Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease

Background:Pulmonary artery hypertension associated withh congenital heart disease(PAH-CHD)occurs predominantly among patients with uncorrected CHD.Treatment of severe pediatric PAH-CHD remains a major intractability.This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction.Methods:The data for 59 children with severe PAH-CHD who underwent surgical correction,with or without postoperative medication,between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively.A regression analysis,receiver-operating characteristic(ROC)curves,and Kaplan-Meier curves were used for survival analysis.Results:Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction,with or without specific anti-PAH drugs postoperatively,were included in this study.The pulmonary pressure,heart function,and ending events were observed and median observation period was 49±20 months.Twenty-eight patients(50%)received at least one additional anti-PAH drug after correction.The survival rate after 2 years was 91.5%(54/59);two patients were in a critical condition,and three were lost to follow-up.Twelve patients(29%)still received over one additional PAH-specific therapy at follow-up,whereas 42(75%)had successfully stopped drug treatment.Two patients(3.5%)died and one underwent a second thoracotomy to remove the ventricular septal defect patch.Acute vasoreactivity test(AVT)criteria had limited efficacy in predicting pediatric PAH-CHD,whereas pulmonary vascular resistance(PVR)<6.65 Wood units(WU)/m^2 or PVR/systemic vascular resistance(SVR)<0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3%(95%confidence interval[CI]:96.0-100%),98.4%(95%CI:96.0-100%)sensitivity of 100%,100%and specificity of 82.1%,92.9%,respectively.Conclusions:Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD,PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD.Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR<6.65 WU/m2 and PVR/SVR<0.39 after iloprost aerosol inhalation.

Follow-up of percutaneous transcatheter closure of pulmonary arteriovenous fistulas

To the Editor:Pulmonary arteriovenous fistula(PAVF)is abnormally dilated vessels that bypass the lung capillary bed,providing a direct right-to-left shunt:.The clinical manifestations of PAVF can vary from asymptomatic to dyspnea on exertion,fatigability,cyanosis,and neurological complications.Patients with congenital PAVF are often associated with hereditary hemorrhagic telangiectasia(HHT)and congenital heart disease.The primary aim of treatment through transcatheter techniques or surgery is to reduce or abrogate abnormal shunt and to prevent severe complications.The development of medical apparatus and instruments has expanded the in dications of interventional closure of PAVF.However,data on the efficacy and safety of transcatheter closure of PAVFs is still lacking.

Pediatric Hemorrhagic Stroke Complicates Interventions for Congenital Heart Disease:Experiences from Two Centers

To the Editor:Pediatric stroke refers to the rupture and/or occlusion of arteries or veins.It is one of the highest causes of pediatric preterm death and long-term disability.Health-care providers find dealing with pediatric stroke a challenge because of the difficult diagnosis,the lack of standard guidelines,the rapid progress,and the high mortality.In this study,we observed and analyzed the characteristics of six pediatric hemorrhage strokes complicated with interventions for congenital heart disease (CHD).We found that vascular malformation,hemodynamic fluctuation,and postoperative anticoagulant medicines were risk factors for this condition and that there was an urgent need for awareness and acknowledgment.