Clinical characteristics of Danon disease / 中华心血管病杂志

Objective: To review the clinical data of 7 patients with Danon disease and analyze their clinical characteristics. Methods: The medical records of 7 patients with Danon disease, who were hospitalized in Peking Union Medical College Hospital of Chinese Academy of Medical Sciences from April 2008 to July 2021, were reviewed and summarized, of which 6 cases were diagnosed as Danon disease by lysosomal-associated membrane protein-2 (LAMP-2) gene mutation detection and 1 case was diagnosed by clinicopathological features. Clinical manifestations, biochemical indexes, electrocardiogram, echocardiography, skeletal muscle and myocardial biopsy and gene detection results were analyzed, and patients received clinical follow-up after discharge. Results: Six patients were male and average age was (15.4±3.5) years and the average follow-up time was (27.7±17.0) months. The main clinical manifestations were myocardial hypertrophy (6/7), decreased myodynamia (2/7) and poor academic performance (3/7). Electrocardiogram features included pre-excitation syndrome (6/7) and left ventricular hypertrophy (7/7). Echocardiography examination evidenced myocardial hypertrophy (6/7), and left ventricular dilatation and systolic dysfunction during the disease course (1/7). The results of skeletal muscle biopsy in 6 patients were consistent with autophagy vacuolar myopathy. Subendocardial myocardial biopsy was performed in 3 patients, and a large amount of glycogen deposition with autophagosome formation was found in cardiomyocytes. LAMP-2 gene was detected in 6 patients, and missense mutations were found in all these patients. During the follow-up period, implantable cardioverter defibrillator implantation was performed in 1 patient because of high atrioventricular block 4 years after diagnosis, and there was no death or hospitalization for cardiovascular events in the other patients. Conclusion: The main clinical manifestations of Danon disease are cardiomyopathy, myopathy and mental retardation. Pre-excitation syndrome is a common electrocardiographic manifestation. Autophagy vacuoles can be seen in skeletal muscle and myocardial pathological biopsies. LAMP-2 gene mutation analysis is helpful in the diagnose of this disease.

Clinical and imaging characteristics of patients with primary cardiac angiosarcoma / 中华心血管病杂志

Objective: To investigate the clinical, cardiac imaging characteristics and prognosis of patients with primary cardiac angiosarcoma. Methods: The clinical data of 14 patients hospitalized with primary cardiac angiosarcoma from January 2001 to December 2017 in Peking Union Medical College Hospital were collected and analyzed. Metastatic cardiac angiosarcoma was not included in this study. Patients were followed up post discharge per telephone call or clinical visit. Results: Of the 14 patients, 8 were males and 6 were females, average age was 48 years. The main clinical symptoms were shortness of breath (8/14), hemoptysis (6/14), fever (5/14), chest pain (4/14) and cough (3/14). Imaging examinations showed that the tumors of 8 patients were located in the right heart and 6 in the pericardial cavity. Tumors in the right heart often infiltrate the atrial wall and cause pericardial effusion (7/8). Tumors in the pericardium were characterized by recurrent bloody pericardial effusion (6/6), prone to progressive constrictive pericarditis (3/6), pericardial fluid cytology was often negative (6/6). MRI showed heterogeneous high signal intensity (cauliflower aspect) on T2-weighted image and heterogeneous enhancement with a"sunray" aspect at the perfusion study. At the time of diagnosis, 8 patients developed lung or adrenal metastasis (8/14). The median survival was only 305 days. Conclusions: Primary cardiac angiosarcoma is a rare disease with non-specific clinical manifestation and poor prognosis. Imaging examinations may help diagnosis. The high invasiveness and the easy-to-metastasis feature of the tumor contribute to the poor prognosis of cardiac angiosarcoma.

Comparison of Two-dimensional Speckle Tracking Echocardiography and Late Gadolinium Enhancement Cardiac MRI for Detecting Cardiac Amyloidosis / 中国循环杂志

Objective: To compare the efficiency of two-dimensional speckle tracking echocardiography (2D-STE) and late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) for detecting cardiac amyloidosis. Methods: Systolic longitudinal peak strain by 2D-STE and LGE by CMR were retrospectively analyzed in 10 patients with cardiac amyloidosis. Results: CMR showed 10 patients with LGE and 2D-STE showed 9 patients with ventricular systolic longitudinal peak strain decreasing, the diagnostic consistency of CMR and 2D-STE was 90％. There were 4 patients having both strain abnormality and LGE at meanwhile, 4 patients without CMR presented right ventricular involvement while with 2D-STE indicated abnormal right ventricular systolic longitudinal peak strain, 1 patient with CMR presented LGE in ventricular sepatum while 2D-STE indicated systolic longitudinal peak strain decreasing in both ventricles. Conclusion: 2D-STE and CMR had good consistency for diagnosing left ventricular involvement in patients with cardiac amyloidosis; 2D-STE may have better sensitivity for diagnosing right ventricular amyloidosis.

Effect of Right Heart Systolic Function on Outcomes in Patients with Constrictive Pericarditis Undergoing Pericardiectomy / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>To determine the influence of right ventricular function in patients with constrictive pericarditis (CP) undergoing surgery and to compare the outcomes of patients who received surgery with those managed medically.</p><p><b>METHODS</b>Patients with the diagnosis of CP and healthy volunteers were recruited from January 2006 to November 2011. Patients with CP chose to either receive pericardiectomy or medical management. Echocardiographic measurements were performed to evaluate heart function, and survival was recorded.</p><p><b>RESULTS</b>A total of 58 patients with CP (36 received pericardiectomy, 22 managed medically), and 43 healthy volunteers were included. CP patients who received surgery had a higher survival rate than those managed medically (P = 0.003), and higher survival was also seen in the subgroup of CP patients with severely impaired right systolic function. Albumin level, left ventricular end-diastolic dimension, and tricuspid regurgitation velocity were associated with survival in CP patients who received surgery.</p><p><b>CONCLUSIONS</b>Preoperative right heart function does not affect surgical outcomes. Patients with severely impaired preoperative right systolic function obtain a greater survival advantage with surgery than with medical treatment.</p>

Ultrasonic characteristics of intravenous leiomyomatosis with intracardiac extension / 中国医学科学院学报

<p><b>OBJECTIVE</b>To explore the sonographic characteristics of intraveous leiomyomatosis (IVL) with intracardiac extension and improve its diagnosis.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>IVL with intracardiac extension has certain sonographic characteristics. Ultrasonography is a valuable tool in the diagnosis of IVL with intracardiac extension.</p>

Long-term effects of arteriovenous fistula on cardiac structure and function in non-diabetic hemodialysis patients / 中国医学科学院学报

<p><b>OBJECTIVE</b>To evaluate the long-term effects of arteriovenous fistula (AVF) on heamodynamic changes and cardiac structure and function in non-diabetic hemodialysis patients.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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).</p><p><b>CONCLUSIONS</b>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.</p>

Infective endocarditis in patients with hypertrophic obstructive cardiomyopathy: five cases report / 中华心血管病杂志

<p><b>OBJECTIVE</b>To analyze the clinical characteristics of infective endocarditis in patients with hypertrophic obstructive cardiomyopathy.</p><p><b>METHODS</b>Clinical characteristics from 5 patients with infective endocarditis and hypertrophic obstructive cardiomyopathy hospitalized from January 2000 to December 2010 in our hospital were analyzed.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>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.</p>

Clinical and echocardiographic features of cardiac myxomas in 64 patients / 中华心血管病杂志

<p><b>OBJECTIVE</b>To summarize the clinical and echocardiographic features of cardiac myxomas.</p><p><b>METHODS</b>The medical records of patients with diagnosis of cardiac myxomas who hospitalized in our department from October 1985 to February 2011 were analyzed.</p><p><b>RESULTS</b>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].</p><p><b>CONCLUSIONS</b>Clinical manifestations of cardiac myxomas were various and non-specific. Echocardiography remains the most valuable diagnosis tool for patients with cardiac myxomas.</p>

Association between cardiac function and intradialytic hypotension / 中国医学科学院学报

<p><b>OBJECTIVE</b>To prospectively determinate the association of left ventricular systolic and diastolic function with intradialytic hypotension (IDH) in patients on maintenance hemodialysis.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Hemodynamic parameters obtained by transthoracic echocardiography and right heart catheterization: a comparative study in patients with pulmonary hypertension / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Hemodynamic evaluation is crucial for the management of patients with pulmonary hypertention. Clinicians often prefer a rapid and non-invasive method. This study aimed to examine the feasibility of transthoracic echocardiography for the measurements of hemodynamic parameters in patients with pulmonary hypertension.</p><p><b>METHODS</b>A prospective single-center study was conducted among 42 patients with pulmonary hypertension caused by different diseases. Transthoracic echocardiography and right-heart catheterization were performed within 24 hours. Pulmonary artery systolic, diastolic and mean pressure (PASP, PADP and PAMP), cardiac output (CO), and pulmonary capillary wedge pressure (PCWP) were measured by both methods. A linear correlation and a Bland-Altman analysis were performed to compare the two groups of hemodynamic parameters.</p><p><b>RESULTS</b>A good correlation was found between invasive and non-invasive measurements for PASP (r = 0.96), PADP (r = 0.85), PAMP (r = 0.88), CO (r = 0.82), and PCWP (r = 0.81). Further agreement analysis done by the Bland-Altman method showed that bias and a 95% confidence interval for PASP, PADP, and CO were clinically acceptable while great discrepancies existed for PAMP and PCWP.</p><p><b>CONCLUSIONS</b>The non-invasive measurements by PASP, PADP, and CO in patients with pulmonary hypertension correlate well with the invasive determinations. Transthoracic echocardiography (TTE) was inappropriate for estimating PCWP and PAMP.</p>

Value of cardiac magnetic resonance imaging for the diagnosis of cardiac amyloidosis / 中华心血管病杂志

<p><b>OBJECTIVE</b>To observe the clinical features and cardiac magnetic resonance imaging (CMR) characteristics of patients with endomyocardial biopsy (EMB)-proven cardiac amyloidosis (CA).</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Electrocardiographic and echocardiographic features of patients with primary cardiac amyloidosis / 中华心血管病杂志

<p><b>OBJECTIVE</b>To summarize the electrocardiography and echocardiography features of patients with cardiac amyloidosis (CA) diagnosed by endo-myocardial biopsy (EMB).</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Clinico-pathological evaluation of restrictive cardiomyopathy / 中华心血管病杂志

<p><b>OBJECTIVE</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>RCM may result from various local and systemic disorders. EMB is helpful for identifying the underlying etiology.</p>

Cardiovascular manifestations in Chinese patients with hypereosinophilic syndrome / 中华心血管病杂志

<p><b>OBJECTIVE</b>To evaluate the cardiovascular involvements in Chinese patients with hypereosinophilic syndrome.</p><p><b>METHOD</b>We respectively reviewed 149 inpatients with hypereosinophilic syndrome admitted to Peking Union Medical College Hospital and analyzed the cardiovascular involvements in these patients.</p><p><b>RESULTS</b>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).</p><p><b>CONCLUSIONS</b>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.</p>

Low-dose adenosine echocardiography for detection of myocardial viability in patients with acute myocardial infarction / 中华心血管病杂志

<p><b>OBJECTIVE</b>To evaluate the value of low-dose adenosine echocardiography (LDAE) for detection of myocardial viability in patients with acute myocardial infarction (MI).</p><p><b>METHODS</b>Thirty-six patients underwent LDAE within 3 - 10 days after onset of first acute MI before (n = 4) or after (n = 32) percutaneous coronary intervention. A 17-segment semi-quantitative scoring model was adopted. Wall motion improvement derived from two dimensional images at follow-up (2 - 3 months after acute MI) comparing baseline before adenosine infusion was used as gold criteria for myocardial viability.</p><p><b>RESULTS</b>Low-dose adenosine slightly increased heart rates [(70.7 +/- 10.8) beats/min vs. (78.1 +/- 10.9) beats /min, P < 0.01] and also significantly reduced left ventricular endsystolic volume [(30.4 +/- 1.9) ml vs. (20.1 +/- 9.3) ml, P < 0.01] and increased ejection fraction (62.6% +/- 10.4% vs. 74.7% +/- 9.8%, P < 0.01). The sensitivity, specificity, diagnostic accuracy, positive and negative prective values of LDAE for identification of viable myocardium were 90.3%, 80.8%, 86.0%, 84.8% and 87.5%, respectively. Incidence of mild adverse reaction during LDAE was 38.9% (14/36). LDAE at dose of 100 microgxkg(-1)xmin(-1) was ideal in terms of balanced sensitivity and specificity for detecting viable myocardium without increasing the adverse effects compared to lower doses.</p><p><b>CONCLUSIONS</b>LDAE (100 microgxkg(-1)xmin(-1)) has excellent sensitivity and specificity for detecting viable myocardium in acute MI with only minimal adverse effects.</p>

Cardiac manifestations of patients with mitochondrial disease / 中华心血管病杂志

<p><b>OBJECTIVE</b>To analyze the cardiac manifestations of mitochondriopathy patients.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Comparison of low-dose dobutamine echocardiography and dual-isotope emission simultaneous myocardial perfusion acquisition for myocardial viability assessment / 中华心血管病杂志

<p><b>OBJECTIVE</b>To compare the value of low-dose dobutamine echocardiography (LDDE) and dual-isotope emission simultaneous myocardial perfusion acquisition (technetium-99-m-tetrofosmin/fluorine 18-fluorodeoxy-glucose) single-photon emission computed tomography (DISA-SPECT) for myocardial viability assessment in patients with acute myocardial infarction (MI).</p><p><b>METHODS</b>LDDE and DISA-SPECT were performed in 44 patients within 5-10 days after onset of first acute MI and percutaneous coronary intervention was made thereafter. A 16-segment semi-quantitative scoring model was adopted for both techniques. Wall motion improvement at follow-up (3 months after acute MI) compared with baseline before dobutamine infusion derived from two dimensional images was used as golden criteria for myocardial viability.</p><p><b>RESULTS</b>The sensitivity, specificity, diagnostic accuracy, positive and predictive values for identification of viable myocardium were 77%, 82%, 79%, 82% and 77%, respectively by LDDE and 85%, 62%, 74%, 71% and 79%, respectively by DISA. No difference was found between LDDE and DISA for identifying viable myocardium in hypokinetic segments (74.1% vs. 77.6%, P > 0.05) but less viable myocardium was detected by LDDE than DISA in akinetic segments (29% vs. 53%, P < 0.01).</p><p><b>CONCLUSIONS</b>Sensitivity was higher while specificity was lower on detecting viable myocardium by DISA compared to LDDE. Combined use of the two techniques could improve viable myocardium detection in patients with acute MI.</p>