Long-term Follow-up Of Patients With Persistent Pulmonary Hypertension After PDA Interventional Closure And Detection Of EGFR Gene Mutation In Patients With Pulmonary Hypertension

1 Patent ductus arteriosus with persistent pulmonary artery hypertension after transcatheter closureBackground:Transcatheter closure(TCC)of Patent ductus arteriosus(PDA)with various occluders has been well established and become the treatment of choice for the majority of patients.However,whether to attempt TCC of PDA in patients with severe pulmonary artery hypertension remains as a challenging clinical problem.Especially,there is not much information on the immediate and long-term effects of TCC of a PDA in patients who already have PAH,especially in patients with persistent pulmonary artery hypertension after occlusion.Methods:A chest x ray,an electrocardiogram and an echocardiogram were performed on all patients at 24 hours,one month,six months and 1 year intervals serially.During the follow up,pulmonary artery systolic pressure(PASP)was obtained from echo/Doppler study.Results:There was a significant fall(p<0.05)in mean PASP after occlusion(to 59.3±12.7mmHg).However,the aortic pressure and SaO2 changed slightly(p>0.05).During the follow up,there was a further fall of PASP in 5 patients(NO 1,5,6,7 and 8).Four patients(NO 2,3,4 and 9)showed the evidence of worsening of the PAH and were treated with sildenafil.Patient 2 died from acute right heart failure after a period of 11 months from the time of TCC,triggered by pulmonary infection.Conclusions:Some patients with borderline hemodynamic data with PDA and PAH can deteriorate or keep sustained PAH after PDA closure.The treatment of permanent closure to these patients must be cautious.2 the Detection of Epidermal Growth Factor Receptor Mutation in Patients with CHD-PAHBackground:PAH is now regarded as a disease caused mainly by pulmonary vascular remodeling.The increased proliferation,migration,and survival of pulmonary vascular cells within the pulmonary artery wall in PAH have allowed successful transposition of pathophysiological elements from oncologic researches.Plateletderived growth factor(PDGF),epidermal growth factor(EGF)and fibroblast growth factor,are emerging as important players in the pathomechanism,suggesting that the field of growth factors and their receptor tyrosine kinases(RTKs)as targets for treatment of cardiopulmonary diseases is expanding.In recent years,the EGFR/ErbB family of RTKs has turned out to be critically involved in many human cancers and is already the target of several anticancer therapeutics.However,EGFRs are activated either by mutation or by interacting with their ligands.Methods:We collected 23 plasma samples with ES who were hospitalized from August 2008 to may 2015,including 11 cases of patent ductus arteriosus,9 cases of atrial septal defect and 3 cases of ventricular septal defect.Free DNA was isolated from plasma.EGFR gene was detected by fluorescence quantitative PCR.Results:Somatic mutations in EGFR gene in plasma were identified from 2 of 23(13%)patients.Among the 2 mutants,1 showed p.L858R mutation and 1 showed p.E 19-del mutation.Conclusions:p.L858R and p.E19-del gene mutations were found in patients with ES.