Clinical Analysis For 33 Children Of Cardiac Rhabdomyoma

Objective: To summarize and analyze the clinical data of pediatric cardiac rhabdomyoma(CR),and to explore the clinical characteristics of CR and the relationship with children's tuberous sclerosis(TSC),so as to provide reference for clinical diagnosis and treatment.Methods: Clinical data of 33 CR children diagnosed and treated in our hospital from January 2005 to December 2018 were collected and described.The age of onset,growth characteristics,clinical manifestations and treatment of CR were summarized.Survival and tumor changes were followed up for 2.5 months to 8 years and 6 months.Results: 1.CR initial diagnosis age: 3 cases were found in the pregnancy examination,the rest were 5 hours to 11 years and 6 months,the median age was 5 months and 5 days,and the most cases were found in the age group within 1 year(23/33 cases,69.7%).2.A total of 91 tumors were detected at the initial diagnosis,including left ventricular wall 31/91(34.1%),interventricular septum 25/91(27.5%)and right ventricular wall 23/91(25.3%),which also involved atrium and valve.There were 14/33 single cases(42.2%)and 19/33 multiple cases(57.6%).Left and right ventricular outflow tract obstruction in 2/33 cases each(6.1%).3.At the initial diagnosis,the most patients with convulsion were 18/33 cases(54.8%),6/33 cases(15.2%)with cardiovascular system performance,and 9/33 cases(30.3%)with other system performance,among which 22/33 cases(66.7%)were diagnosed as TSC.4.2/33 cases(6.1%)of the children received surgical treatment,including 1 case of total tumor resection,followed up for 2.5 months without recurrence,1 case of partial resection,treated with sirolimus orally,followed up for 41.5 months with complete tumor regression.31/33 cases(93.9%)without surgery,UCG was reviewed in 7/31 cases(22.6%),among which 1 case had complete tumor regression,1 case had partial regression,1 case had no significant change,3 cases had new tumor,and 1 case had tumor enlargement.Telephone follow-up was conducted in 20/33(60.6%)patients,of whom 18/20(90%)survived and 2/20(10%)died.Conclusion: CR is more common in infants and children,can be multiple or single,most located in the ventricular wall or interventricular septum,a few involving atrium and valve,lead to ventricular outflow tract obstruction may cause heart failure,involvement of the conduction system can appear arrhythmia,severe cases can lead to neonatal death.Patients with obvious symptoms or obstruction can be surgically resected to reconstruct normal hemodynamics,and most of the children who survive the perioperative period safely have a good long-term survival.For children who cannot tolerate the risk of surgery,mTOR inhibitors may be another option if combined with TSC.CR has a tendency of natural regression,but it still may be increased or newly developed.Therefore,children with CR should be followed up regularly for a long time.There is a close relationship between CR and TSC.For children whose CR is found at a young age,especially in the fetal or neonatal period,it is recommended to screen for TSC as soon as possible.For children with TSC,UCG examination should be performed at least once at the first diagnosis,and then regular re-examination should be conducted in case of new lesions.