Surgical Treatment Of Chronic Thromboembolic Pulmonary Hypertension: Long-term Results And Risk Factor Analysi

Objectives:Chronic thromboembolic pulmonary hypertension(CTEPH),combined with pulmonary artery sarcoma(PAS)and isolated pulmonary vasculitis(IPV),is categorized as Group 4 in the classification of pulmonary hypertension.The definitive treatment for CTEPH is pulmonary endarterectomy(PEA),which has not widely developed both at home and abroad.This study aimed to evaluate the early and long-term outcomes of PEA in CTEPH patients,analyze risk factor for residual pulmonary hypertension(RPH)and long-term survival in Fuwai hospital.Meanwhile,this study reports the experience of our center in the PEA surgical management of PAS and IPV,both of which are infrequent.Methods:Patients were evaluated retrospectively and classified into early(1997-2014)and late(2015-2021)groups.The clinical characteristics and perioperative outcomes of the two groups were compared,and risk factor analysis for RPH and long-term outcome for all cases was performed.In addition,the medical records for PAS and IPV were retrospectively reviewed to evaluate the clinical characteristics,operative findings,the postoperative outcomes and the long-term results.Results:(1)Between March 1997 and December 2021,253 CTEPH patients were treated at our center with PEA.There was no statistically significant difference in demographics between the two groups.However,the Early Group had a significantly higher rate of perioperative death(9.8%versus 1.2%,P=0.001),RPH(48.8%versus 14.0%,P<0.001),and reperfusion pulmonary edema(18.3%versus 2.9%,P<0.001).The median follow-up time was 66.0 months,and overall survival rates at 5,10,15,and 18 years after PEA were 91.2%,83.9%,64.5%,and 46.0%,respectively.Age and postoperative systolic pulmonary artery pressure(sPAP)were independently related to long-term outcomes in the multivariate Cox analyses.Patients with postoperative sPAP no less than 46 mmHg had a lower chance of survival.(2)Between 2000 and 2022,26 patients underwent operations for PAS at our center.Six patients underwent tumour resection alone,three patients underwent tumour resection combined with pulmonary artery vascular replacement,whereas the other 17 patients received tumour resection and PEA.There were 3 perioperative deaths.Follow-up was completed for all the other patients with median time of 18 months(1-78 months).For all patients,the median postoperative survival was 36 months,and estimated cumulative survival rates at 1,3 and 5 years were 73.7%,49.1%and 21.0%,respectively.The mean survival was 44.6 months after PEA and 16.3 months after tumor resection only(P=0.01).(3)There were 7 occlusive IPV patients underwent operation between January 2018 and June 2022 in Fuwai hospital.All patients underwent PEA,while patch angioplasty was performed in four patients with severe pulmonary artery trunk stenosis.One patient died from RPH after limited PEA of a transmural vascular lesion.In addition,no obvious surgical complications were observed.Three months after PEA,a substantial relief in symptoms was achieved,while the mean pulmonary artery pressure(preoperation 33 mmHg versus post-operation 21 mmHg;P=0.018)and pulmonary vascular resistance(pre-operation 234 dyn.s.cm-5 versus post-operation 180 dyn.s.cm-5;P=0.310)decreased.Conclusions:PEA improved CTEPH hemodynamics immediately and had a positive effect on long-term results.However,the outcomes correlated with surgical experience.Patients with postoperative sPAP≥46 mm Hg indicate clinically significant RPH and have a lower long-term survival rate.Superior to tumor resection alone,PEA surgery with PH relieved provide better chance of survival.PEA combined with patch angioplasty for pulmonary artery trunk is a valuable choice for treating occlusive IPV,with notable hemodynamic and clinical advantages.