ObjectiveTo analyze the early- and middle-term prognosis of various surgical methods in children with congenital aortic valve diseases, to provide reference for surgical methods in children with aortic valve stenosis or regurgitation.MethodsThe clinical data of 85 children with various aortic valve diseases treated in the Children’s Hospital of Fudan University from January 2005 to December 2018 were retrospectively analyzed. There were 64 males and 21 females, with an average age of 45 months ranging from 5 days to 15 years. Among them 18 patients underwent balloon aortic valvuloplasty (BAV), 8 surgical aortic valvotomy (SAV), 27 aortic valve autogenous pericardium repair, 16 mechanical arterial valve replacement and 16 Ross operation. They were followed up for 6.25±2.76 years. The re-intervention and survival status after different operations were analyzed.ResultsThere were 3 deaths and 17 reoperations in 85 children. The 5-year survival rate of the patients with SAV, BAV, aortic valve autogenous pericardium repair, mechanical arterial valve replacement and Ross operation was 87.4%, 88.9%, 100.0%, 100.0% and 100.0%, respectively; there was no statistical difference in the early and middle-term survival rates among various operations (P>0.05). The 5-year free from re-intervention rate of the patients with SAV, BAV, aortic valve autogenous pericardium repair, mechanical arterial valve replacement and Ross operation was 44.4%, 18.4%, 100.0%, 66.9% and 80.5%, respectively; there was a statistical difference in the early and middle-term re-intervention rate among various operations (P<0.05).ConclusionThe operation of congenital aortic stenosis or regurgitation needs to be performed according to the pathological changes of the valvular tissues. For children with severe lesions, SAV is recommended for the first intervention. For congenital aortic stenosis, SAV and BAV are both palliative operations which need further evaluation and re-intervention. Ross operation and mechanical arterial valve replacement have low re-intervention rate, and the middle- and long-term follow-up shows that the effect is accurate. Aortic valve autogenous pericardium repair is expected to become a method to delay or replace Ross operation and valve replacement.
Abstract: Objective To summarize onestage repair of coarctation of the aorta(CoA) with intracardiac malformation in infants. Methods From January 2001 to January 2006, 28 patients with CoA and associated intracardiac malformation underwent onestage repair. There were 18 patients with preductal and another 10 patients with juxtaductal CoA. Six patients were accompanied by diffuse hypoplasia of aortic arch and 22 patients were associated with patent ductus arteriosus (PDA). Associated cardiac malformation included ventricular septal defect in 16 patients, ventricular septal defect and atrial septal defect in 5 patients, atrioventricular canal defect in 3 patients, transposition of great artery and ventricular septal defect in 2 patients and double outlet right ventricle in 2 patients. Left posterolateral thoracotomy combined with median sternotomy were used in 12 patients and single median sternotomy was used in 16 patients. End to end anastomosis of aorta with removal of the coarctated segment were performed in 20 patients, Gore-Tex patch graft aortoplasty were performed in 4 patients and aortoplasty with left subclavain flap were performed in 4 patients. Results There was no death.The lasting time of intubation was 7h to 13d, and intensive care unit (ICU) staying time was 3 to 18d. Four patients received delayed sternal closure in 48 to 72h after surgery because of cardiac dilatation. Three patients received peritoneal dialysis for acute renal failure following cardiac surgery. Three patients received inhalation of nitric oxide for treatment of postoperative pulmonary hypertension. The systolic pressure difference between lower limb and upper limb measured after operation was 10 to 20mmHg in 18 patients.There were no obvious systolic pressure difference between arm and leg in 8 patients.The systolic pressure of upper limb was higher than that of lower limb in 2 patients, and the pressure difference was 15mmHg and 20mmHg. All patients were followed up from 3 to 50 months. They were all survival except one died of severe tricuspid regurgitation, pulmonary infection and respiratory failure 3 months after surgery which diagnosis was transposition of great artery and ventricular septal defect. The results of other 27 patients were satisfactory. Recoarctation occurred in 1 patient with end to end anastomosis and 1 patient with Gore-Texpatch graft aortoplasty. The pressure difference through the measurement of echocardiography was 25mmHg and 28mmHg. No reoperation were undergone. Conclusion CoA with intracardiac malformation can be repaired by single median sternotomy or left posterolateral thoracotomy combined with median sternotomy in onestage. This operative approach is beneficial, not only with shorten period of therapy and less operative cost, but also advantageous for the recovery of cardiac and pulmonary function.
ObjectiveTo analyze and evaluate the clinical characteristics, efficacy, and tolerability of lacosamide monotherapy in the treatment of primary paroxysmal kinesigenic dyskinesia (PKD). Methods The Clinical data of children with primary PKD who received lacosamide monotherapy between July 2021 and June 2025 in the Children's Hospital of Soochow University were analyzed, and their efficacy and tolerability were followed up. Results During the study period, a total of 7 children with primary PKD received lacosamide monotherapy. Among them, 4 had simple-type PKD and 3 had complex-type PKD; 2 had familial PKD and 5 had sporadic PKD; 4 were male and 3 were female. The age of onset was 11.0 (0.5 ~ 12.0) years. All 7 cases presented with dystonia as the form of onset, with 1 case also exhibiting athetosis. The attack frequency ranged from once every few days or weeks to 20 times per day, with a duration of 3 ~ 60 seconds. Bilateral limb involvement was most common, and 2 cases also had facial involvement. In auxiliary examinations, 3 cases showed abnormal EEG results, and 4 cases had abnormal genetic test results. Among genetic test results, 2 cases had PRRT2 gene mutations, 1 had a TMEM151A gene mutation, and 1 had a CLCN1 gene mutation. During lacosamide treatment, the initial and long-term maintenance doses were similar, at 2.87 (0.50 ~ 4.44) mg/(kg·d) and 2.67 (0.50 ~ 4.00) mg/(kg·d), respectively. At both the 4-week and long-term follow-ups, all 7 children showed good efficacy and tolerability.ConclusionLacosamide is effective and well-tolerated in children with both familial and sporadic, simple-type and complex-type primary PKD. A low dose of 2.67 (0.50 ~ 4.00) mg/(kg·d) is sufficient to control attacks, which is lower than the minimum maintenance dose of 4 ~ 6 mg/(kg·d) used in the treatment of epilepsy with lacosamide.
