary capillary wedge pressure (PCWP) obtained by right heart catheterization (RHC) in patients with pulmonary hypertension (PH). METHODS: RHC and a simultaneous echocardiography were performed in 86 PH patients. The correlation between the echocardiographic parameters and PCWP was analyzed. RESULTS: The early diastolic velocity (E) of mitral valve blood flow was significantly correlated with PCWP (r = 0.36, P < 0.01). Tissue Doppler imaging measured the diastolic velocity of mitral annulus on the lateral (E'L) and the septal (E'S) wall. Then the average E'M was calculated. The ratio of E to E'L, E'S and E'M showed a strong correlation with PCWP (r = 0.62, 0.34 and 0.47 respectively, P < 0.01). E/E'L < 6 accurately predicted 100% PCWP ≤ 15 mm Hg (1 mm Hg = 0.133 kPa), and E/E'L > 15 could predict PCWP > 15 mm Hg with specificity of 98.5%. CONCLUSION: There are positive correlation between E/E' and PCWP obtained by RHC in PH patients.

[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.

A retrospective study of congenital scoliosis and associated cardiac and intraspinal abnormities in a Chinese population.

Our objective is to assess the incidence of cardiac and intraspinal abnormities in Chinese congenital scoliosis (CS) patients and to study the relationship between the associated abnormities and the different CS types. Five-hundred and thirty-nine consecutive Chinese patients with CS were retrospectively studied, and the records of echocardiography, plain radiograph of the entire spine, magnetic resonance imaging of the entire spine and/or myelogram were reviewed. The results indicated that the incidence of cardiac and intraspinal abnormities in CS patients was 14.1 and 24.5%, respectively. There was no difference in the incidence of associated cardiac and intraspinal abnormities in different CS types (P > 0.05). The most common cardiac abnormities in CS patients was mitral valve prolapse, which was followed by congenital heart diseases, including atrial septal defect, ventricular septal defect, bicuspid aortic valve and patent ductus ateriosus. The cardiac abnormities were not likely to be concurrent with intraspinal abnormities in CS patients (P = 0.04). The intraspinal abnormities were more common in female and older patients (all P < 0.05). One or more abnormities mentioned above could be found in 36.8% CS patients and were more likely to be found in female patients (P < 0.01). We concluded that CS is not a simple abnormity, due to the high incidence of associated deformities of other organs, comprehensive assessment was strongly recommended before the surgical correction for CS patients.

[A report of eight cases of Behcet's disease with intracardiac thrombus and literatures review].

OBJECTIVE: To analyze the clinical characteristics of Behcet's disease with intracardiac thrombus. METHODS: The data of 8 patients diagnosed as Behcet's disease with intracardiac thrombus in Peking Union Medical College Hospital from January, 1990 to January, 2011 were studied retrospectively. RESULTS: Behcet's disease with intracardiac thrombus was found in 8 patients (5 men and 3 women) with a median age of 28.5 years. Most of them were young men. Thrombus was mostly found in the right side of the heart. Most of the patients had pulmonary thromboembolism with negative anticardiolipid antibody and basically normal C-reactive protein and erythrocyte sedimentation rate. CONCLUSION: Intracardiac thrombus associated with Behcet's disease most commonly occurs in young men and usually involves the right side of the heart.

[The value of brain natriuretic peptide in connective tissue diseases associated with pulmonary arterial hypertension].

OBJECTIVE: To explore the potential role of brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) in the assessment of patients with connective tissue diseases (CTD) associated pulmonary arterial hypertension (PAH). METHODS: Thirty patients with CTD associated PAH were evaluated by WHO function, echocardiography, right heart catheterization and blood biomarkers. All the clinical data was analyzed statistically. RESULTS: All patients [age (39.5 ± 11.6) yr] were female. Both NT-proBNP and BNP were significantly increased and significantly correlated (all P < 0.05) respectively with mean pulmonary arterial pressure (r = 0.53 and r = 0.40), right arterial pressure (r = 0.55 and r = 0.42), pulmonary vascular resistance (r = 0.69 and r = 0.61), cardiac index (r = -0.58 and r = -0.46), mixed venous blood oxygen saturation (r = -0.62 and r = -0.54), pericardial effusion (r = 0.46 and r = 0.43), right atrial sizes (r = 0.40 and 0.53, and r = 0.39 and 0.45) and right ventricular size (r = 0.55 and r = 0.37). Furthermore, NT-proBNP, but not BNP, significantly correlated with WHO function class (r = 0.55). CONCLUSION: Blood NT-proBNP and BNP were elevated in patients with CTD associated PAH and paralleled the extent of function class, pulmonary hemodynamic changes and right ventricular remodeling.

[Effects of inhaled aerosolized iloprost in adult patients with pulmonary arterial hypertension on right heart functions].

OBJECTIVE: To explore the immediate effects of inhaled aerosolized iloprost on right heart function in adult patients with pulmonary arterial hypertension (PAH). METHODS: A total of 30 PAH patients were recruited. Right heart catheterization and echocardiography were performed before and immediately after the inhalation of iloprost (20 µg). RESULTS: After inhalation, the values of mean pulmonary artery pressure (PAPm) and pulmonary vascular resistances (PVR) decreased markedly (42.5 ± 9.6 to 34.4 ± 11.9 mm Hg, P < 0.001; 9.6 ± 5.7 Wood unit to 7.1 ± 4.8 Wood unit, P < 0.001). And the value of tricuspid annular systolic motion peak velocity (TASm) increased markedly [(10.7 ± 2.1) cm/s vs (11.9 ± 2.5) cm/s, P < 0.01]. The baseline level of TASm was higher in acute responders than non-responders [(12.0 ± 2.2) cm/s vs (10.1 ± 1.8) cm/s, P = 0.01] and TASm increased markedly after inhalation in non-responders [(10.1 ± 1.8) cm/s vs (11.6 ± 2.3) cm/s, P < 0.01]. CONCLUSION: The inhalation of iloprost decreases the levels of PAPm and PVR and improve right heart functions in adult PAH patients. For non-responders, right heart function is worse and more benefits may be achieved after the inhalation of iloprost.

[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.

Right ventricular systolic function is associated with health-related quality of life: a cross-sectional study in community-dwelling populations.

BACKGROUND: Considerable evidence has been presented that heart and health-related quality of life are directly linked in patients with various diseases. This exploratory study investigated whether cardiac structure and function were associated with health-related quality of life in the general population. METHODS: This cross-sectional study was performed in five villages of Shunyi, a suburban district of Beijing, from June 2013 to April 2016. All inhabitants aged 35 years or older living in five villages of Shunyi were invited to participate. Exclusion criteria were individuals who declined participation, who had incomplete Health-related quality of life (HRQoL) data, and who had suboptimal echocardiograms. HRQoL was evaluated by the Mandarin version of SF-36. The association between the echocardiography-derived cardiac structure and function and each domain of SF-36 was analyzed by the multivariate linear regression analysis after adjusted for conventional risk factors affecting HRQoL. RESULTS: The baseline data of 990 individuals were analyzed. The median age of the participants was 57 (50-63) years, and 367 (37.1%) were male, the average physical and mental component summary scores were 89.3 (79.8-94.3) and 90 (83.5-95) respectively. Tricuspid annular plane systolic excursion, an echocardiography-derived right ventricular parameter, was associated with all the subscales and summarized scores of SF-36 (all P<0.05). The independent association between tricuspid annular plane systolic excursion and physical/mental component summary scores remained after adjusting for age, gender, body mass index, education level, annual personal income, smoking and drinking status, and comorbidities (ß=0.65, 95% confidence interval 0.30-1.01, P<0.01 and ß=0.49, 95% confidence interval 0.23-0.76, P<0.01 for physical and mental component summary scores respectively). Compared with the participants with tricuspid annular plane systolic excursion ≥21 mm, the participants with tricuspid annular plane systolic excursion <21 mm had lower adjusted scores of physical and mental component summary scores (81.8 vs. 84.5, P=0.015, and 85.5 vs. 88.1, P<0.01 for physical and mental component summary scores respectively). CONCLUSIONS: In this population-based study, right ventricular systolic function assessed by tricuspid annular plane systolic excursion was independently associated with health-related quality of life assessed by SF-36.

[The clinico-pathological manifestation of cardiac involvement in eosinophilic diseases].

OBJECTIVE: To investigate the clinical and pathological features of eosinophilic diseases with cardiac involvement. METHODS: We analyzed the clinical and cardiac pathological data of 7 patients with eosinophilic diseases with cardiac involvement under endomyocardial biopsy or autopsy. RESULTS: Seven patients (5 male, average age 51 years) were enrolled. Four patients were diagnosed as idiopathic hypereosinophilic syndrome and three were Churg-Strauss syndrome. Peripheral blood eosinophilia count increased significantly in all patients. Cardiac involvement included angina pectoris, myocardial infarction, heart failure, presyncope and sudden death. Electrocardiogram showed cardiac ischemia, bundle branch block and third degree atrioventricular block. Echocardiography suggested ventricular and atrial enlargement, decreased ventricular systolic function, pulmonary hypertension, valvular prolapse and insufficiency and endocarditis. Pathology displayed infiltration of eosinophils, formation of granulomata, necrotizing vasculitis, myocardial necrosis and endomyocardial fibrosis in heart. Coronary artery could be also affected and led to myocardial infarction. CONCLUSIONS: Cardiovascular complications of eosinophilic diseases are a major source of morbidity and mortality in these disorders. The manifestations are multiple and early recognition and treatment with steroid and immunosuppressant can improve prognosis.

Design of the Shunyi study on cardiovascular disease and age-related brain changes: a community-based, prospective, cohort study.

BACKGROUND: Rapid economic growth and increasing lifespan have contributed to an increasing burden of chronic non-communicable diseases in China. Population-based studies focusing on cardiovascular disease and age-related brain changes, with extensive clinical, genetic, and imaging data as well as a comprehensive evaluation of brain function are lacking in China. The Shunyi cohort study aimed to investigate the determinants and consequences of cardiovascular disease and age-related brain alterations among individuals residing in a rural area of Beijing. METHODS: This prospective, community-based study included individuals aged 35 years and older living in five villages in Shunyi, a rural district located 20 miles from urban Beijing. A total of 1,586 individuals were enrolled between June 2013 and September 2014. Biological samples and brain magnetic resonance images were collected along with baseline clinical data through face-to-face interviews. Whole exome sequencing and quantitative assessments of cognitive and motor function were performed. RESULTS: Among the 1,586 participants included, 40% were men, and the mean age of the cohort was 56.7±10.0 years. This population had a relatively low education level. A heavy burden of vascular risk factors with a low control rate was observed in the Shunyi population. Since 2017, the cohort has been followed up annually. As of October 2019, we had failed to obtain the follow-up data of five participants. CONCLUSIONS: With an extensive range of clinical, genetic, and imaging data, the Shunyi cohort study has the potential to contribute significantly towards identifying the causes and consequences of cardiovascular disease and age-related brain changes in older people in China.

[Comparison of adenosine versus iloprost by acute vasodilator test in patients of pulmonary arterial hypertension complicated with connective tissue diseases].

OBJECTIVE: To compare the acute hemodynamic effects of adenosine versus iloprost in patients of pulmonary arterial hypertension (PAH) complicated with connective tissue diseases. METHODS: During right heart catheterization, 18 patients of PAH complicated with connective tissue diseases sequentially received intravenous infusion of adenosine and inhaled iloprost. After the baseline hemodynamic data were obtained, an adenosine infusion was started and titrated to the maximal tolerated dose. The hemodynamic parameters were allowed to return to baseline. Then inhalation of iloprost was administered. The effects of both medicines on the patient's hemodynamics were monitored. RESULTS: As compared with the baseline values, the systolic pulmonary artery pressure and pulmonary vascular resistance significantly decreased [(71 +/- 30) vs (80 +/- 29) mm Hg and (712 +/- 440) vs (824 +/- 464) dyn x s x cm(-5) respectively, both P < 0.05) while the heart rate increased significantly [(93 +/- 17) vs (83 +/-16) beat/min, P < 0.05] in the adenosine group. Inhaled iloprost could also lower the systolic pulmonary artery pressure [(66 +/- 29) vs (79 +/- 28) mm Hg, P < 0.05], mean pulmonary artery pressure [(43 +/- 19) vs (52 +/- 19) mm Hg, P < 0.05] and pulmonary vascular resistance [(632 +/- 440) vs (816 +/- 448) dyn x s x cm(-5), P < 0.05] without any effect upon heart rate. Inhaled iloprost exerted more potent effect on lowering mean pulmonary artery pressure and pulmonary vascular resistance than adenosine (P < 0.05). The two medicines did not affect cardiac output, pulse oxygen saturation or systemic blood pressure. The side effects were fewer in the iloprost inhalation group than the adenosine group. CONCLUSION: During acute vasodilator testing, inhaled iloprost was more potent than infused adenosine as a pulmonary vasodilator in PAH complicated with connective tissue diseases.

[Clinical characteristics of Behcet's disease with cardiac involvement - an analysis of forty-two cases].

OBJECTIVE: To analyze the clinical characteristics of Behcet's disease with cardiac involvement. METHODS: Patients diagnosed as Behcet's disease with cardiac involvement in Peking Union Medical College Hospital from 1990 to 2006 were included in this analysis. The clinical characteristics of these patients were studied retrospectively. RESULTS: Forty-two patients were included. Cardiac involvement included valvular disease, angina, pericardial effusion, arrhythmia, intracardiac thrombus and pulmonary artery hypertension. The effect of valve replacement or intervention treatment was related to the activity of Behcet's disease. The involvement of pulmonary artery was an independent correlative factor of mortality (P = 0.03). CONCLUSIONS: The manifestation of cardiac involvement in Behcet's disease is various. If needed, valve replacement operation and intervention treatment should be performed when the Behcet's disease is stable. The involvement of pulmonary artery is an independent correlative factor of mortality.

[Clinical characteristics of angina pectoris in patients of coronary artery disease complicated with renal stenosis and the diagnostic value thereof: analysis of 2820 cases].

OBJECTIVE: To analyze the characteristics of angina symptom complex of patients with coronary artery disease (CAD) complicated with renal stenosis, and to analyze their sensitivity and specificity of angina symptom complex for the diagnosis of CAD. METHODS: The medical records of 2820 in-hospital patients who underwent coronary angiography and renal angiography simultaneously during the period from Jan 1998 to May 2005 and could be diagnosed as with CAD or renal stenosis with the stenotic degree more than 50% of the coronary or renal artery in angiography, were analyzed. The diagnosis of CAD was based on the 3 groups of symptoms recommended by American College of Cardiology/American Heart Association: (1) substernal chest discomfort with a characteristic quality and duration, (2) chest pain provoked by exertion or emotional stress, and (3) chest pain that can be relieved by rest or nitroglycerin. RESULTS: 243 of the 2820 patients had renal stenosis and 2577 of the 2820 patient did not have renal stenosis. The prevalence rates of CAD and hypertension were higher in the renal stenosis group and in the patients without renal stenosis. To diagnose CAD with any one of the 3 groups of above mentioned angina symptom complex, the sensitivity rates were 94.2%, 69.7%, and 75.5% respectively, the specificity rates were 14.3%, 40.0%, and 25.7% respectively, the positive predictive values were 86.7%, 87.3%, and 85.8% respectively, and the negative predictive values were 29.4%, 18.2%, and 15.0% respectively. To diagnose CAD by the symptoms of groups (2) plus (3), (1) plus (2), and (1) plus (3) the sensitivity rates were 68.8%, 56.7%, and 72.6% respectively, specificity rate were 40.0%, 62.9%, and 45.7% respectively, positive predictive values were 87.2%, 90.1%, and 88.8% respectively, and negative predictive values were 17.7%, 19.6, and 21.9% respectively. To diagnose CAD based on all three groups of angina symptom complex, the sensitivity was 56.3%, specificity was 62.9%, positive predictive value was 90.0%, and negative predictive value was 19.5%. CONCLUSION: For detecting CAD based on one, two or three groups of angina symptom complex, there are not significant differences in the diagnostic sensitivity and specificity, however, the positive predictive value is greater and the negative predictive value is smaller in the patients with renal stenosis compared with those without renal stenosis.

Novel Phenotype-Genotype Correlations of Restrictive Cardiomyopathy With Myosin-Binding Protein C (MYBPC3) Gene Mutations Tested by Next-Generation Sequencing.

BACKGROUND: MYBPC3 dysfunctions have been proven to induce dilated cardiomyopathy, hypertrophic cardiomyopathy, and/or left ventricular noncompaction; however, the genotype-phenotype correlation between MYBPC3 and restrictive cardiomyopathy (RCM) has not been established. The newly developed next-generation sequencing method is capable of broad genomic DNA sequencing with high throughput and can help explore novel correlations between genetic variants and cardiomyopathies. METHODS AND RESULTS: A proband from a multigenerational family with 3 live patients and 1 unrelated patient with clinical diagnoses of RCM underwent a next-generation sequencing workflow based on a custom AmpliSeq panel, including 64 candidate pathogenic genes for cardiomyopathies, on the Ion Personal Genome Machine high-throughput sequencing benchtop instrument. The selected panel contained a total of 64 genes that were reportedly associated with inherited cardiomyopathies. All patients fulfilled strict criteria for RCM with clinical characteristics, echocardiography, and/or cardiac magnetic resonance findings. The multigenerational family with 3 adult RCM patients carried an identical nonsense MYBPC3 mutation, and the unrelated patient carried a missense mutation in the MYBPC3 gene. All of these results were confirmed by the Sanger sequencing method. CONCLUSIONS: This study demonstrated that MYBPC3 gene mutations, revealed by next-generation sequencing, were associated with familial and sporadic RCM patients. It is suggested that the next-generation sequencing platform with a selected panel provides a highly efficient approach for molecular diagnosis of hereditary and idiopathic RCM and helps build new genotype-phenotype correlations.