Objective: The growing body of literature showed a link between uric acid and pulmonary hypertension (PH), but the impact of hyperuremia on outcome of patients with PH has not been well defined. Therefore, the present study was performed to analyze the impact of uric acid on outcome of PH patients. Methods: One hundred seventy-three PH patients (112 females, mean age 38 years old), who were hospitalized in our department between January 2010 and December 2015, were included in our study, the PH diagnosis was made based on right heart catheterization examination result (mean pulmonary artery pressure≥25 mmHg(1 mmHg=0.133 kPa)). PH patients were divided into mild to moderate PH group (Rp/Rs≤0.6, n=97) and severe PH group (Rp/Rs>0.6, n=76). Fifty-one patients (33 females, mean age 45 years old) without PH based on right heart catheterization were included as control subjects. All participants were followed up for a median of 24 months(6-71 months). Clinical endpoints were defined as cardiogenic death or heart-and-lung transplantation. Results: Uric acid was positively correlated with pulmonary vascular resistance(r=0.398, P<0.01), systemic vascular resistance(r=0.244, P<0.01) and mean right atrial pressure (r=0.26, P<0.01), and was negatively correlated with cardiac index(r=-0.278, P<0.01)and mixed venous oxygen saturation (r=-0.322, P<0.01)in PH patients. Serum uric acid level was significantly higher in patients with severe PH than in patients with mild-to-moderate PH and the control subjects (both P<0.05). According to the receiver operating characteristic curve (ROC), 425.5 μmol/L was found to be the best cut-off value of serum uric acid level to predict the outcome of PH patients (sensitivity 50%, specificity 72%). During follow-up, patients with higher level of uric acid (>425.5 μmol/L) were linked with poorer clinical outcome compared to patients with uric acid <425.5 μmol/L(P=0.027). Conclusion Our findings suggests that uric acid is associated with the severity of PH and higher uric acid level serves as an important predictor for poor clinical outcome of PH patients.

OBJECTIVETo evaluate the therapy efficacy of iloprost combined with low dose tadalafil in adult congenital heart disease (CHD) patients with severe pulmonary arterial hypertension (PAH).

METHODSAdult CHD patients with severe PAH were included and divided into the sequential combination therapy group [iloprost: 10 µg/inhalation, 6 times per day for 6 months, and then add oral tadalafil (5 mg/d) till 12 months, n = 32] and upfront combination therapy group [iloprost: 10 µg/inhalation, 6 times per day combined with oral tadalafil (5 mg) for 12 months, n = 36]. Data on 6 min walking test (6MWT), Borg dyspnea score, oxygen saturation measurement, WHO classification, and cardiac catheterization were obtained at baseline, 6 and 12 months.

RESULTSSeventy-two patients were enrolled in the study and 68 patients completed the study. Pulmonary vascular resistance (PVR) was significantly reduced in the sequential combination therapy group[ (12.96 ± 6.48 ) Wood U vs. (16.94 ± 8.11) Wood U, P < 0.05] and in the upfront combination therapy group [(12.45 ± 7.32) Wood U vs. (16.73 ± 9.28) Wood U, P < 0.05] while pulmonary blood flow [(6.77 ± 3.17) L/min vs. (5.08 ± 2.36) L/min, P < 0.05; (6.95 ± 3.32) L/min vs. (5.03 ± 2.32) L/min, P < 0.05], the 6 MWD were significantly increased [(458 ± 59) m vs. (427 ± 65) m, P < 0.05; (494 ± 59) m vs. (436 ± 62) m, P < 0.01], the Borg dyspnea score (2.04 ± 0.72 vs. 2.52 ± 0.79, P < 0.05; 1.72 ± 0.73 vs. 2.51 ± 0.77, P < 0.01) was significantly improved in both groups at 6 months compared to baseline levels. In the upfront combination therapy group, venous oxygen saturation [(68.4 ± 9.3)% vs. (62.9 ± 9.5)%, P < 0.05] and systemic oxygen saturation during exercise[ (87.2 ± 9.7)% vs. (83.1 ± 15.6)%, P < 0.05]at 6 months were also significantly improved compared to baseline. At month 12, significantly lowered pulmonary artery pressure, PVR, Rp/Rs and increased pulmonary blood flow and cardiac index were evidenced in both groups compared to baseline.

CONCLUSIONIloprost combined with low dose tadalafil regimen can effectively reduce PVR, increase 6MWD, and improve cardiopulmonary function in adults CHD patients with severe PAH. Compared with the sequential therapy regimen, the upfront combination therapy regimen can more rapidly improve the clinical symptoms of patients.

Adolescent ; Adult ; Carbolines ; therapeutic use ; Female ; Follow-Up Studies ; Heart Defects, Congenital ; complications ; drug therapy ; Humans ; Hypertension, Pulmonary ; complications ; drug therapy ; Iloprost ; therapeutic use ; Male ; Middle Aged ; Tadalafil ; Treatment Outcome ; Young Adult

Administration, Inhalation ; Adult ; Carbolines ; administration & dosage ; therapeutic use ; Female ; Heart Defects, Congenital ; drug therapy ; Humans ; Hypertension, Pulmonary ; drug therapy ; Iloprost ; administration & dosage ; therapeutic use ; Male ; Tadalafil ; Young Adult

BACKGROUNDWhile echocardiography has been a pivotal screening test in pulmonary arterial hypertension (PAH), the presence of structural cardiac defects may affect the ability to reliably predict pulmonary artery pressures (PAPs). This study sought to evaluate the accuracy of Doppler echocardiography (DE) for estimating PAPs in adult atrial septal defect (ASD) patients with PAH.

METHODSA prospective study was carried out to compare the echocardiographic assessment of PAP with the same pressures obtained by right heart catheterization (RHC) in adult ASD patients with PAH who underwent simultaneous DE and RHC. Bland-Altman analyses were performed to evaluate the agreement between DE and RHC measurements of PAPs.

RESULTSTwo hundred and fifty-seven patients were included in the study. A significant overestimation of the systolic pulmonary arterial pressure (sPAP) and mean pulmonary artery pressure (mPAP) was reported by echocardiography compared with those by catheterization ((81.8 ± 26.9) mmHg vs. (72.9 ± 26.9) mmHg, P < 0.01; (51.9 ± 16.4) mmHg vs. (41.4 ± 17.2) mmHg, P < 0.01, respectively). Twenty-one percent (55/257) of the patients had PAH when estimated by echocardiography whereas showed normal results in the subsequent catheterization test. Using Bland-Altman analytic methods, the bias for the echocardiographic assessment of the sPAP was 9.1 mmHg with 95% limits of agreement ranging from -24.4 to 42.6 mmHg. For mPAP measurement, the bias was 10.5 mmHg with 95% limits of agreement ranging from -12.4 to 33.4 mmHg. On multiple linear regression analysis, age, gender, body surface area, ASDs' diameter, PVR, diastolic blood pressure, and echocardiographic assessment of right atrial pressure (RAP) explained 68.8% of the total variability in the model (r(2) = 0.688, P < 0.01).

CONCLUSIONInaccuracy was frequently reported in Doppler echocardiographic assessment of the PAP in adult ASD patients with PAH and was often associated with age, gender, body surface area, ASDs' diameter, pulmonary vascular resistance, diastolic blood pressure and echocardiographic estimation of RAP.

Adolescent ; Adult ; Aged ; Echocardiography, Doppler ; methods ; Female ; Heart Septal Defects, Atrial ; diagnosis ; Humans ; Hypertension, Pulmonary ; diagnosis ; Male ; Middle Aged ; Prospective Studies ; Pulmonary Artery ; pathology ; Young Adult