Application of targeted drugs in diagnostic treatment of severe pulmonary hypertension associated with left-to-right shunt congenital heart disease and its values in surgical indication selection

Wang Zhiyi; Jing Xiaoyong; Yang Xueyong; Li Gang; Su Junwu; Liu Yinglong; Fan Xiangming

doi:10.3760/j.issn.1673-4777.2018.05.016

Abstract：Objective To evaluate the value of targeted drugs in diagnostic treatment and surgical indication selection for left-to-right shunt congenital heart disease patients with severe pulmonary hypertension.Methods Clinical data of 55 left-to-right shunt congenital heart disease patients with severe pulmonary hypertension were reviewed from January 2011 to June 2017 in Beijing Anzhen Hospital,Capital Medical University.All patients were treated with bosentan on the basis of cardiotonic and diuretic agents;patients with no significant response to bosentan had subcutaneous/intravenous infusion of prostacyclin drug (treprostinil).Patients who showed surgical indications after diagnostic treatment had radical operation;patients without surgical indications and patients with residual pulmonary hypertension after operation were treated with long-term drug therapy and had regular echocardiography reexamination.Results Thirty-two patients(58.2％) had bosetan monotherapy;the level of SpO2 increased from 86％-94％ [(91.5 ± 2.2)％] to 95％-99％ [(97.1 ± 1.7)％] after treatment.Twenty-three patients(41.8％) had sequential targeted drug therapy;the level of SpO2 in 12 patients increased from 70％-94％ [(89.4 ± 8.2) ％] to 95 ％-99％ [(96.6 ± 2.1) ％] after treatment;the level of SpO2 in 4 patients with complex congenital heart disease increased from 68 ％-79％ [(72.1 ± 4.3) ％] to 82％-91％ [(86.0 ± 6.0) ％] and the average rise level was (17.7 ± 3.4) ％;the level of SpO2 in 7 patients remained at 78％-92％ [(89.1 ± 5.3) ％] after treatment.Forty-eight patients(87.3％) had surgical radical operation after diagnostic treatment without perioperative death and recovered well;7 patients(12.7％) continued to be treated with target drugs.All patients were followed up for 3-6 months;pulmonary artery pressure recovered to normal in 6 patients;37 patients recovered to mild-to-moderate pulmonary hypertension;5 patients had residual severe pulmonary hypertension after surgery.Conclusion Diagnostic treatment with combination of cardio-diuretic agents and targeted drugs provide patients who do not conform to surgical indications with opportunity and possibility to have radical surgery,and it is of great significance to reduce postoperative residual pulmonary artery pressure and improve the quality of life.