Clinical Characteristics And Outcomes Of Childhood Takayasu Arteritis Complicated With Heart Failure:A Retrospective Study Of 25 Patients

Objective:Childhood Takayasu arteritis is the most common large vessel vasculitis in the pediatric population.Heart failure is a severe complication and one of the main causes of death in patients with Takayasu arteritis.This study aims to investigate the clinical features and long-term outcomes in childhood Takayasu arteritis patients with heart failure.Methods:A total of 25 consecutive childhood Takayasu arteritis patients complicated with heart failure who were hospitalized at Fuwai Hospital,Chinese Academy of Medical Sciences from January1,2003 to December31,2017 were enrolled.The demographic,clinical,laboratory,imaging features and treatment plans were collected.Kaplan-Meier survival curve was used to assess the overall,heart failure event-free and Takayasu arteritis event-free survival.Results:The average onset age of childhood Takayasu arteritis was(13.50±3.04)years and 96%were female.The median(P25,P75)delay time to diagnosis was 1.04(0.46,3.52)years.The common clinical manifestation included symptoms relating to heart failure(dyspnea[100%],cough and expectoration[40%],palpitation[36%]and lower extremity edema[40%]),bruits(88%),hypertension(68%),blood pressure asymmetry in the extremities(60%),retinopathy(44%)and pulse deficits(40%).Decrease of left ventricle ejection fraction(68%),enlargement of left ventricle(64%)and arterial pulmonary hypertension(40%)were most prevalent echocardiographic features.Elevation of Nterminal pro-brain natriuretic peptide(NT-proBNP)was observed in all patients and the median(P25,P75)NT-proBNP was 3504(1216,9440)pg/mL.HATA type V(64%)was the most frequent involved pattern,while the descending aorta(80%),supra-aortic artery(72%)and renal artery(64%)were more likely to be implicated.Renovascular hypertension(48%)and descending aorta stenosis(40%)were the most common causes of heart failure in childhood Takayasu arteritis patients.For treatment,80%patients were treated glucocorticoids-based the anti-inflammatory therapy.Only 40%patients received revascularization of the target arteries.At a median(P25,P75)follow-up of 6.65(2.97,10.52)years,heart failure events,Takayasu arteritis events and death were observed in 28%,64%and 16%patients respectively.The 5-year heart failure event-free and Takayasu arteritis event-free survival were 72.2%and 42.3%.The 1-year overall survival was 91.7%,and all patients died of heart failure.Conclusions:The most common causes of heart failure in childhood Takayasu arteritis are renovascular hypertension and descending aorta stenosis.The 5-year heart failure eventfree and Takayasu arteritis event-free survival were low.Enhancing the early recognition of this disease and deepening the understanding of the disease process may help to identify the disease timely,so as to intervene in time and then improve the prognosis.Objectives:Subclinical Cushing syndrome(SCS)and primary aldosteronism(PA)both result from abnormal hormone secretion of adrenal cortex.This study is designed to investigate the incidence of SCS in Chinese patients with PA,and explore the impact of the presence of SCS on the glucose and lipid metabolism in patients with PA.Methods:A total of 309 consecutive patients with PA admitted to Fuwai hospital from January 2017 to September 2019 were screened.Patients were divided into SCS group(n=31)and non-SCS group(n=278).The baseline data,data reflecting glucose and lipid metabolism and results of adrenal CT were collected and analyzed.Results:SCS occurred in 10.03%PA patients in this cohort.The median 0am serum cortisol level was significantly higher in the SCS group than in the non-SCS group(4.05[2.65,7.58]ug/dl vs.2.30[1.50,3.93]ug/dl,P<0.05).However,the average age,sex composition,body mass index,data reflecting glucose metabolism(fasting blood glucose and HbAlc)and lipid metabolism(triglyceride,total cholesterol,LDL-C and HDL-C)were similar between the two groups(all P>0.05).Prevalence of normal adrenal CT results was significantly lower in the SCS group than in the non-SCS group(15.38%vs.34.78,P<0.05),while the prevalence of multiple nodules was significantly higher in the SCS group than in the non-SCS group(15.38%vs.3.56%,P<0.05).Conclusions:In addition to hypersecretion of aldosteronism,hypersecretion of cortisol was detected in PA patients complicating with SCS.However,the abnormal secretion of cortisol did not affect the lipid and glucose metabolism of patients with PA.