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Objective:To investigate the effect of drug therapy on heart failure caused by supraventricular tachycardia(SVT) in infants.Methods:Fifty-five infants with heart failure caused by SVT, including 24 boys and 31 girls, were treated at Beijing Children′s Hospital of Capital Medical University from January 2014 to December 2021.The drug treatment effects of heart failure caused by SVT were analyzed.Results:The average age of 55 infants at the first diagnosis was 5.8 months(1-11 months). All of them had heart failure, including three cases of atrial flutter, 23 cases of atrial tachycardia(13 cases of disordered atrial tachycardia and ten cases of monomorphic atrial tachycardia), and 29 cases of paroxysmal supraventricular tachycardia.Ultrasonic cardiogram showed that the left ventricular diameter increased and/or left ventricular systolic function decreased.Anti-heart failure therapy was effective in 55 cases(100.0%). Anti-arrhythmic drug therapy: atrial flutter and atrial tachycardia were mainly treated by controlling ventricular rate, digitalis combined with metoprolol was effective in 21 cases(80.8%, 21/26), digitalis alone was effective in four cases(15.4%, 4/26), and sotalol was effective in one case(3.8%, 1/26); paroxysmal supraventricular tachycardia was mainly treated with sinus rhythm conversion.The success rates of conversion were: ATP 20.7%(6/29), ATP combined with digitalis 26.1%(6/23), propafenone combined with digitalis 42.9%(3/7), amiodarone combined with digitalis 60.0%(3/5), and sotalol 92.9%(12/13). During follow-up period for 1 to 12 months, heart failure symptoms of all 55 cases(100.0%) improved, and ultrasonic cardiogram of 53 cases(96.3%)returned to normal.Conclusion:Anti-heart failure and anti-arrhythmic drugs for infants with heart failure caused by SVT need to be selected individually.Atrial tachycardia, especially disordered atrial tachycardia, is sensitive to digitalis.Sotalol can be used to treat refractory SVT.
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Objective:To investigate the effect of radiofrequency ablation on infants with incessant ventricular tachycardia(IIVT) and heart failure.Methods:Twenty-eight infants with IIVT combined with heart failure admitted to Beijing Children′s Hospital of Capital Medical University from January 2006 to December 2021 were selected, including 16 boys and 12 girls; 26 cases were treated with radiofrequency ablation.The characteristics of IIVT and heart failure and the results of radiofrequency ablation were analyzed.Results:The average age of the first diagnosis of 28 infants was 13.9 months old, and all of them had heart failure.Eleven infants had cardiogenic shock, three infants had cardiogenic syncope, two infants had respiratory failure for respiratory support, and one infant died.Color Doppler echocardiography showed that the left ventricular diameter increased and/or left ventricular systolic function decreased.Anti-heart failure treatment was effective in 27 cases(96.4%), electrical cardioversion in five cases, effective in three cases, and anti-arrhythmic drugs were effective in 17 cases(60.7%). Twenty-six cases(92.9%, 26/28) were treated with radiofrequency ablation, with immediate success in 23 cases(88.5%, 23/26) and effective in three cases(11.5%, 3/26). During the follow-up period for 3 to 36 months, cardiac function returned to normal in 25 cases(96.2%, 25/26) and recurred in three cases(11.5%, 3/26 cases), which were cured after radiofrequency ablation again.Temporary complications of atrioventricular block occurred in one case(3.8%, 1/26). In 26 cases of surgical children, 15 cases were measured by X-ray two-dimensional mapping and 11 cases were measured by three-dimensional mapping.The cumulative X-ray exposure was 87.0(51.5, 151.5) mGy and 1.2(0, 15.9) mGy, respectively, and the dose area product was 39.8(19.2, 427.8) μGy/m 2 and 2.8(0, 44.3) μGy/m 2.The cumulative X ray exposure and the dose area product were significantly reduced under the three-dimensional mapping method( P<0.001). Conclusion:The infants with IIVT combined with heart failure are prone to serious complications, and the effects of cardioversion and anti-arrhythmic drugs are limited.Radiofrequency ablation should be performed as soon as possible after heart failure is controlled.The application of three-dimensional mapping technology in surgery can significantly reduce ionizing radiation.
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Infant tachycardia is a critical disease, mainly with supraventricular tachycardia and ventricular tachycardia.The treatment of tachycardia in infant is quite different from that of older children, and there is no relevant guidelines at present.Drug therapy in the acute stage of supraventricular tachycardia and atrial fluttery is mainly intravenous adenosine injection.Digoxin is widely used in neonates.Propranolol is the first choice for prophylactic treatment, and landilolol is in the development stage.Ventricular tachycardia can be spontaneously subsided, the treatment is dominated by intravenous lidocaine.For non-drug therapy, heart cardioerter is the emergency treatment for serious rapid arrhythmia.Radiofrequency ablation is used in infants with more severe conditions and where the onset of tachycardia can not be controlled.Bury cardioverter-defibrillator is effective in preventing infant ion channel disease complicated with malignant ventricular tachycardia induced sudden cardiac death.Subcutaneous implantion of a defibrillator may be superior to intravenous implantation in infants and young children.
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Objective:To summarize the clinical and genetic characteristics of catecholaminergic polymorphic ventricular tachycardia (CPVT) in children caused by CASQ2 gene variants. Methods:The clinical data of 8 children (4 males and females, respectively) with CPVT caused by CASQ2 gene variants admitted to Beijing Children′s Hospital, Capital Medical University from January 2017 to November 2018 were retrospectively analyzed.The targeted next generation sequencing was employed to identify CASQ2 variants and Sanger sequencing was conducted to conform the candidate variants and determine the parental origin. Results:As for 8 children in this study, the average age of onset was 6.4 years, the mean age at diagnosis was 9.4 years, and the average interval from onset to diagnosis was 3 years.Only 2 cases had clearly diagnosis at onset, other 6 cases had a delay to diagnosis and 3 cases of them were diagnosed at other hospitals as having epilepsy and did not respond to anti-epileptic therapy.During physical activity and/or emotional stress, 8 cases presented with recurrent syncope and were able to regain consciousness after a few minutes.They had no a history of sudden cardiac death or family history.There was no abnormality on resting electrocardiogram during the paroxysmal interval in 6 cases and mild sinus bradycardia in 2 cases.Typical bidirectional ventricular tachycardia (VT) and/or polymorphic VT were detected in 8/8 cases and 5/5 cases, respectively, based on Holter electrocardiography and cardiac stress test.The CASQ2 gene variant was found in all children, with 6 cases carrying compound heterozygous variants and 2 cases carrying homozygous variants.A total of 9 different CASQ2 variants were detected in 8 cases, of which 5 had not been previously reported.According to the family-line verification, all of them had a familial variant, with no novel variants.All 8 cases were treated orally with β-blockers, with asymptomatically recurrent episodes, with a mean follow-up of 1.5 years, during which implantable cardioverter defibrillation was performed in 1 case owing to severe sinus bradycardia.There was no death case among them. Conclusions:CPVT with CASQ2 variants is characterized by early onset before preschool age, recurrent syncope after exercise or emotional stress and bidirectional/polymorphic VT.Early diagnosis of CPVT remains challenging due to delayed diagnosis or misdiagnosis.Treatment with β-blockers can achieve favorable effectiveness and safety.Five novel variants in this study would further expand the database of CASQ2 genes.
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Objective:To summarize the characteristics of hypertension and target organ damage in hospitalized children, so as to provide scientific basis for comprehensive prevention and management of hypertension children.Methods:The clinical data of 165 hospitalized children diagnosed with hypertension at Beijing Children′s Hospital from January 2017 to December 2019 were analyzed retrospectively.Data including medical history, clinical symptoms, signs, auxiliary examination and treatment strategy were collected and analyzed statistically.Results:Of 165 admitted hypertensive children who met the inclusion criteria, 35(21.2%)were classified as having primary hypertension, and 130(78.8%)were secondary hypertension.The body mass index(BMI)and the rate of family history of children with primary hypertension were higher than those of children with secondary hypertension, the differences were statistically significant( t=2.052, P=0.026; χ2=3.321, P=0.009). Among 165 children with hypertension, 137 had varying degrees of clinical symptoms(83.0%), only nine primary hypertension cases showed symptoms.The original etiologies of secondary hypertension included 78 cases of renal disease(60.0%), 23 cases of cardiogenic disease(17.7%), six cases of endocrine disease(4.6%)and five cases of rheumatic disease(3.8%). Of all admitted patients, the blood pressure of 158 cases(95.8%)decreased to normal with weight control, low-fat and low-salt diet, antihypertensive drugs and treatment of primary disease.Besides, the parents of seven cases gave up any treatment and left hospital. Conclusion:Secondary hypertension is the main type of hypertension in children, among which renal parenchymal disease is the most common etiology.The clinical symptoms also vary by its original etiology.The overall goal of this study is to reduce the risk of target organ damage, and get blood pressure under control relatively early and effectively.
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Objective:To analyze the clinical characteristics of Kawasaki disease(KD)with complications of acute abdominal disease and to improve the diagnosis, treatment and prognosis of patients.Methods:A total of 2 931 cases with KD hospitalized from January 2016 to December 2019 in our department were retrospectively analyzed, including 14 children with acute abdominal disease(acute abdomen group). And 62 patients with KD but without acute abdominal disease were randomly selected as the control group.The clinical characteristics, laboratory examination and treatment between two groups were compared and analyzed.Results:There were 8 males and 6 females.The average age of patients was(4.46±0.74) years.Compared with control group, there was no significant difference in age, sex and proportion of coronary artery injury in acute abdomen group( P>0.05). Acute abdominal disease group were more likely to have gastrointestinal symptoms, intravenous immunoglobulin(IVIG) resistance and longer fever duration( P<0.05). C-reactive protein(CRP), alanine aminotransferase(ALT), aspartate aminotransferase(AST), γ-glutamyltranspeptidase(GGT) and total bilirubin(TBIL) in acute abdominal disease group were significantly higher( P<0.05). There was no significant difference in white blood cell, erythroayte sedimentation rate, hemoglobin, platelet, Na + , albumin between two groups( P>0.05). All of the 14 patients used IVIG, 3 of them were treated with hormone(methylprednisolone)and 1 patient accepted infliximab.All patients were discharged from hospital.After follow-up for 6 months to 3 years, all patients had no sequelae of digestive system. Conclusion:KD can be complicated with acute abdominal disease, some of which as the first symptom.Gastrointestinal manifestations such as abdominal pain are common.For children with obvious gastrointestinal symptoms, IVIG resistance, and long duration of fever, attention should be paid to the possibility of acute abdominal disease.For children with significantly elevated CRP, ALT, AST, GGT, and TBIL should to be alert to the possibility of acute abdominal disease.KD complicated with acute abdominal disease generally has good prognosis.It is important to treat KD in the first place.
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Objective:To analyze the changes of myocardial damage markers during ventricular tachycardia (SVT) onset and efficacy of various therapeutic approaches.Methods:The clinical data of 256 children with SVT admitted to Beijing Children′s Hospital Affiliated to Capital Medical University from October 2018 to October 2019 were retrospectively analyzed.According to the age of onset, children were divided into groups of 0~3 years old, >3-6 years old, and >6 years old.According to the different heart rates at the time of attack, patients were divided into the groups of <180 times/min, 180~260 times /min, and >260 times /min.According to the treatment methods, children were divided into physical therapy group, drug therapy group and radiofrequency ablation group.Frequency of SVT attacks, heart rate at the onset, serum creatine kinase isoenzyme (CK-MB), troponin (cTn) I, and brain natiuretic peptide(BNP) were collected within 2 h after the onset stop.The recovery rate of each treatment group was counted.Results:Among the 256 cases, there were 135 males and 121 females, which aged from 1 month to 18 years, mean age was (10.2±2.2) years and SVT attacked from 1 to 8 times.The faster the heart rate during SVT attack, the higher CK-MB, cTnI and BNP ( P<0.05). The recovery rate was 27.6% in the mechanical stimulation group, 98.0% in the drug treatment group and 100% in the radiofrequency ablation group, respectively.Successful recovery rate was 47.2%(34 cases)with ATP, 71.7%(109 cases)with Propafenone, 67.3%(64 cases)with Cediland, 59.6%(31 cases)with Verapamil, 61.5%(24 cases)with Sotalol and 54.7%(29 cases)with Amiodarone.Eithty-two cases were successfully converted by two drugs(32.7%) and 38 cases were successfully converted by three drugs(15.1%). Conclusion:When SVT attacks, most children have myocardial damage.The faster the heart rate, the greater the damage in the heart muscle; The treatment of SVT in children should still be the first choice of physical therapy in older children, and if it is not effective, drug therapy should be selected.Radiofrequency ablation is the most effective method to cure tachyarrhythmia.
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Objective To evaluate the efficacy of antiplatelet agents in patients with Kawasaki dis-ease (KD) by using thrombelastography (TEG). Methods A retrospective study of KD patients admitted in our hospital from May 2016 to December 2016 was conducted. Platelet inhibition rates of Arachidonic acid pathway(AA% ) and Adenosine diphosphate pathway were assessed using TEG platelet mapping. The effects of aspirin and dipyridamole on platelet inhibition were compared,and the differences of platelet inhibition rates in different aspirin dose and duration of medication were determined. Results There were significant individual differences in the inhibition of platelets by aspirin and dipyridamole. The inhibition rate of aspirin on platelets[M(P25 ,P75 )] was 62. 45% (35. 58% ,90. 95% ),which was higher than that of dipyridamole [23. 75% (11. 60% ,48. 38% )],there was significant difference (P < 0. 01). The incidence of dipyridamole resistance in children with KD ( 56. 75% ) was higher than that in patients with resistance to aspirin (35. 71% ),and there was significant difference (P < 0. 01). There was a linear correlation between platelet inhibition rates of two antiplatelet agents in children with KD (r = 0. 351,P < 0. 01). There was no significant difference in the effect of aspirin and dipyridamole on platelet inhibition rate after 4 days of administration. There was no significant difference in the effect of different doses of aspirin on AA% . Conclusion TEG is an effective way to evaluate the efficacy of antiplatelet therapy in children with KD.
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Objective To investigate the clinical features and prognostic factors in children with idi-opathic pulmonary arterial hypertension(IPAH). Methods The data of children with IPAH diagnosed in Beijing Children′s Hospital from January 2006 to December 2015 were collected. The cases were divided into survival group and death group according to the prognosis. The risk factors influencing prognosis in children with IPAH were identified by Logistic regression analysis. Results Total 26 patients were enrolled in this study,and 17 of them survived and 9 of them were dead. IPAH was found to be more common in school-age children. The average age of the patients was(6. 6 ± 3. 6)years and the male to female ratio was 1. 17∶1. All of the children had the symptoms with decreased activity tolerance and fatigue. The incidence of syncope and dyspnea and edema of lower extremity were 34. 6%,23. 1%. Accentuated pulmonic second sound(P2) was detected in 92. 3% of patients during physical examination, which was also the most common sign. About 50. 0% patients were functional class Ⅲ-Ⅳ. Echocardiography showed that all childrens′ mean pulmonary artery systolic pressure(PASP) was(79. 0 ± 20. 3) mmHg(1 mmHg=0. 133 kPa) and 16 of them with right ventricular dilatation. Electrocardiogram with ST-T changes and right ventricular high voltage were common. The average plasma level of brain natriuretic peptide(BNP) in all cases was(870. 4 ± 720. 9) pg/ml. The single factor analysis illustrated that syncope(OR=26. 25,95%CI 3. 04 -226. 60,P=0. 003),heart func-tional class Ⅲ-Ⅳ(OR=19. 199,95%CI 1. 88 -196. 53, P=0. 0128), PASP≥70 mmHg(OR =9. 936, 95%CI 1. 81-∞,P=0. 005),BNP≥850 pg/ml(OR=59. 991,95%CI 4. 69-767. 62,P=0. 002) indica-ted the worse outcome. Conclusion IPAH in children is rare and the prognosis is poor. Syncope, cardiac function grade,PASP and the levels of BNP are the main important factors affecting the prognosis of children with IPAH. BNP has important clinical significance for the evaluation of IPAH and the prognosis of the disease.
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Objective To observe the clinical characteristics and prognosis in children with high take -off coronary artery.Methods The clinical characteristics,laboratory examinations and prognosis were retrospectively ana-lyzed in 49 children with high take -off coronary artery who had been admitted to Beijing Children′s Hospital Affiliated to Capital Medical University from April 2009 to December 201 2.Results Among 49 medical records of children with high take -off coronary artery,34 cases were male and 1 5 cases were female,with a mean age of (9.28 ±4.42)years old,and the youngest patient was 1 3 months old,and the oldest patient was 1 7 years and 2 months old.The 64 -section multidetector CT angiography of all patients showed one or both coronary arteries originated from 1 .0 cm higher than the junction area of sinus and aorta,including 38 high take -off of the left coronary artery,7 high take -off of the right coronary artery and 4 high take -off of both coronary arteries.The chief complaints of the patients included chest pain (22 cases,44.90%),chest tightness(1 3 cases,26.53%),declining of exercise tolerance(7 cases,1 4.29%),fatigue (5 cases,1 0.20%),palpitation(3 cases,6.21 %),syncope(1 case,2.04%).Electrocardiogram(ECG)of those pa-tients showed ST -T changes,atrioventricular block,sinus tachycardia,pathological Q -wave,proiosystole and bundle branch block.Ultrasonic cardiogram(UCG)indicated 1 4 patients with the enlarged left ventricle,including 2 patients with ejection fraction lower than 60%.After 1 0 days hospitalization,the anesis of chest pain and chest tightness were significantly different statistically(P <0.05),but there was no significant difference in ECG and UCG.Thirty -nine out of 49 children were followed for 1 year.After 3 months,6 patients with ST -T problem showed normal ST -T segment. After 6 months,1 0 patients with ST -T problem showed normal ST -T segment.After 1 year,1 7 patients with ST -T problem showed normal ST -T segment.Conclusions High take -off coronary artery is a rare malformation of the coronary artery,which can cause myocardial ischemic,leading to multiple clinical manifestations and may result in ECG changes and the left ventricle enlargement.High take -off coronary artery can be confused with myocarditis and myocar-dial lesion clinically.Patients receiving systemic treatment may have their symptoms released,but ECG and UCG may not relieve in a short term.Patients with high take -off coronary artery need long -term observation.
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Objective To report one case of the children's acute rhabdomyolysis(RM) with myocardial hypertrophy for the first time and the related data in order to improve the clinical diagnose and the treatment efficacy.Methods Clinical data of the patient with myocardial hypertrophy and pericardial effusion treated in the Heart Center of Beijing Children's Hospital Affiliated to Capital Medical University were retrospectively analyzed.According to the symptoms,signs,laboratory examination,the diagnosis was made and related literature was reviewed.Results A 7-years-old girl was characterized by myalgia,weakness,and dark urine.Physical examination showed extremity swelling,clammy skin,lower extremity muscle tenderness positive.Creatine kinase (CK) and myoglobin levels increased.So she was clearly diagnosed of acute RM undoubtedly.Without physical,metabolic and autoimmune factors,the patient was only had positive mycoplasma antibodies and might be coursed by mycoplasma infection.The muscular involvement was so severe that the patient had the heart failure,myocardial hypertrophy and pericardial effusion was conformed to ultrasound cardiogram(UCG).After treatment with hydration,alkalization,anti-infection,support and symptomatic therapy for a few days,the clinical symptoms improved obviously,CK returned to normal,myocardial hypertrophy and pericardial effusion disappeared.One-year-follow-up study indicated that the child's animal force and body movements were norrmal,and CK and UCG were negative.Conclusions Acute RM is a rare disorder and less recognized in children,and myocardial hypertrophy and pericardial effusion may be caused by acute RM.If the diagnosis and treatment are done earlier,the prognosis will be good,without permanent complications.