| [Objective] Pulmonary arterial hypertension(PAH) is a common complication of congenital heart disease(CHD). The pulmonary arterial pressure(PAP)of some patients of severe PAH associated with CHD(PAH-CHD)remains at a high level or even continues to elevate after surgery. However, the current criteria of clinical examinations are disable to differentiate the reversibility of pulmonary artery remodeling. Our study aims to investigate the count of circulating endothelial cells(CECs)and expression of plasma micro RNAs between patients with reversible pulmonary arterial hypertension(RPH) and irreversible pulmonary arterial hypertension(IRPH) in order to discover several special biomakers to distinguish RPH and IRPH.[Method]In the first part of our trials: a hundred pulmonary hypertension patients associated with congenital heart disease treated at Shanghai Children's Medical Center from September 2013 to June 2014 were enrolled in this study. According to the systolic pulmonary arterial pressure(s PAP) measured by echocardiography in six months after treatment, the patients were separated into 2 groups: IRPH group(s PAP?50 mm Hg,1 mm Hg=0.133 k Pa), RPH group(s PAP<50 mm Hg). Patients who died of pulmonary hypertensive crisis were included in IRPH group.In the second part of our trials: eightteen PAH-CHD patients were enrolled in between September 2013 to June 2014 in our hospital. The patients were divided into 3 groups according to the s PAP(systolic pulmonary arterial pressure) after followed up for six months after surgery: s PAP?50 in IRPH group, s PAP<50 mm Hg in RPH group, s PAP<30mm Hg before surgery in the control group. The plasma specimens of 18 patients(4 cases in the control group, 7 cases in RPH group, 7 cases in IRPH) underwent microRNAs microarray analysis.[Results] Totally the clinical data of 94 patients were enrolled in the statistic analysis, 93 patients finished the follow-up in 6 months after the surgery, one patient died from pulmonary hypertensive crisis.Six patients died of heart failure in the early period after the surgery. The count of CECs was not correlated with the extent of pulmonary hypertension, but was relevant of the outcome of pulmonary hypertension. The count of CECs was higher in IRPH group than that of RPH group(P=0.000). And also the age of patients in IRPH group was elder than that of patients in RPH group(P=0.000).The demographics of eighteen patients collected from preoperative clinical examination can not identify RPH and IRPH and assess the degree of pulmonary vascular obstructive lesions. The micro RNAs microarray showed that five micro RNAs were up-regulated in IRPH group compared with RPH group and control group, that is, micro RNA-21, micro RNA-126, micro RNA-130 a, micro RNA-374 a and micro RNA-1974, on the other hand, six micro RNAs in IRPH group were down-regulated, that is, micro RNA-1, micro RNA-99 a, micro RNA-324-3p, micro RNA-335, micro RNA-93 and micro RNA-423-5p. Expression of micro RNA-21, micro RNA-130 a and micro RNA-126 were significantly higher in IRPH group than those in the RPH group(p <0.01, p=0.007, p=0.006,p=0.009), and also higher than the control group(p <0.05). Moreover, the expression of micro RNA-1 in IRPH group was obviously lower than RPH group(p <0.01, p = 0.010), and also lower than the control group(p <0.05).[Conclusion] The count of CECs is significantly increased in IRPH group. It can be used as a biomarker to distinguish RPH from IRPH in the preoperation of congenital heart disease.Micro RNA-21, micro RNA-126, micro RNA-130 a, micro RNA-1 maybe distinguish RPH from IRPH by micro RNA chip,but still need larger samples to make q RT-PCR validation to further verify the diagnostic value. |
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