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.

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.

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.

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.

Prognostic Value of Tricuspid Valve Geometry and Leaflet Coaptation Status in Patients Undergoing Tricuspid Annuloplasty: A Three-Dimensional Echocardiography Study.

BACKGROUND: The advent of three-dimensional echocardiography (3DE) enables detailed evaluation of the tricuspid valve (TV) apparatus; nonetheless, the clinical value of preoperative 3DE is unknown in patients undergoing tricuspid annuloplasty (TA). The aim of this study was to evaluate the prognostic value of TV geometric parameters and leaflet coaptation status evaluated by 3DE in patients undergoing TA. METHODS: A total of 122 patients who underwent TA during left-sided heart valve surgery were prospectively evaluated. Detailed 3DE was performed before surgery. Adverse outcome was defined as the occurrence of heart failure requiring hospital admission or all-cause mortality following TA. RESULTS: A total of 33 adverse events (17 heart failures and 16 deaths) occurred during a median follow-up of 36 months. Tethering volume (hazard ratio = 1.32; 95% CI = 1.05-1.66; P = .01) and ratio of total leaflet length to closure length (hazard ratio = 1.07; 95% CI = 1.03-1.12; P < .01) were associated with adverse events after adjustment for age, sex, and tricuspid regurgitation vena contracta width. Receiver-operator characteristic curve analysis revealed that tethering volume (area under curve = 0.73) and ratio of total leaflet length to closure length (area under curve = 0.75) were most associated with adverse events at 1-year follow-up. The presence of either a large tethering volume or a low ratio of total leaflet length to closure length was predictive of an adverse outcome 1 year following TA. CONCLUSIONS: Our study suggests that 3DE-derived TV tethering volume and ratio of total leaflet length to closure length are important preoperative measures associated with adverse events in patients undergoing TA.

Left ventricular strain is associated with acute postoperative refractory hypotension in patients with constrictive pericarditis and preserved ejection fraction.

BACKGROUND: Pericardiectomy is an effective treatment for constrictive pericarditis (CP). Early postoperative complications such as refractory hypotension and congestive heart failure occur in these patients and are associated with increased morbidity and mortality. We hypothesized that left ventricular (LV) myocardial strain measured by two-dimensional speckle tracking echocardiography (2DSTE) could identify early cardiac dysfunction and relate to acute postoperative adverse events in CP patients. METHODS: Forty-four CP patients with preserved left ventricular ejection fraction (LVpEF, 64%±8%) and 44 age- and sex-matched controls were enrolled. Conventional 2DSTE was performed before pericardiectomy. Global and segmental peak systolic strain values were measured. The primary endpoint was a composite of postoperative refractory hypotension, congestive heart failure and cardiogenic death. Refractory hypotension was defined as hypotension requiring prolonged usage of intravenous inotropic medication (IVIM) (≥2 days). RESULTS: Postoperative refractory hypotension occurred in 26 cases, and no patients experienced congestive heart failure or cardiogenic death. Compared to controls, CP patients had decreased absolute global and segmental circumferential strain (CS), radial strain (RS), and longitudinal strain (LS) except septal LS. Patients with refractory hypotension exhibited lower epicardial CS (P=0.04). Epicardial CS was an independent risk factor correlated with postoperative adverse outcome [P=0.014, OR =1.236 (1.044-1.464)] while LVEF was not. Lower absolute value of epicardial CS was related to higher (P=0.02) and longer usage of intravenous furosemide (P=0.04) to keep negative fluid balance perioperatively. CONCLUSIONS: LV strain value is markedly reduced in patients with CP and LVpEF. Lower preoperative epicardial CS value is associated with greater risk of early refractory hypotension and more aggressive fluid management.

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.

Cerebral embolism secondary to cardiac amyloidosis: A case report and literature review.

Cardiac amyloidosis (CA) describes a group of heterogeneous diseases that are characterized by the extracellular fibril deposition of amyloid protein in the myocardium. The abnormal protein is usually derived from light-chain amyloidosis, mutant transthyretin amyloidosis and wild-type transthyretin. Patients with ischemic strokes and amyloidosis have been sporadically reported, however, they are not well summarized. In the present study, a case of cerebral ischemic stroke, secondary to CA was described. This patient presented with dyspnea on exertion, without any evidence of atrial fibrillation. A biopsy revealed deposition of amyloid in the myocardium and Congo Red staining was positive. He suffered from acute infarction of left basal ganglia, resulting from occlusion of the left middle cerebral arterial 6 months prior to admission. However, re-examination of cerebral magnetic resonance imaging in the present hospital revealed an old infarction in the region of the left basal ganglia with a normal appearance of the left middle cerebral artery. Transesophageal echocardiography (TEE) and cardiac magnetic resonance (CMR) both discovered intra-cardiac thrombi, confirming the diagnosis of cardiogenic cerebral embolism. The present study indicates that patients with CA may additionally present with cardiogenic cerebral embolism, and TEE and CMR imaging may help to avoid missing the presence of intra-cardiac thrombi.

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.

Effect of Right Heart Systolic Function on Outcomes in Patients with Constrictive Pericarditis Undergoing Pericardiectomy.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Doppler Echocardiographic Measurements in Normal Chinese Adults (EMINCA): a prospective, nationwide, and multicentre study.

AIMS: Currently, available Doppler echocardiographic reference values are derived mainly from North American and European population studies, which may not applicable to the Chinese population. We aimed to establish normal reference values of Doppler echocardiographic parameters in a nationwide, population-based cohort of healthy Han Chinese adults. METHODS AND RESULTS: A total of 1394 qualified healthy subjects (mean age 47.3 ± 16.0 years, 678 men) were enrolled at 43 collaborating laboratories, 37 transvalvular flow and tissue Doppler parameters were obtained, and the impacts of gender and age on each parameter were analysed. Significant differences were found between men and women in 48.6% (18/37) of the parameters analysed, and among different age groups in 83.8% (31/37) of the parameters in men and in 86.5% (32/37) of the parameters in women. CONCLUSIONS: Normal reference values of Doppler echocardiographic parameters were established for the first time in a nationwide, population-based cohort of healthy Han Chinese adults. Since most of these parameters differed by gender and/or age, reference values specified for gender and age should be recommended in clinical practice.

Echocardiographic measurements in normal chinese adults focusing on cardiac chambers and great arteries: a prospective, nationwide, and multicenter study.

BACKGROUND: Currently available echocardiographic reference values are derived mainly from North American and European population studies, and no echocardiographic reference values are available for the Chinese population. The aim of this study was to establish normal values of echocardiographic measurements of the cardiac chambers and great arteries in a nationwide, population-based cohort of healthy Han Chinese adults. METHODS: A total of 1,586 healthy Han Chinese volunteers aged 18 to 79 years were screened at 43 collaborating laboratories throughout China. Standard M-mode and two-dimensional echocardiography was performed to obtain measurements of the cardiac chambers and great arteries. The impacts of gender and age on all echocardiographic measurements were analyzed. RESULTS: A total of 1,394 qualified healthy subjects (mean age, 47.3 ± 16.0 years; 678 men) were ultimately enrolled. Except for left ventricular ejection fraction, values of cardiac chamber and great arterial dimensions were significantly higher in men than in women. Most measurements of the atrial and great arterial dimensions, left ventricular wall thickness, and left ventricular mass increased with age in both men and women. CONCLUSIONS: Normal reference values of cardiac dimensional parameters were established for the first time in a nationwide, population-based cohort of healthy Han Chinese adults. Because most of these parameters were found to vary with gender and age, reference values stratified for gender and age should be used in clinical practice.

[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.

[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.

[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.

Anomalous origin of the left coronary artery from the pulmonary artery detected by echocardiography in an asymptomatic adult.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation. It is unusual for an ALCAPA patient to survive to adulthood. We present a case of an asymptomatic 54-year-old woman with this syndrome in which visualization of a markedly enlarged and tortuous right coronary artery and intercoronary collaterals by echocardiography raises suspicion for this disease and subsequently guides a step-by-step diagnosis. The patient lives well without surgery 3 years after diagnosis.

[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.

[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.