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.

Anesthetic considerations of an emergency decompressive craniotomy complicated with Ebstein's anomaly and atrial septal defect / 中华医学杂志(英文版)

Despite considerable published papers regarding Ebstein's anomaly (EA) patients receiving open-heart tricuspid valve replacement, non-cardiac emergency surgeries were rarely reported. We report a case of emergency decompressive craniotomy in a patient with EA. Anesthesiologists should pay special attention to the complications and anesthetic management during the non-cardiac surgeries performed in EA patients.

Cardiovascular risk profile of patients with glycogen storage disease type I / 中华心血管病杂志

<p><b>OBJECTIVE</b>To investigate the cardiovascular risk profile in patients with glycogen storage disease (GSD) type I.</p><p><b>METHOD</b>The clinical information of 62 patients with GSD type I who admitted to Peking Union Medical Hospital were reviewed and the cardiovascular risk profile was analyzed.</p><p><b>RESULTS</b>The age of the patient cohort was (8.4 ± 6.9) years and the ratio of male vs. female was 36:26. The median disease duration was (6.7 ± 6.2) years and treatment duration was (38.3 ± 35.2) months. The rate of abnormal change in electrocardiogram and echocardiography was 17.7% and 24.2%, respectively. The serum concentration of total cholesterol (TC), triglyceride (TG), low-density lipoprotein cholesterol (LDL-C), high density lipoprotein cholesterol (HDL-C) and uric acid in patient before and after treatment were (6.18 ± 2.47) mmol/L vs. (5.61 ± 1.84) mmol/L (P = 0.020), (11.17 ± 9.85) mmol/L vs. (6.81 ± 5.97) mmol/L (P = 0.010), (2.55 ± 1.27) mmol/L vs. (2.78 ± 1.07) mmol/L (P = 0.617), (0.98 ± 0.37) mmol/L vs. (0.96 ± 0.23) mmol/L (P = 0.005), (526.53 ± 127.09) µmol/L vs. (490.78 ± 129.79) µmol/L (P = 0.977), respectively. The high-sensitivity C-reactive protein levels tended to be higher after therapy compared before treatment (2.33 ± 3.30) mg/L vs. (3.35 ± 3.39) mg/L, P = 0.431.</p><p><b>CONCLUSION</b>Patients with GSD I are associated with an increased risk for cardiovascular disease.</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>

Clinical and pathological characteristics of constrictive pericarditis in China / 中华心血管病杂志

<p><b>OBJECTIVE</b>To summarize the clinical and pathological characteristics of constrictive pericarditis in China.</p><p><b>METHOD</b>Data from 150 patients with constrictive pericarditis who admitted to our hospital from 2000 to 2007 were retrospectively analyzed.</p><p><b>RESULTS</b>Constriction pericarditis was detected by echocardiography in 149 out of 150 patients. Pericardial effusion was evidenced in 59.3% patients (89/150). The diagnostic accuracy rate for identifying constrictive pericarditis by echocardiography (98.7%, 107/109) was comparable to that of surgical diagnosis (100%, 109/109). Tuberculosis was the main cause of constrictive pericarditis in this cohort (78.7%, 118/150) including 25 (16.7%) cases with pathological or etiological evidences of tubercular pericarditis, 8 (5.3%) cases with pathologically active tuberculous focus elsewhere in the body, 66 (44.0%) cases with typical clinical tuberculosis manifestation and responded to anti-tubercular therapy and 19 (12.7%) cases with a diagnosis of suspicious tuberculosis. Pericardiectomy was performed in 108 cases and pericardial biopsy and surgical drainage was performed in 1 patient. In hospital death rate was 8.7% (13/150, 4 tubercular patients and 9 non-tubercular).</p><p><b>CONCLUSION</b>Tuberculosis is the leading cause of constrictive pericarditis in this cohort and the best diagnosis tool is echocardiography other than pathological and etiological findings in pericardium.</p>