Clinical characteristics and treatment safety evaluation of rheumatic immune diseases complicated with congenital heart disease

Zhang Yuxin; Hu Caiyun; Ma Hongxiu; Shen Haili

doi:10.3760/j.issn.1673-4777.2026.06.016

Abstract：Objective To compare the clinical characteristics among patients with rheumatic autoimmune diseases(RADs)complicated with congenital heart disease(CHD),patients with isolated CHD and patients with isolated RADs,and to preliminarily explore the safety of immunosuppressants in the complicated population.Methods A total of 34 patients diagnosed with RADs complicated with CHD in The Second Hospital,Lanzhou University from March 2022 to June 2025 were retrospectively enrolled as the complicated group.According to a 1∶2 matching(based on age,gender,type of RADs and time of diagnosis),68 patients with isolated RADs without structural heart disease were selected as the control group(isolated RADs group)from the same period.Another 71 patients with isolated CHD(isolated CHD group)were enrolled in the same period.Baseline data,clinical symptoms,cardiac structure and function parameters,and adverse events were collected and compared among the three groups.Results Patients in the complicated group had diverse types of CHD but generally mild defects,and 91.2％(31/34)of them did not require cardiac intervention,which was in sharp contrast to the isolated CHD group,showing significant phenotypic differences.Cardiac structure and function evaluation showed that patients in the complicated group had more significant cardiac remodeling.The left ventricular end-diastolic diameter and main pulmonary artery diameter in the complicated group were significantly larger than those in the isolated CHD group[45.0(43.0,48.0)mm vs 44.0(41.0,46.0)mm,22.0(22.0,26.0)mm vs 22.0(20.0,23.0)mm](both P＜0.05).Meanwhile,the ejection fraction was significantly lower than that in the isolated CHD group 65％(63％,65％)vs 66％(64％,69％)](P＜0.05),and pulmonary artery systolic pressure was significantly higher than that in the isolated RADs group[27.5(25.0,35.0)mmHg vs 25.0(22.0,25.0)mmHg](P＜0.001).Among 30 patients in the complicated group treated with immunosuppressants,cardiac function indicators remained stable after a median follow-up of 15 months.The incidence of severe infection was 3.3％(1/30),and there were no treatment-related heart failure,thromboembolism or death events.Conclusion Cardiac defects in patients with RADs complicated with CHD are usually milder,and the vast majority do not require cardiac intervention.This is significantly different from the isolated CHD population admitted due to symptoms.In this context,immunosuppressive therapy for controlling RADs shows acceptable safety under close monitoring.A treatment strategy focusing on rheumatic immune disease management and individualized evaluation of cardiac intervention needs should be adopted for this special population.