OBJECTIVETo evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus (SLE) accompanied by pulmonary hypertension (PH).

METHODSThe clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed.

RESULTSAmong the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0.1 year (range: 0-6.5 years). Aside from PH-related symptoms, Raynaud's phenomenon was observed in 6 (40%) of the 15 patients. There was no significant difference in SLE disease activity (evaluated by complements 3 and 4 levels, erythrocyte sedimentation rate, and positive rate of anti-double-stranded DNA) between patients with mild-to-moderate PH and those with severe PH (P<0.05). As for treatment, 13 patients received immunosuppressive therapy with glucocorticoids, and among them 2 patients received PH-targeted therapy. During a median follow-up of 8.0 years (range: 0.5-18.1 years) since the diagnosis of PH, 2 deaths were noted with class III or IV cardiac function (World Health Organization), while the other patients were in a stable condition.

CONCLUSIONSRaynaud's phenomenon is a common clinical manifestation in children with SLE accompanied by pulmonary hypertension (PH). PH severity is not significantly associated with SLE disease activity, and thus greater focus should be placed upon early screening of pulmonary arterial pressure in SLE patients. Early diagnosis and early treatment can improve the prognosis of children with SLE.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Hypertension, Pulmonary ; complications ; drug therapy ; Infant ; Lupus Erythematosus, Systemic ; complications ; drug therapy ; Male

OBJECTIVETo study the effects of puerarin on pulmonary Vascular remodeling in rats with pulmonary hypertension induced by chronic hypoxia and hypercapnia.

METHODForty male rats (180-220) g of grade two were randomly divided into five groups: normal control group (NC), hypoxia-hypercapnia 1, 2, 3 week groups (LH1, LH2, LH3) and hypoxia-hypercapnia 3-week + puerarin group (LHP3 group, puerarin intraperitoneal injection, 20 mg x kg(-1) x d(-1)). Collagen I, III and their mRNA were observed in pulmonary arterioles by the technique of immunohistochemistry and in situ hybridization.

RESULTLight microscopy showed media thickness of pulmonary arterioles was much higher in LH3 group than that of NC group, and, vessel cavity turned more straiter in LH3 group than that of NC group. Howerer, the damage of pulmonary arterioles in LHP3 group was much slighter than that of LH3 group. The levels of plasma ET-1 and lung homogenates Hyr and MDA were much higher in rats of LH3 group than those of NC group (P < 0.01), and lower in LHP3 group than LH3 groups (P < 0.01). The activities of SOD in lung homogenates were significantly lowered in hypoxic and hypercapnic groups compared with control group (P < 0.01), but higher in LHP3 group than that of LH3 group. Plasma NO content of group LH was lower than that of group NC (P < 0.01), it was highter in group LHP3 than that of group LH3 (P < 0.01). Expression of collagen I and collagen I mRNA in pulmonary arterioles were significantly higher in rats of LH groups than those of NC group (P < 0.01), and they were lower in rats of LHP3 group than those of LH3 group (P < 0. 01). Expression of collagen III and collagen III mRNA were not significant difference among three groups.

CONCLUSIONPuerarin could improve pulmonary vascular remodeling in rats with pulmonary hypertension by inhibiting the deposition of collagen.

Animals ; Hypercapnia ; complications ; Hypertension, Pulmonary ; drug therapy ; Hypoxia ; complications ; Isoflavones ; pharmacology ; Male ; Oxidative Stress ; drug effects ; Pulmonary Artery ; drug effects ; Random Allocation ; Rats ; Rats, Sprague-Dawley

PURPOSE: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. MATERIALS AND METHODS: Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9+/-14.5 years, 80% of female) were enrolled. RESULTS: Causes were congenital heart disease in 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6+/-45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, median survival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021). CONCLUSION: WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore, early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.
Adult ; Antihypertensive Agents/*therapeutic use ; *Disease Management ; Familial Primary Pulmonary Hypertension ; Female ; Heart Defects, Congenital/complications ; Humans ; Hypertension/complications ; Hypertension, Pulmonary/*classification/*drug therapy/mortality ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Molecular Targeted Therapy/*methods ; Prognosis ; Retrospective Studies ; Survival Rate

OBJECTIVETo observe the acute hemodynamic effects of intravenous diltiazem in patients with congenital heart defect (CHD) and severe pulmonary hypertension (HP) post cardiac surgery.

METHODSFrom November 2003 to September 2005, 12 patients with CHD and severe HP (4 male, mean age 17.8 +/- 9.8 years) after cardiac surgery received intravenous diltiazem (3 - 5 microg x kg(-1) x min(-1)) in the Intensive Care Unit. Mean pulmonary artery pressure (mPAP), mean arterial pressure (MAP), heart rate (HR), stroke volume (SV), systemic vascular resistance (SVR), pulmonary vascular resistance (PVR) were monitored with Swan-Ganz catheter before (T1) and 6 hours (T2) after diltiazem injection, before weaning patients off of ventilator (T3), 1 hour (T4) and 24 hour (T5) after extubation.

RESULTSAll patients survived during the observation period and no patient developed pulmonary hypertension crisis. The average ventilation time was (88.7 +/- 50.1) hours. Mean ICU stay time was (5.8 +/- 3.1) days. Compared to T1, mPAP was significantly decreased at T3 and T5, MAP significantly increased at T4 and T5, HR significantly reduced at T2 and thereafter, SV significantly increased at T3, T4 and T5 and PVR significantly increased at T3 and T5 while SVR remained unchanged after diltiazem therapy.

CONCLUSIONIntravenous use of diltiazem is safe and effective for patients with CHD with severe HP post cardiac surgery.

Adolescent ; Adult ; Child ; Child, Preschool ; Diltiazem ; therapeutic use ; Female ; Heart Defects, Congenital ; complications ; drug therapy ; Humans ; Hypertension, Pulmonary ; complications ; drug therapy ; Injections, Intravenous ; Male ; Young Adult

OBJECTIVETo investigate the effect of oral bosentan in the treatment of congenital heart disease-associated pulmonary arterial hypertension.

METHODS24 patients with congenital heart disease-associated pulmonary arterial hypertension, including 4 receiving heart surgery and 20 with surgical contraindications, were enrolled in this study. All the patients were given oral bosentan and followed up regularly for analyzing the outcomes and side effects.

RESULTSOne patient was lost to follow up and one patient died. Systolic pulmonary artery pressure showed no significant changes at 2 (93.6 ± 17.2 mmHg) and 4 months (85.7 ± 25.5 mmHg) of bosentan treatment compared to that before the medication (97.8 ± 14.9 mmHg) (P=0.096), but decreased significantly after a 6-month therapy (80.9 ± 25.0 mmHg, P=0.029). The 6-minute walking distance increased significantly after a 2, 4, and 6-month therapy [(488 ± 98.8, 496.3 ± 89.0, and 491.3 ± 114.2 m, respectively; P=0.004, 0.003, and 0.004 vs the distance before medication (317.0 ± 134.1)]. The New York heart functional classification was improved significantly after a 2, 4, and 6-month therapy [(2.0 ± 0.5, 1.8 ± 0.4, and 1.7 ± 0.5, respectively; P<0.001 vs pre-medication score (2.9 ± 0.5)). Hepatic and renal function remained normal, and ALT and AST showed no significant variations during the medication (P>0.05).

CONCLUSIONOral bosentan can effectively relieve the symptoms, decrease pulmonary artery hypertension, and improve exercise tolerance and cardiac function classification in patients with pulmonary artery hypertension associated with congenital heart disease with good safety and mild side effects.

Administration, Oral ; Antihypertensive Agents ; therapeutic use ; Heart Defects, Congenital ; complications ; Humans ; Hypertension, Pulmonary ; drug therapy ; etiology ; Sulfonamides ; therapeutic use

OBJECTIVETo observe simvastatin treatment of pulmonary hypertension in patients with coal workers pneumoconiosis (CWP).

METHODS96 CWP patients with pulmonary hypertension were randomly divided into treatment and control groups. The control group was treated with 2.5 mg warfarin, once a day for four months; the treatment group was treated with 20 mg simvastatin, taken in evening, for 4 months. 6 min walking distance (6MWD) test and inspection pulmonary artery pressure were measured by echocardiography before and after treatment.

RESULTSIn the treatment group, the 6MWD were (258 ± 26) m after treatment and (225 ± 19) m before treatment, respectively. Compared with control group, pulmonary artery pressure was (41 ± 9) mm Hg in the treatment group before treatment, (36 ± 3) mm Hg in the treatment group after treatment, and (39 ± 5) mm Hg in control group, respectively, the difference was statistically significant (P < 0.05).

CONCLUSIONSSimvastatin can improve pulmonary hypertension in coal workers pneumoconiosis, and shows a definite curative effect.

Aged ; Aged, 80 and over ; Anthracosis ; complications ; drug therapy ; Coal Mining ; Humans ; Hypertension, Pulmonary ; drug therapy ; etiology ; Male ; Middle Aged ; Simvastatin ; therapeutic use

Pulmonary arterial hypertension (PAH) secondary to chronic obstructive pulmonary disease (COPD) is incurable and it has an unpredictable survival rate. Two men who suffered from COPD presented with progressive dyspnea and edema, respectively. PAH, as estimated by the peak velocity of tricuspidal regurgitation, and the depressed myocardial performance index (MPI) of the right ventricle (RV) were noted on echocardiography. In addition to the baseline therapy for their depressed ventilatory function, we prescribed tadalafil 10 mg orally every other day for 2 weeks and then we doubled the dosage. They well tolerated the medication without any notable side effects. After 4 weeks of tadalafil treatment, the patients' pulmonary arterial pressure was decreased and the MPI of the RV was improved in both. The exercise capacity, as measured by the respiratory oxygen uptake, also improved from 10.9 mL/kg/min to 13.8 mL/kg/min in one patient. We report here on 2 patients with PAH secondary to COPD, and they showed notable improvement of their pulmonary hemodynamics and exercise capacity with the administration of tadalafil.
Pulmonary Disease, Chronic Obstructive/*complications ; Pulmonary Artery/drug effects/*pathology ; Phosphodiesterase Inhibitors/*therapeutic use ; Oxygen Consumption/drug effects ; Middle Aged ; Male ; Hypertension, Pulmonary/*drug therapy/etiology ; Humans ; Exercise Tolerance/drug effects ; Carbolines/*therapeutic use

OBJECTIVETo observe the clinical efficacy and safety of sildenafil in the treatment of high altitude heart disease associated with severe pulmonary arterial hypertension (PAH) in children.

METHODSFifty children (aged 2 months to 2 years) with high altitude heart disease associated with severe PAH, who were continuously transferred to the Intensive Care Unit between January 2011 and October 2013, were randomly assigned to observation and control groups. The control group was given conventional treatment, while the observation group received oral sildenafil [1 mg/(kg . d)] three times daily for 7-10 days in addition to the conventional treatment. Before and after treatment, hemodynamics, blood gas, routine blood parameters, and blood biochemical parameters were recorded.

RESULTSAfter treatment, the observation group had a significantly higher decrease in mean pulmonary artery pressure and significantly higher increases in arterial partial pressure of oxygen, cardiac output, cardiac index, and oxygenation index compared with the control group (P<0.05). In the observation group, there were no significant changes in mean arterial pressure, routine blood parameters and blood biochemical parameters (P>0.05), and no obvious adverse reactions were found.

CONCLUSIONSFor children with high altitude heart disease associated with severe PAH, sildenafil can effectively reduce pulmonary artery pressure and improve cardiac function and does not cause adverse reactions. This therapy has good safety according to the preliminary evaluation.

Altitude ; Familial Primary Pulmonary Hypertension ; Female ; Heart Diseases ; drug therapy ; Humans ; Hypertension, Pulmonary ; complications ; physiopathology ; Infant ; Male ; Piperazines ; adverse effects ; therapeutic use ; Purines ; adverse effects ; therapeutic use ; Sildenafil Citrate ; Sulfones ; adverse effects ; therapeutic use ; Vasodilator Agents ; adverse effects ; therapeutic use

OBJECTIVESTo assess the hemodynamic, oxygen-dynamic and ventilative effects of Zafirlukast in chronic obstructive pulmonary disease (COPD) induced chronic cor pulmonale at acute exacerbation stage and the mechanisms of Zafirlukast efficacy.

METHODSEleven cases of chronic cor pulmonale at acute exacerbation were examined using Swan-Ganz catheter and peripheral intra-artery catheter. The hemodynamic, oxygen-dynamic parameters and respiratory rate, plasma endothelium-1 (ET-1) level, and urea leukotriene E(4) (LTE(4)) level were measured before and at the 1st, 3rd, 5th, 7th, 9th, 12th hour after taking 40 mg Zafirlukast orally. Arterial and mixed venous blood gas analyses were done correspondingly.

RESULTSThe average pulmonary arterial pressure (mPAP) and pulmonary vascular resistance index (PVRI) were lowered at the 3rd hour after taking Zafirlukast by 23% and 36.5%, respectively. They returned to the baseline around 12th hour. Respiratory rate decreased significantly within the 3rd-7th hour after taking Zafirlukast. LTE(4) and ET-1 levels lowered at the 3rd hour and showed a positive correlation with change of mPAP.

CONCLUSIONSZafirlukast can reduce mPAP, pulmonary vascular resistance (PVR) and does not affect the ambulatory blood pressure monitoring (ABPM) and oxygenation in cases of chronic cor pulmonale at acute exacerbation stage. Zafirlukast may play a role as an alternative to decrease PAP in COPD patients.

Aged ; Female ; Hemodynamics ; drug effects ; Humans ; Hypertension, Pulmonary ; drug therapy ; Leukotriene Antagonists ; therapeutic use ; Leukotriene E4 ; urine ; Male ; Oxygen ; metabolism ; Pulmonary Disease, Chronic Obstructive ; complications ; Respiration ; drug effects ; Tosyl Compounds ; therapeutic use

OBJECTIVETo summarize the experience of surgical treatment of secundum atrial septal defects in adults over 30 years old.

METHODSThere were 469 patients with secundum atrial septal defects in our study (male 144, female 325; ages 30-68, mean 38.6 years old). There were 105 cases with pulmonary hypertension and 458 cases with arrhythmia in the group. Surgical closure of defects were performed in all patients. Surgical closure of 358 cases were done by patches including 305 autologous pericardial patches. The low dose (6 x 10(-6)) nitric oxide inhalation was used in 25 postoperative patients with pulmonary hypertension. Right sided maze procedures were done in 5 cases with atrial fibrillation.

RESULTSSurgical mortality was 0.6% (3 cases), the others were healed. In the group, there were 180 cases with arrhythmia, 27 cases with left ventricular function amyoplasia, 28 cases with low cardiac output syndrome, 12 cases in secondary operation for bleeding and 1 case with air-embolism. The level of mean pulmonary artery pressure of 25 postoperative patients with pulmonary hypertension inhaled nitric oxide was down 28.5%. After right sided maze procedures were done in 5 cases with atrial fibrillation, atrial fibrillation disappeared. 352 cases were followed up from 3 months to 20 years (mean 5.6 years). Twenty-nine cases were in class I-II of cardiac function, and the others were better than class I of cardiac function.

CONCLUSIONSAtrial septal defects in adult should be operated as early as possible. When patch is needed, an autologous pericardial patch is the first selection. Inhaled nitric oxide is an effective method to postoperative pulmonary hypertension. The maze operation should be performed for atrial septal defect with atrial fibrillation while the surgical closure of defect was done. During and after operation, much attention should be paid to preventing and curing arrhythmia and protecting and supporting left heart function.

Administration, Inhalation ; Adult ; Age Factors ; Aged ; Atrial Fibrillation ; surgery ; Endothelium-Dependent Relaxing Factors ; therapeutic use ; Female ; Heart Septal Defects, Atrial ; surgery ; Humans ; Hypertension, Pulmonary ; complications ; drug therapy ; Male ; Middle Aged ; Nitric Oxide ; therapeutic use ; Postoperative Complications ; drug therapy ; Retrospective Studies