| Background and Objective:Pulmonary arterial hypertension (PAH) is a pathophysiological syndrome characterized with elevated pulmonary artery pressure and pulmonary resistance and caused by all sorts of pathogenesisa. Congenital heart disease associated with pulmonary arterial hypertension (CHD-PAH) is due to cardiac anatomic deformity, that lead to intracardiac shunting of blood, hemodynamic changes, too much blood flow in right ventricular, the state of relative more blood of pulmonary circulation and finally the increase of pulmonary arterial pressure. Since 1990s, the drugs for pulmonary arterial hypertension gradually become targeted therapy drugs, including sildenafil, prostacyclin, bosentan and so on. A series of clinical studies on targeted agents in pulmonary arterial hypertension has indicated that these drugs could improve hemodynamics and improve exercise tolerance, and most of clinical observations have found that it’s beneficial to improve long-term prognosis. Sildenafil is a phosphodiesterase type 5 inhibitor and Beraprost sodium belongs to prostacyclin. In theory, the combination of Sildenafil and Beraprost sodium can decrease pulmonary artery pressure effectively because they work in different ways. Unfortunately, Sildenafil and Beraprost sodium have not been applied for the treatment of pulmonary artery hypertension. So we choose the cheaper drug targeting, Sildenafil and Beraprost sodium, which are relatively cheap, for the treatment of congenital heart disease with pulmonary hypertension, observe the therapeutic effects of such combination and related complications, supply condition and reference to treat pulmonary artery hypertension.Clinical data and research methods:All cases were from the Second Affiliated Hospital of Zhengzhou University, during January in 2010 to September in 2011. A total number of 21 cases, including 9 men and 12 women is at the age of 8 to 46 years (average age 31+/-8 years). There are 3 cases of the atrial septal defect (ASD),10 ventricular septal defects (VSD),3 patent ductus arteriosus (PDA),4 atrial septum associated with ventricular septal defects and 1 ventricular septum and patent ductus arteriosus. All patients underwent bidirectional shunt, including 11 cases with mainly left-to-right shunt,10 people with mainly left-to- right shunt. According to the cardiac function standard of New York Heart Association,7 patients were gradeⅡ,10 patients were grade III,4 patients were grade IV.Patients was gave oxygen by mask before hand (2～5L/min,2h/time,3 times/ day) after admission, took continuously Sildenafil (0.5mg/kg,3 times a day) and Beraprost sodium tablets (20～40 ug/time,3 times one day) from the next day. To take various datum, blood flow velocity with tricuspid regurgitation (m/s), bidirectional shunt flow velocity (m/s) that includes the left-to-right and right-to-left flow velocity and evaluate the pulmonary artery systolic pressure (sPAP) by ultrasonic cardiography, anterior right ventricular wall. At 1 hour and 2 hours post taking Beraprost and Sildenafil, the heart rate, blood pressure, femoral artery blood gas analysis, transcutaneous oxygen saturation with distal extremities (SPO2) and the content of cAMP and cGMP were detected while quiet, inhaling oxygen and lying down at the second or third day after admission. Ultrasonic cardiography and transcutaneous oxygen saturation with distal extremities (SPO2) were checked at 3, 6 months after taking drugs. Patients admitted to the hospital after the assessment indexes of cardiac function during hospitalization, and tests ProBNP.6 minutes walk test, percutaneous distal extremities blood oxygen saturation index, at the same time in 6 months when the project review. In all patients,10 cases had right heart catheterization.Results were expressed mean±S.D and finally statistically ananlyzed using SPSS 17.0. The differences between before and after treatment were analyzed with paired-t test and hierarchical data (heart function classification changes) were done with non-parametric rank test with ststistical significance level of 5% (P<0.05).Results:The SPO2, PO2, cAMP, cGMP are different statistically between 1hours after medication and the base value. Differences of SPO2, PO2, cAMP, cGMP between 2 hours after drugs and basic value were extremely significant (P<0.01). There were significantly statistical difference between 1 and 2 hours after taking drugs in cGMP (P< 0.01), and so did the SPO2 (P< 0.05). For 6 months later(n=15) and the base value of the phase comparison, three tricuspid regurgitation velocity (4.68+/-0.55 m/s reduced to 4.07+/-0.37m/s), right ventricular anterior wall thickness, estimation of pulmonary artery systolic pressure (94.76+/-16 mmHg reduced to 77.50+17.54mmHg). For 6 months, the base value of transcutaneous oxygen saturation compared limbs (79.52+/-2.06 to 84.12+/-2.89). For 6 months, the cardiac function of the patients with level reduced (2.86+/-0.7 reduced to 2.4+/-0.5), improved cardiac function (ProBNP from 780+/-910 pg/ml reduced to 325+/-531 pg/ml),6 minutes walking distance (269+/-110 m increased to 380+/-93 m). For 6 months, right heart catheterization results mean pulmonary artery pressure (80+/-25 mmHg reduced to 70+/-16 mmHg), pulmonary blood flow index (3.1+/-1.5 to 3.5+/-1.2), pulmonary vascular resistance index (19+/-10 reduced to 15+/-8).Conclusions:1 Combined with sildenafil and beraprost therapy in severe pulmonary hypertension can increase cAMP and cGMP, increased in patients with transcutaneous oxygen saturation, correct hypoxemia.2 Combined with sildenafil and beraprost sodium in 6 months, can improve the cardiac function of the patients with increased,6 minutes walking distance.3 Combined with sildenafil and beraprost sodium in 6 months, can reduce the mean pulmonary arterial pressure, increased pulmonary vascular blood flow index, lower pulmonary vascular resistance index. |
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