The clinical effect of lung transplantation for pediatric pulmonary artery hypertension / 中华胸心血管外科杂志

ABSTRACT

Objective To evaluate the clinical effect of lung transplantation for pediatric pulmonary artery hypertension (PAH).Methods The recepter 1 and 2 with idiopathic pulmonary artery hypertension (IPAH) were diagnosed by right catheterization.The heart fuction was NYHA IV.The pulmonary artery pressure and mean pulmonary artery pressure were 110/70mmHg and 148/72 mmHg respectively.They underwent bilateral sequential lung transplantation with extracorporeal membrane oxygenation (ECMO) support.During operation,the ECMO support time was 550 min and 450 min and the blood loss was 3000 ml and 1200 ml respectively.The recepter 3 with end-stage congenital ventricular septal defect with Eisenmenger syndrome (ES) who had received open heart exploration underwent right single lung transplantation and ventricular septal defect repair under cardiopulmoanry bypass(CPB).There were ventricular septal defect and bidirectional shunt through UCG assessment The pulmonary artery pressure and mean pulmonary artery pressure were 110/60 mmHg.CPB time was 244 min.The three recepters had the same ABO blood group and the similar body type with the three donors.Results The recepter 1 and 2 was sustained by ECMO after operation for 16 h and 13h respectively.But unstable hemadynamics and acute left heart failure occured on 3rd and 4th day after the operation respectively.We treated them with ventilate support and tracheotomy on 3rd and 6th day respectively.They were also treated with cardiotonic; dieresis and the patients were weaned away from the ventilation on 33rd and 12ed day after the operation respectively.The transplanted lung of the receptor 3 had pneumochysis in the first 3 days.The patient was treated with ventilate support and tracheotomy on 7th day and was weaned from the ventilation on 12ed day after the operation.An acute rejection episode occurred on 14th day.The cordioform and heart function of the three cases improved and especially the receptor 3 had intact repaired of ventricular septal defect.They were discharged from the hospital on 93rd,32ed and 62ed day afer the operation.The heart function all reached NYHA I and the pulmonary artery pressure and mean pulmonary artery pressure was reduced to 54/32 mmHg,60/36 mmHg and 53/39 mmHg respectively.The three cases have been followed up for 41 months,21 months,and 82 months.They are having an excellent quality life.Conclusion Lung transplantation is effective to improve the quality of life for end-stage pediatric pulmonary artery hypertension even with slight right ventricular dysfunction with satisfying short-term results.