| Background:Chronic thromboembolic pulmonary hypertension,mainly caused by acute pulmonary embolism,was characterized by fibrothrombotic obstructions of large pulmonary arteries and microvascular disease.The purpose of this study was to compare the characteristic and long-term outcome of patients who had chronic thromboembolic pulmonary hypertension treated in three distinct ways which were pulmonary endarterectomy,balloon pulmonary angiography and drug therapy.Meanwhile,we also aimed to evaluate outcome correlates in these patients.Methods:Patients diagnosed with chronic thromboembolic pulmonary hypertension were continuously and prospectively included in a Chinese registry which was set up in 19 centers from August 2009 to July 2018.Characteristics included symptoms,sign,comorbidities and hemodynamics were collected in all patients.The diagnosis was based on the following criteria:pulmonary hypertension was confirmed by right heart catheterisation(mean pulmonary arterial pressure?25mmHg and pulmonary capillary wedge pressure ?mmHg at rest);mismatch on ventilation/perfusion scintigraphy(usually ventilation/perfusion single-photon emission computed tomography)with at least one large perfusion defect in one segment or in two subsegments,or evidence of pulmonary vascular lesions on computed tomography and/or magnetic resonance imaging or pulmonary angiography;all these above findings should be obtained after at least 3 months of effective anticoagulation.Characteristic and survival of patients with chronic thromboembolic pulmonary hypertension in different treatments were compared.Prognostic factors were evaluated by cox regression model.Results:A total of 593 patients were included.Of whom,81 were treated by pulmonary endarterectomy(group 1),61 treated by balloon pulmonary angiography(group 2),451treated by drug therapy(group 3).58.0%of the Patients had a histoty of acute pulmonary embolism.Compared with the other two group,operated patients were younger,mainly constituted of male and had less comorbidities.59.0%of them were in World Health Organization functional class ? and ?.Estimated survival at 1,3,5 and 8 years was 95.2%(95%CI,94.3-96.1),84.6%(95%CI,82.9-86.3),73.4%(95%CI,71.0-75.8),66.6%(95%CI,63.5-69.7)in all patients;92.6%(95%CI,89.7-95.5),89.6%(95%CI,86.1-93.1),87.5%(95%CI,83.5-91.5),80.2%(95%CI,72.3-88.1)in operated patients;95.4%(95%CI,94.4-96.4),83.3%(95%CI,83.3-83.3),71.0%(95%CI,68.2-73.8),64.1%(95%CI,60.6-67.6)in not-operated patients.While the estimated survival rate at 1,3,5 and 7 years in patients treated with balloon pulmonary angiography was 96.7%(95%CI,94.4-99.0),88.1%(95%CI,82.9-93.3),70.0%(95%CI,68.8-71.2),70.0%(95%CI,68.8-71.2),respectively.For all Patients,the surgery of pulmonary endarterectomy was the strongest independent predictor of survival(hazard ratio[HR],0.134;95%CI,0.031-0.575,P=0.007),while coronary heart disease was the strongest independent predictor of death(HR,6.232;95%CI,1.78-21.816,P=0.004).Other independent risk factors were cardiac index,pulmonary vascular resistance,big endothlin-1,acute pulmonary hypertension and six minutes walking distance.However,mortality was associated with pulmonary vascular resistance(HR,6.508;95%CI,1.403-30.185,P=0.017),cardiac index(HR,0.161;95%CI,0.029-0.899,P=0.037)and carbon monoxide diffusing capacity(HR,0.408;95%CI,0.173-0.996,0.041)in operated patients.While in drug therapy patients,mortality was associated with pulmonary vascular resistance(HR,1.48;95%CI,1.149-1.907,P=0.002),cardiac index(HR,0.372;95%CI,0.195-0.707,P=0.003),mean pulmonary arterial hypertension(HR,1.429;95%CI,1.015-2.011,P=0.041),mixed venous oxygen saturation(HR,0.712;95%CI,0.518-0.98,P=0.037),big endothlin-1(HR,4.576;95%CI,1.155-18.123,P=0.03)and coronary heart disease(HR,7.168;95%CI,1.988-25.847,P=0.003).Furthermore,in patients treated with balloon pulmonary angiography,mortality was associated with warfarin(HR,0.112;95%CI,0.015-0.812,P=0.03)and inferior vein cava filter(HR,25.612;95%CI,2.084-314.714,P=0.011).Conclusion:Baseline characteristics and estimated survival in patients with chronic thromboembolic pulmonary hypertension of China is similar to the Western country.Long-term survival of operated patients was better than the prognosis of not-operated patients.However,Bridge therapy before pulmonary endarterectomy could not improve the survival of patients,but prolonged the time from diagnosis to pulmonary endarterectomy.In not-operated patients,off-label pulmonary arterial hypertension-targeted therapy could improve 4 years-survival.For all patients,pulmonary endarterectomy was the strongest independent predictor of survival.Additional correlates of mortality were cardiac index,pulmonary vascular resistance,big endothlin-1,six minutes walking distance and comorbidity of coronary heart disease. |
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