Imaging Characteristics of Primary Cardiac Angiosarcoma / 罕见病研究

Primary cardiac angiosarcoma is a type of soft tissue sarcoma originating in vascular endothelial cells, without obvious gender differences in the incidence rate and specific early clinical manifestations, whilstpericardial effusion often found at the first presentation of most patients. Tumors are mostly located in the right atrium and pericardium. Echocardiography is the preferred examination method for diagnosing cardiac angiosarcoma and multimodal imaging is important in the diagnosis and differential diagnosis of benign and malignant cardiac mass. This article retrospectively analyzes the 25 cases of clinical manifestations and imaging features of primary cardiac angiosarcoma.

The impact of early surgery on long-term outcome of patients with left-sided infective endocarditis / 中华内科杂志

Objective This study aims to evaluate the impact of early surgery on long-term outcome of patients with left-sided native valve infective endocarditis (IE).Methods Clinical data were retrospectively reviewed in 239 consecutive patients with left-sided native valve IE from 2002 to 2012 in Peking Union Medical College Hospital (PUMCH).Propensity score was used to match patients in the early operation and conventional treatment groups.Results Early surgery was performed in 70 (29.3％)patients and the conventional treatment strategy was applied in 169 (70.7％) patients.The median followup period was 2 years.IE-related mortality was lower in the early operation group than in the conventional treatment group (10.0％ vs 23.1％,P =0.02).For 58 propensity score-matched pairs,the cumulative survival free from IE related death was significantly higher in the early operation group than in the conventional treatment group (P =0.027).Regression analysis of the matched cohorts revealed that early surgery was independently associated with decreased IE-related mortality (HR 0.286 ; 95％ CI 0.092-0.893 ;P =0.031).While either cardiac function with NYHA Class Ⅲ-Ⅳ (HR 4.044; 95％ CI 1.318-12.407;P =0.015) or uncontrolled infection (HR 52.064; 95％ CI 10.996-247.194; P ＜ 0.001) was associated with poor prognosis of increased mortality.Conclusions Early surgery improved long-term outcome in patients with left-sided native valve IE compared with conventional therapy.Risk factors related to increased mortality included heart failure and uncontrollable infection.

Cardiac involvement of the Type Ⅰ mucopolysaccharidosis / 中华内科杂志

Objective To investigate the manifestations of cardiac involvement in the patients with mucopolysacharidosis Ⅰ (MPS Ⅰ).Methods The clinical data of 10 MPS Ⅰ patients were collected.Electrocardiography (ECG) and echocardiography (Echo) were performed in all patients and then analyzed.Results Among the ten patients,seven were men.The onset age of MPS was (0.5 ～ 8.0) years old and the age of diagnosis was (1.8 ～ 20.0) years old.Two patients had grade 2 precordial systolic murmur.ECG was abnormal in three patients with right ventricular hypertrophy in two and right axis deviation in another one.Echo showed valvular thickening and insufficiency in nine patients,enlarged left atrium and ventricle in one patient,hapulmonary hypertension and right ventricular hypertrophy in two patients and abnormal left ventricular configuration in five patients.Conclusions Cardiac involvement is common in MPS Ⅰ patients and may present as valvular thickening with regurgitation,abnormal left ventricular configuration and pulmonary hypertension.The cardiac involvement progresses with age.ECG and Echo should be done regularly during follow-up of MPS Ⅰ patients.