Abstract: Objective To analyze the results of preoperative evaluation of the operability of chronic thromboembolic pulmonary hypertension (CTEPH), and to find parameters to define the inconsistency between the operable lesions of CTEPH and pulmonary vascular resistance (PVR). Methods A total of 133 patients with CTEPH admitted into Anzhen Hospital were enrolled for preoperative assessment of operability from March 2002 to May 2010. There were 86 males and 47 females with an age of 49.10±22.70 years. The patients were divided into operable group (group A, n=82，age of 47.80±21.60 years) and inoperable group (group B, n=51, age of 50.30±23.40 years) according to the assessment suggestion. We evaluated the occluded pulmonary segment（OPS） of all the patients through pulmonary ventilation/perfusion scintigraphy， and measured the plasma aminoterminal Btype natriuretic peptide (NT-pro BNP) and PVR. Then the ratio of NT-pro BNP to OPS and PVR to OPS were calculated. [CM(159mm]Results Out of the 82 patients with CTEPH in group A, 81 were positive in the anesthesia test and were subject to pulmonary thromboendarterectomy(PTE). In the whole cohorts of operated patients, there was one early death due to persistent pulmonary hypertension and right heart failure. The specificity of the anesthesia test was 98.78%. Eighty patients (98.77%) were followed up in this group for a period of 1 to 95 months (42.70±28.40 months). During the followup, there was one late death due to pulmonary artery hypertension crisis. Among the 51 patients with CTEPH in group B, there were 32 patients (62.75%) with surgically inaccessible lesions, 13 patients (25.49%) with surgical accessible CTEPH concomitant with severe diseases, and 6 patients (11.76%) with inconsistency between the surgical accessible lesion and high PVR. The ratio of NT-pro BNP to OPS and PVR to OPS for the 81 positive patients in group A was in the range of 80-150 pg·ml-1/OPS and 50-100 dyn·s·cm-5/OPS, respectively. The ratio of NT-pro BNP to OPS (315.00±83.00 pg·ml-1/OPS vs. 115.60±40.50 pg·ml-1/OPS, P=0.000) and PVR to OPS (190.00±57.00 dyn·s·cm－5/OPS vs. 76.40±26.30 dyn·s·cm-5/OPS, P=0.000) for the 6 patients with incosistency between the surgical accessible lesion and high PVR in group B were significantly higher than that for the 81 positive patients in group A. Conclusion Surgically inaccessible CTEPH lesions, CTEPH concomitant with severe diseases, and inconsistency between surgical accessible lesion and high PVR are the three most frequent reasons for denying PTE procedure. The ratio of NTpro BNP to OPS and PVR to OPS may serve as the parameters to define the inconsistency between the surgical accessible lesion and high PVR. Anesthesia test before the PTE procedure may serve as the last evaluation method for the assessment of the operability of CTEPH.
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 investigate the effects of simvastatin on the collagen synthesis of rat pulmonary arterial smooth muscle cells ( PASMCs ) induced by hypoxia. Methods Under hypoxic condition, rat PASMCs were cultured with different concentrations of simvastatin. Collagen synthesis of PASMCs with or without simvastatin were measured by 3H-proline incorporation assay. The mRNA expression of TGF-β1 and the contents of super oxide dismrtase ( SOD) ,malondialdehyde ( MDA) in mediumwere also measured. Results The incorporation data of 3H-TdR in the hypoxia group was significantly increased as compared with that in the control group ( P lt;0. 01) , and simvastatin significantly reduced the incorporation data of 3H-TdR induced by hypoxia. The expression of TGF-β1 mRNA in the hypoxia group was significantly increased as compared with that in the control group ( P lt; 0. 01 ) , and simvastatin could significantly inhibited hypoxia-induced expression of TGF-β1 mRNA in a dose-dependent manner. Compared with the hypoxia group, the expression of TGF-β1 mRNA decreased by 55% in simvastatin( 10 - 6mol /L) group ( P lt; 0. 01) , and by 70% ( P lt; 0. 01) in simvastatin ( 10 - 5mol /L) group. Compared with the control group, the activity of SOD was reduced and the contents of MDA were increased significantly in the hypoxia group. Simvastatin can increase the activity of SOD and reduced the content of MDA in a dose-dependent manner. Conclusions Simvastatin can decreases collagen synthesis of PASMCs. This effect might be explained that simvastatin can reduce lipid peroxide and expression of TGF-β1 mRNA.
Objective To observe the efficacy and safety of sildenafil in one case of PoPH patients. Methods A case of PoPH patient who was successfully treated with sildenafil was retrospectively analyzed，and literatures related sildenafil and PoPH were reviewed. Results A case of PoPH patient admitted with dyspnea on extetion was diagnosed with echocardiography，gastroscrope and other examinations. She was treated with sildenafil and responded well by decreased pulmonary arterial pressure，improved exercise tolerance and life quality，without obvious side effects. Literatures review suggested that PoPH is an under-recognized but life-threatening complication of cirrhosis or noncirrhotic portal hypertension with poor prognosis which exists in decompensated chronic liver diseases patients. The most classic symptoms of PoPH is progressive dyspnea on extertion. PoPH patients may benefit from sildenafil therapy with decreased pulmonary arterial pressure and improved life quality. Conclusions Theawareness of PoPH should be increased and Sildenafil may be effective and safe for PoPH patients. However，more evidences from clinical trials are needed.
Congenital heart disease refers to the structural or functional abnormality of the macrovascular in the heart or thoracic cavity caused by the failure of the formation of the heart and large blood vessels during the embryonic development or the abnormal closure of the heart or the closure of the channel after birth. In the past few years, a new and broader definition of structural heart disease has been gradually proposed. Structural heart disease narrowly refers to the pathological and physiological changes of the heart caused by abnormal anatomical structures in the heart, including congenital heart disease. A few decades ago, congenital heart disease was considered as a pediatric disease, because most patients with severe lesions rarely survive to adulthood. Due to recent advances in echocardiography, anesthesia, intensive care, percutaneous intervention, especially cardiac surgery in recent decades, the treatment and intervention strategies for congenital heart disease in children have been greatly improved, a fatal defect in childhood can now be successfully repaired or alleviated. Because of these successes, more than 90% of congenital heart disease patients are expected to survive to adulthood, which has led to emerge a new population: adult patients with congenital heart disease. Adult congenital heart disease patients are different from children. Pulmonary hypertension leads to right heart failure and eventually progresses to whole heart failure. The appearance of Eisenmenger syndrome leads to severe cyanosis and worsening of the disease. At present, the continuous development of mechanical assisted circulation support devices and heart or cardiopulmonary transplantation technology has increased the survival rate of end-stage adult congenital heart disease patients with heart failure. The high incidence of cardiovascular events in pregnant patients requires comprehensive multidisciplinary team care and early coordination planning for delivery, including early counseling for pregnancy-related risks, close monitoring of cardiac function and regular scan of fetal assessment. The prenatal and postpartum integrated diagnosis and treatment model and the development of intrauterine treatment technology reduce the incidence of congenital heart disease in adults from the source through fetal intervention. Other complications such as arrhythmia, infective endocarditis, cerebrovascular accidents, and other medical underlying metabolic diseases also challenge future diagnosis and treatment. The incidence and epidemiology of adult congenital heart disease, pulmonary hypertension and end-stage heart failure complications, as well as prenatal and postpartum integrated diagnosis and treatment and intrauterine treatment are summarized in this review.
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.
ObjectiveTo determine the ability of cardiopulmonary exercise testing (CPET) to distinguish chronic thromboembolic pulmonary hypertension (CTEPH) from chronic thromboembolic disease (CTED). MethodsFifty patients diagnosed with CTED and fifty-eight patients with CTEPH in the the First Affiliated Hospital of Guangzhou Medical University from April 2019 to February 2022 were retrospectively included. The basic characteristics including age, gender, body mass index, symptom duration, and N-terminal prohormone of brain natriuretic peptide (NTpro-BNP), parameters of arterial blood gas analysis, right heart catheterization, echocardiography, pulmonary function, and CPET were all compared between patients with CTED and those with CTEPH. ResultsPatients with CTEPH displayed longer symptom duration, increased NTpro-BNP, decreased arterial partial pressure of oxygen, larger right atrial and ventricular diameters, and impaired worse resting pulmonary diffusing function compared with those with CTED (P<0.05). However, there was no statistically significant difference in the resting pulmonary ventilation function between the two groups (P>0.05). Among the CPET parameters of the CTEPH group, peak exercise oxygen uptake per kilogram, oxygen uptake at anaerobic threshold, oxygen pulse, oxygen uptake efficiency slope and oxygen saturation were all decreased, while the minute ventilation-carbon dioxide production at anaerobic threshold (VE/VCO2@AT) and VE/VCO2 slope were increased (P<0.05). However, there was no statistically significant difference in heart rate reserve and breathing reserve (P>0.05). Furthermore, VE/VCO2@AT showed high accuracy for distinguishing CTEPH from CTED (sensitivity, 0.825; specificity, 0.860; and the area under the receiver operating characteristic curve 0.897). ConclusionsPatients with CTEPH showed more significant decreased exercise endurance, diffusion dysfunction, and hypoxemia during exercise and insufficient increase in cardiac output compared with CTED patients. Therefore, it is feasible to apply CPET as a new objective examination to distinguish CTED from CTEPH.
ObjectiveTo explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease.MethodsFrom May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed.ResultsPreoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥1.2 m/s at discharge was an independent risk factor for mortality and restenosis.ConclusionThe prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.
Medical nitric oxide (NO) flow control system plays an important role in lowering pulmonary hypertension. The design requirements, overall scheme, delivery system and hardware circuits of a medical NO flow control system were introduced in this paper. Particularly, we proposed the design of NO delivery system and hardware circuits in detail. To deliver nitric oxide of a variable concentration, the designed system needs to work with a ventilator. The system can adjust and monitor the inhaled nitric oxide concentrations and send out sound and light alarms when the inhaled nitric oxide concentrations are out of the set range. To validate reliability and efficacy, we measured specifications such as linearity, stability and response time of the proposed NO flow control system by continuously administering nitric oxide into inspiratory circuit to deliver nitric oxide of variable concentrations to a test lung. The experiments showed that these specifications can meet the desired requirements.
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.