A case of portopulmonary hypertension characterized by repeated syncope was retrospectively analyzed. Intrahepatic or extrahepatic factor-induced portal hypertension complicated with metabolic disorder of vasoactive substances, vascular pressure, inflammation, etc. may result in systolic and diastolic dysfunction of pulmonary arteries and systemic hyperdynamic circulation, the long-term effect of which can induce vascular remodeling and consequently, pulmonary hypertension. The pathogenic process is rather insidious. Pulmonary hypertension is clinically characterized by the raised average pulmonary artery pressure, normal pulmonary capillary wedge pressure and high pulmonary vascular resistance. Currently available therapeutic approaches include drug therapy targeting on pulmonary hypertension and liver transplantation.
Blood Pressure ; Humans ; Hypertension, Portal ; complications ; diagnosis ; Hypertension, Pulmonary ; complications ; diagnosis ; Liver Transplantation ; Syncope ; complications ; diagnosis

Pulmonary hypertension is a frequent complication of chronic obstructive pulmonary disease (COPD) and associated with a worse survival and increased risk of hospitalization for exacerbation of COPD. However, little information exists regarding the potential role of systemic inflammation in pulmonary hypertension of COPD. The purpose of the present study was to investigate the degree of C-reactive protein (CRP) and endothelin-1 (ET-1) levels in COPD patient with and without pulmonary hypertension. The levels of CRP and ET-1 were investigated in 58 COPD patient with pulmonary hypertension and 50 patients without pulmonary hypertension. Pulmonary hypertension was defined as a systolic pulmonary artery pressure (Ppa) > or =35 mmHg assessed by Doppler echocardiography. Plasma CRP and ET-1 levels were significantly higher in patients with pulmonary hypertension than in patients without hypertension. There were significant positive correlations between the plasma ET-1 level and CRP level in the whole study groups. For COPD patients, systolic Ppa correlated significantly with plasma CRP levels and plasma ET-1 levels. These findings support a possibility that CRP and ET-1 correlate to pulmonary hypertension in COPD patients.
Aged ; Blood Pressure ; C-Reactive Protein/*analysis ; Echocardiography, Doppler ; Endothelin-1/*blood ; Female ; Humans ; Hypertension, Pulmonary/*blood/complications ; Male ; Middle Aged ; Pulmonary Disease, Chronic Obstructive/*blood/complications

OBJECTIVETo examine the correlation of the changes in the serum markers (C-reactive protein, endothelin-1, interleukin-6, and brain natriuretic peptide) with chronic obstructive pulmonary disease (COPD) and pulmonary hypertension secondary to COPD.

METHODSA total of 174 COPD patients with acute exacerbation, admitted between February 2011 and February, 2013, were enrolled in this study, with 43 volunteers with normal pulmonary functions as controls. Pulmonary arterial pressure was determined by Doppler echocardiograph, and the severities (mild, moderate and severe) of PH secondary to COPD was evaluated. The levels of serum markers were determined using ELISA kits.

RESULTSThe levels of serum markers in patients with COPD was significantly elevated compared with those of the control subjects (P<0.05), and further increased in patients with pulmonary hypertension secondary to COPD (P<0.05). A positive correlation was found between these serum markers and pulmonary artery pressure in COPD patients with mild and moderate pulmonary hypertension. In patients with severe pulmonary hypertension, only the serum level of brain natriuretic peptide continued to increase with pulmonary artery pressure (P<0.05), and the other markers did not further increase.

CONCLUSIONSEarly and combined examination of these serum markers in patients with COPD can help to identify pulmonary hypertension in early stage and estimate the severity of pulmonary hypertension. Hemodynamic monitoring of the changes of these serum markers can be of important clinical value in the treatment of pulmonary hypertension secondary to COPD and in evaluation of the prognosis of COPD.

Aged ; Biomarkers ; blood ; Blood Pressure ; C-Reactive Protein ; metabolism ; Endothelin-1 ; blood ; Female ; Humans ; Hypertension, Pulmonary ; blood ; complications ; physiopathology ; Interleukin-6 ; blood ; Male ; Natriuretic Peptide, Brain ; blood ; Pulmonary Disease, Chronic Obstructive ; blood ; complications ; physiopathology

OBJECTIVETo assess the perioperative changes in plasma endothelin (ET) and calcitonin gene-related peptide (CGRP) in children with congenital heart disease complicated by pulmonary hypertension.

METHODSIn 33 patients, radioimmunoassay was used to measure plasma ET and CGRP levels at different time points: Preoperative, immediate post-op, 3 hrs, 24 hrs, 72 hrs after cardiac surgery with cardiopulmonary bypass.

RESULTSPreoperative plasma ET levels in two groups were markedly increased. The level of ET in group II (severe pulmonary hypertension patients) was significantly higher than those [(138+/-25 compared with 77+/-19)pg/ml, P<0.01] in group I (mild pulmonary hypertension patients). In group I, the plasma ET level rose immediately post-op (P<0.001) and retumed to the initial level after one day (P>0.05). However, in group II plasma ET level was only normalized 3 days post-op (P<0.05). The CGRP level of both group I and group II increased at the first post-op day [(187+/-131 330+/-187)pg/ml], then began to decline by the 3rd day [(88+/-85, 150+/-95) pg/ml].

CONCLUSIONEndothelin and calcitonin gene-related peptide was involved in the pathophysiologic process of pulmonary hypertension and correlated with the degree of pulmonary hypertension. The imbalance of ET and CGRP after cardiopulmonary bypass may affect the prognosis.

Adolescent ; Calcitonin Gene-Related Peptide ; blood ; Child ; Child, Preschool ; Endothelins ; blood ; Female ; Heart Defects, Congenital ; blood ; complications ; surgery ; Humans ; Hypertension, Pulmonary ; blood ; etiology ; Infant ; Male ; Radioimmunoassay

Pulmonary embolism (PE) poses a challenge to physicians, as it can be difficult to diagnose but results in significant mortality and morbidity in patients. Diagnosing PE requires an integrated approach using clinical findings, electrocardiography (ECG), blood investigations and imaging modalities. Abnormalities in ECG are common among patients with massive acute PE and can serve as a prognostic indicator. In this article, we describe the ECG presentations of two patients diagnosed with PE, and review the literature on the various types of ECG presentations and their role in predicting the prognosis of PE.
Anticoagulants ; administration & dosage ; Blood Pressure ; Echocardiography ; Electrocardiography ; methods ; Female ; Hemorrhage ; complications ; Humans ; Hypertension ; complications ; Male ; Middle Aged ; Prognosis ; Pulmonary Embolism ; diagnosis ; Stroke ; complications

OBJECTIVETo study the changes of pulmonary vascular remodeling in the pathogenesis of hypoxia-induced pulmonary hypertension (HPH) in neonatal rats.

METHODSNinety-six newborn Wistar rats were randomly divided into an HPH group (hypoxia exposure) and a control group (room air exposure). The mean pulmonary arteria pressure (mPAP), right ventricle hypertrophy index (RVHI), and vascular remodeling indexes MT% and MA% were measured 3, 5, 7, 10, 14 and 21 days after exposure (n=8 each time point). The ultrastructure of pulmonary vascular was observed under a transmission electron microscope.

RESULTSmPAP in the HPH group 3, 5, 7, 10, 14 and 21 days after hypoxia exposure increased compared with the control group (P<0.05). With the prolonged hypoxia time, mPAP in the HPH group increased more significantly. MT%, MA% and RVHI increased significantly in the HPH group after 7 days of hypoxia exposure in a time-dependent manner compared with the control group (P<0.05). The transmission electron microscopy demonstrated that small pulmonary arterials became thickened, endothelial cell hyperplasia and degeneration, and organelles increased in the HPH group after 7 days of hypoxia exposure. Besides, collagen deposition in the extracellular matrix and the changes of pulmonary vascular remodeling were observed.

CONCLUSIONSmPAP increases between 3 and 5 days of hypoxia exposure, resulting from pulmonary vascular spasm caused by hypoxia. After hypoxia of 7 days, the mPAP increases more significantly, pulmonary vascular remodeling occurs, and right ventricle becomes irreversibly hypertrophic. These changes may be intensified as the prolonged hypoxia time.

Animals ; Animals, Newborn ; Blood Pressure ; Endothelins ; physiology ; Hypertension, Pulmonary ; etiology ; Hypertrophy, Right Ventricular ; etiology ; Hypoxia ; complications ; Pulmonary Artery ; pathology ; ultrastructure ; Rats ; Rats, Wistar

OBJECTIVETo study the changes and significance of serum hypoxia-inducible factor-1α (HIF-1α), endothelin-1 (ET-1) and calcium (Ca(2+)) levels in neonates with hypoxic pulmonary hypertension (HPH).

METHODSSeventy-five neonates with HPH (29 mild, 25 moderate and 21 severe) and 22 hospitalized neonates with non-HPH (control group) were enrolled. Pulmonary artery systolic blood pressure (PASP) was measured by bedside echocardiography within 24 hrs after birth. Serum levels of HIF-1α and ET-1 were measured using ELASA. Serum Ca2+ concentrations were measured with ion selective electrode.

RESULTSSerum levels of HIF-1α and ET-1 in the HPH group increased significantly compared with those in the control group (P<0.01), and were positively related with PASP (Rhif-1α=0.75, P<0.01; Ret-1=0.56,P<0.05). Serum Ca2+ levels in neonates with severe HPH were significantly lower than those in the control group (P<0.05). There were no correlation between serum Ca2+ levels and PASP.

CONCLUSIONSSerum HIF-1α and ET-1 levels are positively related with PASP in neonates with HPH, suggesting that serum HIF-1α and ET-1 may be involved in the occurrence of neonatal HPH. Serum Ca2+ levels are reduced in severe neonates with HPH, suggesting that serum Ca2+ may play a role in the occurrence of severe HPH.

Blood Pressure ; Calcium ; blood ; physiology ; Endothelin-1 ; blood ; Female ; Humans ; Hypertension, Pulmonary ; blood ; etiology ; Hypoxia ; complications ; Hypoxia-Inducible Factor 1, alpha Subunit ; blood ; Infant, Newborn ; Male ; Pulmonary Artery ; physiopathology

OBJECTIVETo study the plasma natriuretic peptide (BNP) level in children with pulmonary artery hypertension (PAH) secondary to congenital heart disease (CHD) and its correlation with left ventricular diastolic function.

METHODSDoppler echocardiography was performed on 95 CHD children with PAH (PAH group) and on 42 CHD without PAH (control group). The plasma BNP level was measured using radioimmunity assay.

RESULTSDoppler echocardiography showed that the left ventricular end-diastolic diameter (LVDd), the right ventricular diameter end-diastolic (RVDd), and the pulmonary artery diameter (PAd) increased significantly in the PAH group compared with those in the control group (p<0.05). The PAH group had higher velocity of tricuspid regurgitation (VTR) and higher pulmonary artery systolic pressure (PASP) than the control group (p<0.05). The PAH group also had higher mitral A peak velocity (AV), higher mitral A peak velocity integral (AVI), higher E peak velocity intgral (EVI), and higher ratio of mitral AV to mitral E peak velocity (EV) and AVI/EVI ratio as well as prolonged left ventricular volumetric relaxation time than the control group. PASP was positively correlated with the AV/EV ratio (p<0.05). The plasma BNP level in the PAH group increased significantly compared with that in the control group. In the PAH group, the plasma BNP level was positively correlated with the pulmonary artery pressure and the ratio of AV/EV.

CONCLUSIONSThe left ventricular diastolic function is positively correlated with the plasma BNP level in children with PAH secondary to CHD. BNP may play an important role in the generation and development of left ventricular diastolic dysfunction induced by PAH.

Child ; Child, Preschool ; Diastole ; Female ; Heart Defects, Congenital ; complications ; Humans ; Hypertension, Pulmonary ; blood ; physiopathology ; Infant ; Infant, Newborn ; Male ; Natriuretic Peptide, Brain ; blood ; Ventricular Function, Left

OBJECTIVETo study the relationship between the age at operation and prognosis in children with severe pulmonary hypertension (PH) due to ventricular septal defect (VSD).

METHODSForty children with severe PH (increased total pulmonary circulation resistance)due to VSD were divided into two groups based on age at operation(Group I aged less than 2 years and group II more than 2 years). The hemodynamic parameter follow-up was measured by cardiac catheterization at presurgery, one week after surgery and 5-7 years postoperatively.

RESULTSThe ratio of pulmonary arterial pressure and systemic arterial pressure (pp/ps),pulmonary resistance and systemic resistance (R(p)/R(s)), and pulmonary vascular resistance (PVR) and small pulmonary arterial resistance (PAR) were significantly different in two groups (P<0.01). During follow-up in the group less than 2 years, all the hemodynamic parameters were at normal level, while in the group more than 2 years, only p(p)/p(s) and R(p)/R(s) were close to normal level. The pulmonary arterial resistance was still abnormal.

CONCLUSIONAn early operation may be the only way to gain optimal long term result of surgery and decrease the incidence of pulmonary vascular disease in children with PH due to VSD.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Heart Septal Defects, Ventricular ; complications ; Hemodynamics ; Humans ; Hypertension, Pulmonary ; physiopathology ; surgery ; Infant ; Male ; Oxygen ; blood ; Vascular Resistance

OBJECTIVETo determine the relationship between the serum sulfur dioxide, homocysteine and the pulmonary arterial pressure in children with congenital heart defects who generated a pulmonary arterial hypertension syndrome (PAH-CHD), and analyze their role in the pathological process of the disease.

METHODThis was a prospective cohort study, children with systemic pulmonary shunt CHD were selected. The patients were divided into three groups: the CHD with no PAH group:n = 20, 10 males, 10 females, 5 with ventricular septal defect (VSD), 8 with atrial septal defect (ASD) and 7 with patent ductus arteriosus (PDA), mean age (1.9 ± 1.8) years; the CHD with mild PAH group:n = 20, 10 males, 10 females, 12 with VSD, 6 with ASD, and 2 with PDA, mean age (1.0 ± 0.8) year; the CHD with moderate or severe PAH group:n = 20, 8 males, 12 females, 12 with VSD, 6 with ASD, and 1 with PDA, 1 with ASD+VSD, mean age (1.8 ± 1.6) year. Twenty healthy children were enrolled from outpatient department as the control group [included 8 males, 12 females, mean age (1.9 ± 1.5) years]. The homocysteine and SO2 concentrations in the serum samples were detected by a modified high performance liquid chromatographic method with fluorescence detection (HPLC-FD), then, multiple comparisons among the groups were performed with analysis of variance, and the pearson correlation.

RESULTThe serum homocysteine concentrations were respectively (11.0 ± 2.7) , (11.7 ± 2.5), (12.0 ± 2.1), (14.3 ± 3.2) µmol/L in the control group, CHD with no PAH group, CHD with mild PAH group, and CHD with moderate or severe PAH group. According to the multiple comparisons, the CHD with moderate or severe PAH group had the highest level (P all < 0.05) .While the comparison within the control group, CHD with none PAH group, and CHD with mild PAH group, the differences were not significant (P all > 0.05). The serum sulfur dioxide strength (concentrated as SO3(2-)) were respectively (10.6 ± 2.4), (8.9 ± 2.3), (7.3 ± 2.9), (4.3 ± 2.1) µmol/L in the control group, CHD with none PAH group, CHD with mild PAH group, and CHD with moderate or severe PAH group. CHD with moderate or severe PAH group had the highest level of serum sulfur dioxide (P < 0.05) . The pearson correlation analysis indicated that in the CHD children, the serum homocysteine were positively correlated with the pulmonary arterial pressure (r = 0.481, P < 0.01), while, the sulfur dioxide were negatively correlated with pulmonary arterial pressure (r = -0.553, P < 0.01).In all children, the serum homocysteine levels were negatively correlated with the sulfur dioxide (r = -0.231, P = 0.039).

CONCLUSIONThe PAH-CHD children had higher homocysteine levels and lower sulfur dioxide levelsl, which demonstrated the disturbance of homocysteine-sulfur dioxide pathway in the sulfur containing amino acids metabolish in the disease. The homocysteine may become a biological marker which reflecting the severities of the PAH-CHD, while the sulfur dioxide can be a new target for the therapy of PAH-CHD.

Biomarkers ; blood ; Case-Control Studies ; Child, Preschool ; Ductus Arteriosus, Patent ; blood ; complications ; physiopathology ; Familial Primary Pulmonary Hypertension ; blood ; etiology ; physiopathology ; Female ; Heart Defects, Congenital ; blood ; complications ; physiopathology ; Heart Septal Defects ; blood ; complications ; physiopathology ; Hemodynamics ; Homocysteine ; blood ; Humans ; Infant ; Male ; Sulfur Dioxide ; blood