肺动脉高压( pulmonary artery hypertension, PAH) 是右心衰竭的首要原因, 而右心衰竭又是 PAH 患者的重要致命因素, 及时而准确地对肺动脉高压患者的右心室功能做出评价, 对其病情评估、治疗决策和预后判断有重要的意义。近年来新发展的超声心动图二维应变及应变率技术、实时三维成像技术、Tei 指数及三尖瓣环收缩期位移等新技术弥补了传统超声评价右心功能的不足。本文就超声新技术在评价PAH 患者右心功能的应用做一综述。
【摘要】 目的 探讨肺动脉高压患者药物靶向治疗的效果与耐受性。 方法 回顾分析2008年1月〖CD3/5〗2009年8月期间8例肺动脉高压患者分别接受波生坦及西地那非治疗的临床资料,评估其临床表现、WHO肺动脉高压功能分级、6 min步行距离及肺动脉收缩压在基线及治疗3个月后的变化。 结果 治疗后3个月,患者均能耐受药物治疗,无严重不良反应发生。WHO肺动脉高压功能分级在治疗前平均(31±04),治疗后为(23±09),明显得到改善(Plt;005)。肺动脉收缩压在治疗前平均(695±112 ) mm Hg(1 mm Hg=0133 kPa),治疗后为(483±124) mm Hg,明显降低(Plt;005)。6 min步行距离在治疗前平均(324±48) m,治疗后为(400±43) m,明显延长(Plt;005)。 结论 肺动脉高压患者药物靶向治疗的疗效显著,且耐受良好。【Abstract】 Objective To examine the effects of target medical therapy in patients with pulmonary arterial hypertension(PAH). Methods To determine the safety and efficacy of bosentan and sildenafil in eight patients with PAH.The patients’ clinical features, six minutes walking diastance, WHO functional class and systolic pulmonary arterial pressure (SPAP) were measured at baseline and at three months after initiating target medial treatment. Results At the three months followup assessments, WHO functional class was improved with 31±04 vs 23±09 (Plt;005); SPAP was significantly decreased with(695±112 ) mm Hg vs (483±124) mm Hg (Plt;005), the six minutes walking distance was significantly increased with(324±48) m vs(400±43) m (Plt;005). Target medical treatment was well tolerated. Conclusion Target medical treatment is well tolerated and has beneficial effects on PAH.
ObjectiveTo summarize the clinical characteristics and the long-term results of pulmonary thromboendarterectomy (PTE) in the chronic thromboembolic pulmonary hypertension (CTEPH) patients with unilateral main pulmonary artery occlusion.MethodsWe retrospectively analyzed the clinical data of 15 CTEPH patients with unilateral main pulmonary artery occlusion in Fuwai Hospital between 2004 and 2018. There were 11 males and 4 females aged 34.1±12.0 years at operation.ResultsThe mean circulatory arrest was 31.1±12.1 minutes. The ICU stay was 5 (2-29) d. The hospital stay was 15 (8-29) d. There was no hospital death. There was a decline in systolic pulmonary artery pressures (sPAP, 69.9±27.9 mm Hg to 35.1±9.7 mm Hg, P=0.020) after surgery. On postoperative V/Q scan, only 6 patients (40.0%) had significant improvement in reperfusion (≥75% estimated) of the occluded lung. There was no death during the median observation period of 49 months follow-up, while 2 patients had recurrence of pulmonary embolism.ConclusionCTEPH patients with unilateral main pulmonary artery occlusion represent a challenging cohort. PTE is a curative resolution in both early- and long- term results, although there is a high requirement of perioperative management and a high risk of postoperative complications and rethrombosis.
Objective To investigate the effect of chronic altitude hypoxia exposure on serum lipoprotein levels in healthy subjects and patients with pulmonary hypertension, and whether there is a difference in serum lipoprotein levels between patients with pulmonary hypertension at middle and high altitude. Methods The case data of 245 Han patients with COPD complicated with pulmonary hypertension admitted to the Affiliated Hospital of Qinghai University from January 2018 to September 2022 were retrospectively analyzed. According to the altitude of their long-term residence before onset, the patients were divided into two groups, 119 cases in the middle altitude group (1500 m~2500 m). 126 cases were in the high altitude group of 2500 m~4500 m. In addition, the physical examination data of 50 healthy people in the intermediate and high altitude groups were collected as the control group (the age and gender of the healthy people in the same altitude group were similar to those in the COPD-PH group), a total of 4 groups were collected. The general data, pulmonary artery systolic blood pressure (PASP), total cholesterol (TC), triglyceride (TG), high density lipoprotein cholesterol (HDL-C) and low density lipoprotein cholesterol (LDL-C) of the four groups were compared, and the correlation between pulmonary artery systolic blood pressure (PASP) and related variables was analyzed. ResultsThere were no significant differences in age, gender, smoking and drinking between the healthy control group and COPD-PH group (all P>0.05). There were significant differences in body mass index, PASP, TC, TG, HDL-C, LDL-C, TG/HDL-C, HDL-C/LDL-C between the healthy control group and the COPD-PH group (all P<0.05). In the healthy control group, only BMI was significantly different between the high altitude group and the middle altitude group (P<0.05). In the COPD-PH group, PASP, BMI, TC, HDL-C and TG/HDL-C in the high altitude group were significantly different from those in the moderate altitude group (all P<0.05). There were no significant differences in age, gender, smoking, drinking, TG, LDL-C and HDL-C/LDL-C between the two groups (all P>0.05), when gender, age, altitude, body mass index, PASP, smoking and drinking were included in the multi-factor linear regression equation of lipoprotein (TC, TG, HDL-C and LDL-C), it was found that different elevations (middle and higher elevations) only had statistically different effects on HDL-C (b=-0.046, t=-2.209, P=0.028). Correlation analysis showed that PASP was not correlated with age, altitude, body mass index and blood lipids (TC, TG, HDL-C, LDL-C) in the healthy control group (all P>0.05). However, in the COPD-PH group, PASP was negatively correlated with blood lipid indicators (TC, HDL-C and LDL-C). PASP was positively correlated with altitude (a risk factor for hypoxia). ConclusionsHypoxia environment factors characterized by altitude are closely related to the severity of pulmonary artery pressure in patients with COPD-PH, and higher pulmonary artery systolic pressure is closely related to lower levels of TC, HDL-C and LDL-C.
Objective To analyze the curative effect of nitric oxide (NO) and bosentan on treatment of the interruption of aortic arch (IAA) with ventricular septal defect (VSD) and serious pulmonary hypertension (SPH). Methods Thirty-two children with IAA and VSD combined SPH from January 2015 to May 2017 confirmed by cardiac CT and ultrasound in Children’s Hospital of Hebei Province were enrolled including 17 males and 15 females, aged 1.10-4.30 months (mean, 2.71±0.98 months) and weighing 3.33-6.10 kg (mean, 4.57±0.88 kg). The 32 children were randomly divided into two groups (n=16 in each), a NO group and a bosentan group. All the patients underwent interruption of aortic arch and ventricular septal defect repair. When patients returned to cardiosurgery intensive care unit (CSICU) half an hour later, patients in the NO group inhaled NO 20 ppm for 36 h and those in the bosentan group were given bosentan by nasogastric feeding 15 mg, twice a day. The cardic index, pulmonary/systemic pressure ratio, oxygenation index at 3 h, 6 h, 12 h, 24 h, 36 h after surgery were evaluated, and the differences between the two groups were compared. Results The pulmonary/systemic pressure ratio in the two groups increased at first and then decreased, while oxygenation index in the two groups decreased at first and then increased, and the differences in the same groups at the adjacent time points were statistically significant (P<0.05). The cardiac index in the two groups decreased at first and then increased, the differences in the same groups at the adjacent time points were statistically significant, except for 6 h and 12 h after surgery in the bosentan group (P>0.05). At postoperative 6 h, 12 h, the oxygenation index in the NO group was significantly higher than that in the bosentan group, and the pulmonary/systemic pressure ratio in the NO group was less than that in the bosentan group (P<0.01). The cardiac index in the NO group was higher than that of the bosentan group after 6 h, 12 h, 24 h of operation, which were statistically significant (P<0.05), and the cardic index of children in the NO group was greatly higher than that in the bosentan group after 12 h of surgery (P<0.01); at the same time point, the corresponding indexes were not statistically significant between the two groups (P>0.05). Conclusion NO inhalation in the treatment of IAA with VSD and SPH in children with early postoperative SPH is better than the bosentan, but in the late postoperative period, the effect is similar.
Objective To explore current results after staged operations in patients with functional single ventricle anomalies and pulmonary hypertension. Methods We retrospectively analyzed the clinical data of 129 patients with functional single ventricle anomalies and pulmonary hypertension undergoing pulmonary artery banding in our hospital between April 2008 and December 2015. There were 81 males and 48 females. There were 71 patients with double outlet of right ventricle, 17 patients with tricuspid atresia, 7 patients with transposition of great arteries, 33 patients with uni-ventricular heart, and one patient with complete atrio-ventricular septal defect. The surgical results, transition to Glenn procedure and subsequent transition to Fontan procedure were analyzed. Results The 129 patients underwent 159 operations of pulmonary artery banding totally. Hospital mortality was 4.7% (6/129). Nine patients were lost to follow-up. Eighty-seven (67.4%) patients underwent the second-stage Glenn procedure, and 43 patients(33.3%) underwent third-stage Fontan procedure. Two patients died after Glenn and 3 patients died after Fontan separately. There were 32 patients who accompanied with coarctation, interruption of aortic arch, heterotaxy, total anomalous pulmonary venous connection or atrio-ventricular valve regurgitation. Fifteen (46.9%) patients succeeded in transition to Glenn, and 6 (18.8%) patients succeeded in transition to Fontan. Fourteen patients developed obstruction of left ventricular outlet tract or bulbo-ventricular foramen. Conclusion Early pulmonary artery banding is an acceptable strategy for patients with single ventricle anomalies and pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good. Accompanied complex anomalies are risk factors for lower ratio of transition to Glenn and Fontan procedure.
Objective To analyze the relation between preoperative pulmonary artery pressure(PAP) and postoperative complications in heart transplant patients, and summarize the experience of perioperative management of pulmonary hypertension (PH), to facilitate the early period heart function recovery of postoperative heart transplant patients. Methods A total of 125 orthotopic heart transplant patients were divided into two groups according to preoperative pulmonary arterial systolic pressure(PASP) and pulmonary vascular resistance(PVR), pulmonary [CM(1583mm]hypertension group (n=56): preoperativePASPgt;50 mm Hg or PVRgt;5 Wood·U; control group (n=69): preoperative PASP≤50 mmHg and PVR≤5 Wood·U. Hemodynamics index including preoperative cardiac index (CI)，preoperative and postoperative PVR and PAP were collected by SwanGanz catheter and compared. The extent of postoperative tricuspid regurgitation was evaluated by echocardiography. Postoperative pulmonary hypertension was treated by diuresis，nitrogen oxide inhaling，nitroglycerin and prostacyclin infusion, continuous renal replacement therapy(CRRT)and extracorporeal membrane oxygenation(ECMO). Results All patients survived except one patient in pulmonary hypertension group died of multiorgan failure and severe infection postoperatively in hospital. Acute right ventricular failure occurred postoperatively in 23 patients, 10 patients used ECMO support, 10 patients with acute renal insufficiency were treated with CRRT. 124 patients were followed up for 2.59 months，7 patients died of multiple organ failure, infection and acute rejection in follow-up period, the survivals in both groups have normal PAP, no significant tricuspid regurgitation. No significant difference in cold ischemia time of donor heart, cardiopulmonary bypass(CPB) and circulation support time between both groups; but the patients of pulmonary hypertension group had longer tracheal intubation time in comparison with the patients of control group (65±119 h vs. 32±38 h, t=2.17，P=0.028). Preoperative PASP，mean pulmonary artery pressure(MPAP) and PVR in pulmonary hypertension group were significantly higher than those in control group, CI was lower in pulmonary hypertension group ［PASP 64.30±11.50 mm Hg vs. 35.60±10.20 mm Hg; MPAP 43.20±8.50 mm Hg vs. 24.20±7.20 mm Hg; PVR 4.72±2.26 Wood·U vs. 2.27±1.24 Wood·U; CI 1.93±0.62 L/（min·m2） vs. 2.33±0.56 L/（min·m2）; Plt;0.05］. Postoperative early PASP, MPAP and PVR in pulmonary hypertension group were significantly higher than those in control group (PASP 35.40±5.60 mm Hg vs. 31.10±5.70 mm Hg, MPAP 23.10±3.60 mm Hg vs. 21.00±4.00 mm Hg, PVR 2.46±0.78 Wood·U vs. 1.79±0.62 Wood·U; Plt;0.05). Conclusion Postoperative right heart insuficiency is related to preoperative pulmonary hypertension in heart transplant patients. Donor heart can quickly rehabilitate postoperatively by effectively controlling perioperative pulmonary hypertension with good follow-up results.
Objective To make an individualized treatment plan concerning a newborn with meconium aspiration syndrome combined with persistent pulmonary hypertension. Methods Based on the clinical questions raised by a newborn with meconium aspiration syndrome combined with persistent pulmonary hypertension, we searched The Cochrane Library (Issue 3, 2009), MEDLINE (1980 to June 2009), ACP Journal Club (1991 to June 2009), and Chinese Journal Fulltext Database (1994 to June 2009) for systematic reviews, randomized controlled trials (RCTs) and case-control studies. The quality of the included studies was assessed. Results A total of 9 RCTs, 1 health economic evaluation, 1 meta analysis, and 2 systematic reviews were considered eligible. The evidence indicated that the use of ECMO in infants of PPHN had shown a decreased risk of death, but not cost-saving from a societal perspective; iNO treatment could improve the PaO2 and resulted in a reduction in the incidence of requirement for ECMO; there were not randomized controlled trials regarding the treatment of PPHN by hyperventilation, high-frequency ventilation, alkali infusion, pulmonary vasodilators (magnesium sulfate, tolazoline, prostaglandin or prostacyclin, milrinone), surfactant therapy; oral sildenafil could lower oxygenation index (OI) and result in a reduction in the incidence of death. The individualized treatment plans of oral sildenafil were developed based on the available evidence, existing conditions of the hospital, and the values of children with families. After 1 month of treatment, the FiO2 returned to normal and symptoms were alleviated. Conclusion The treatment efficacies and the survival rates in meconium aspiration syndrome combined with PPHN have been improved by determining an individualized treatment plan according to evidence-based methods.