Objective To review and summarize the clinical outcomes of neonatal D-transposition of the great arteries by rapid two-stage arterial switch operation. Methods Between September 2002 and May 2003, five neonates with D-transposition of the great arteries were repaired by rapid two-stage arterial switch operation. The operative age was 83.0±72.2 day and weight was 4.7±0.9 kg. Because these patients came to the hospital late, the left ventricle was unable to accommodate the systemic pressure, so the left ventricle had to be prepared by pulmonary artery banding and systemic-pulmonary arterial shunt. After 6-9 days, the arterial switch procedure was performed. Results At first stage, one patient died of supraventricular tachycardia and oliguria after peritoneal dialysis. Four patients were repaired by arterial switch operation with no death. These patients were followed up for 2 to 10 months and had good development. The echocardiogram showed that there were no intracardiac residual shunt , the aorta and pulmonary artery anastomosis had no obstruction . The heart function was good, ejection fraction 0.68-0.77,fractional shortening 0.24-0.37. One patient had mild aortic valve regurgitation. Conclusion Rapid two-stage arterial switch operation is the best way for neonatal D-transposition of the great arteries that the left ventricle was unable to accommodate the systemic pressure.
Objective To explore the correlation between different ultrasound pulmonary artery systolic pressure (PASP) and high-resolution CT (HRCT) pulmonary artery width (PAD) in patients with chronic obstructive pulmonary disease (COPD). Methods A retrospective analysis was conducted on 473 patients with acute exacerbation of chronic obstructive pulmonary disease who were hospitalized in the First Hospital of Lanzhou University from January 2016 to December 2020. They were divided into four groups according to the degree of PASP elevation: PASP normal group: PASP≤36 mm Hg, 182 cases; mildly elevated group: PASP 37 to 50 mm Hg, 164 cases; moderately elevated group: PASP 51 to 70 mm Hg, 89 cases; severely elevated group: PASP>70 mm Hg, 38 cases. The PAD of chest HRCT and the width of the ascending aorta (AAD) on the same plane were measured, and the ratio of PAD to AAD (PAD/AAD) was calculated. The differences of PAD, AAD, PAD/AAD in different PASP groups of COPD were compared. The correlations between PASP, lung function, blood gas analysis and PAD, PAD/AAD were analyzed. Results With the decrease of FEV1%pred, FVC%pred, FEV1/FVC, PaO2 and SaO2 in the patients, PaCO2 increased, PASP gradually increased, PAD and PAD/AAD gradually increased. PAD and PAD/AAD were significantly different between the severely elevated PASP group and the other three groups, and there were significant differences between the moderately elevated group and the normal group, and between the moderately elevated group and the mildly elevated group. PASP and PaCO2 were positively correlated with PAD and PAD/AAD, and FEV1%pred, FVC%pred, FEV1/FVC, PaO2, SaO2 were negatively correlated with PAD and PAD/AAD. Multivariate logistic regression analysis showed that after adjusting for confounding factors, decreased FEV1%pred was an independent risk factor for PAD/AAD>1 in COPD patients. The receiver operating characteristic curve showed that the width of PAD and PAD/AAD had certain predictive value for PASP. Conclusions There is a significant positive correlation between different degrees of ultrasound PASP and PAD and PAD/AAD in patients with COPD. HRCT PAD has certain predictive value for PASP. The heavier the hypoxia and carbon dioxide retention, the worse the pulmonary ventilation function, the higher the pulmonary artery pressure, the greater the possibility of PAD and PAD/AAD.
Objective To evaluate the clinical value of computer tomography angiography( CTA) in the diagnosis of anomalous pulmonary artery. Methods Clinical data of 9 patients with anomalous pulmonary artery diagnosed by CTA were retrospectively analyzed, and CTA results were compared with those of echocardiographic examination. Results CTA clearly demonstrated the origin sites and topography of anomalous pulmonary arteries in all 9 patients. One patients presented with left pulmonary artery sling, and 8 patients presented with anomalous right pulmonary artery which originated fromthe proximal ascending aorta. Among them1 patient was with isolated anomalous origin of right pulmonary, and the others were complicated with patent ductus artery( 5 cases) , ventricular septal defect ( 3 cases) , and aortopulmonary septal defect ( 1 case) , etc. Conclusions CTA is highly valuable in the diagnosis of anomalous origin of pulmonary artery and associated abnormities by clearly developing the pathologic anatomic features and complicated cardiovascular malformations
Objective To improve the knowledge of pulmonary artery sarcoma ( PAS) and early diagnosis.Methods The clinical data of 8 patients with PAS confirmed by biopsy from April 2001 to April 2012 in Beijing Anzhen Hospital were retrospectively analyzed. Results There were 5 males and 3 females, with mean age of 46. 75 ±11. 47 years [ range: 32-67 years] . The main clinical manifestations were chest tightness, shortness of breath, intermittent syncope, heart palpitations at exertion, etc. Laboratory examinations showed the patients with PAS have no obvious hypoxemia and most of them have normal D-dimer level. Echocardiography revealed pulmonary hypertension, right ventricular enlargement, and echo of massive lumps in main pulmonary truck. Lower limb veins were normal in color doppler ultrasonography. Chest X-ray revealed prominent pulmonary artery segment, full segment of the right pulmonary artery, an increased hilum and pleural effusion. CT pulmonary angiography showed expansion of pulmonary artery, large filling defect in main pulmonary truck and left or right pulmonary artery, combined with pericardial effusion, pleural effusion. Lung ventilation/perfusion imaging did not match, showing radioactive sparse and defects in multiple lung segments and subsegments, involved 3 to 13 lung segments. Pulmonary angiography showed filling defects in the main pulmonary artery, left or right pulmonary artery. 8 patients were confirmed pathologically after operation. Pathological results showed leiomyosarcoma differentiation in 3 cases, undifferentiated sarcoma in2 cases, and undefined pathological type in 3 cases. All 8 patients were misdiagnosed as pulmonary embolism before surgery. The average days of misdiagnosis were 85. 6 ±21. 5 days. 7 cases were given simple surgical resection, one case underwent surgical resection combined with radiotherapy and chemotherapy. 7 cases were relieved and discharged, and one case died. Conclusion PAS is a rare disease clinically and is easily misdiagnosed as pulmonary embolism. Clinicians should enhance the recognition in order to diagnose early and treat comprehensively.
ObjectiveTo improve the knowledge of clinical features,image and pathology of Behcet's disease with the pulmonary artery aneurysm. MethodsOne typical female patient aged 40 years with recurrent pulmonary artery aneurysm caused by Behcet's disease was analyzed including clinical data,image and pathological features.The Pubmed (1967.1 to 2013.10) and Wanfang database (1982.1 to 2013.10) were searched with "Behcet's disease","pulmonary artery aneurysm" as search terms. ResultsRecurrent hemoptysis was major clinical manifestation of pulmonary artery aneurysm caused by Behcet's disease.Features of image including hilar enlargement,pulmonary artery aneurysm and primary thrombus.During the first hospitalization,the patient was diagnosed as pulmonary artery aneurysm.Then pulmonary lobectomy was performed and pathology examination revealed artery aneurysm and primary thrombus.During the second hospitalization,she was diagnosed as pulmonary thromboembolism and it was considered as failure of treatment of anticoagulant therapy.During the third hospitalization,she was diagnosed as pulmonary artery aneurysm of Behcet's disease,and recurrent hemoptysis was fully ameliorated by the combination treatment of glucocorticoid and immunodepressant. ConclusionPulmonary artery aneurysm is the typical feature of pulmonary vascular lesions of Behcet's disease,and recurrent hemoptysis is major clinical manifestation.CT angiography can identify location of pulmonary artery aneurysm.The key drugs of therapy are imunosuppressive drugs in combination with steroids.
Objective To explore and compare the diagnostic value of blood pressure, brain natriuretic peptide (BNP), pulmonary artery systolic pressure (PASP) in evaluating right ventricular dysfunction (RVD) in patients with acute pulmonary embolism (APE). Methods A retrospective study was conducted on 84 APE patients who were diagnosed by computed tomographic pulmonary angiography. The patients were divided into a RVD group and a non-RVD group by echocardiography. Eighteen clinical and auxiliary examination variables were used as the research factors and RVD as the related factor. The relationship between these research factors and RVD were evaluated by logistic regression model, the diagnostic value of BNP and PASP to predict RVD was analyzed by receiver-operating characteristic (ROC) curve analysis. Results The patients with RVD had more rapid heart rate, higher diastolic blood pressure, higher mean arterial pressure, higher incidence of BNP>100 pg/ml and higher incidence of PASP>40 mm Hg (allP<0 05="" upon="" logistic="" regression="" model="" bnp="">100 pg/ml (OR=4.904, 95%CI 1.431–16.806, P=0.011) and PASP>40 mm Hg (OR=6.415, 95%CI 1.509–27.261, P=0.012) were independent predictors of RVD. The areas under the ROC curve to predict RVD were 0.823 (95%CI 0.729–0.917) for BNP, and 0.798 (95%CI 0.700–0.896) for PASP. Conclusions Blood pressure related parameters can not serve as a predictor of RVD. Combined monitoring of BNP level and PASP is helpful for accurate prediction of RVD in patients with APE.
Objective To summarize our clinical experience of pulmonary artery banding （PAB） for the treatment of complex congenital heart diseases as a palliative procedure.?Methods?From January 1997 to November 2010, 138 patients with complex congenital heart diseases underwent PAB in Fu Wai Hospital. There were 87 male patients and 51 female patients with their age of 22.2±26.5 months and average body weight of 7.5±4.6 kg. All the 138 patients were divided into 3 groups according to the purpose of PAB：left ventricular retraining （group 1, n=55）, initial procedure for functional single ventricle with unobstructed pulmonary blood flow （group 2, n=32） and initial palliative procedure followed by later biventricular repair （group 3, n=51）. The intraoperative and postoperative clinical parameters of all participants were observed, and follow-up was made for these 3 groups of patients.?Results?The in-hospital mortality of PAB was 5.1% （7/138）. Three patients underwent re-banding procedure to adjust the size of banding. In group 1, there was 1 postoperative death. Among the 55 patients, 36 patients with dextro-transposition of great arteries received PAB at an average age of 19.6±29.5 months, 29 patients of whom underwent concomitant modified Blalock-Taussig shunt. After an average training interval for 42 days, 83.3% of them（30/36）successfully received arterial switch operation. The other 19 patients in group 1 with isolated corrected transposition of great arteries received PAB at an average age of 45.3±27.2 months. Afteran average training interval for 9 months, 42.1% of them （8/19） successfully received double-switch operation. In group 2, there was 2 postoperative death. Thirty-two patients with functional single ventricle and unobstructed pulmonary blood flow received PAB at an average age of 14.1±14.9 months. Their postoperative mean pulmonary artery pressure decreased significantly from 34.00±10.00 mm Hg to 23.00±7.40 mm Hg, and their oxygen saturation of blood （SpO2） significantly decreased from 92.60%±5.90% to 83.30%±6.30%. After a median interval of 2 years, 18.8% of them （6/32） underwent right heart bypass operation. In group 3, there was 4 postoperative death. Fifty-one patients received PAB at an average age of 20.60±25.60 months. After PAB procedure, the ratio of systolic pulmonary artery pressure and systolic blood pressure significantly decreased from 0.81±0.14 to 0.46±0.15, and their SpO2 significantly decreased from 93.10%±7.60% to 85.00%±10.00%. After a median interval of 6 months, 23.5% of them （12/51） received biventricular repair.?ConclusionAlthough PAB is a palliative procedure with comparatively high risks, it still plays an indispensable role in terms of protecting pulmonary vascular beds, retraining ventricular function and two-stage surgical correction for the treatment of complex congenital heart diseases.
Abstract: Objective To investigate the safety and feasibility of fast track (FT) treatment in young children with atrioventricular septal defect (CAVSD) and pulmonary artery hypertension (PAH) following surgical repair. Methods A total of 51 young children patients including 24 boys and 27 girls with age at 12.5±8.9 months from 4 to 36 months, underwent CAVSD repair in the pediatric surgery department of Fu Wai Hospital from January 2006 to March 2009. Among them, 21 patients were administered FT management. PICU length of stay and the rate of reintubation were analyzed retrospectively and the decrease of pulmonary artery pressure (PAP) after operation was also measured. Results Twentyone patients under FT treatment were extubated within 8 hours after operation. The mean pulmonary artery pressure(MPAP) decreased significantly after surgery (39.59 mm Hg vs.24.50 mm Hg,t=5514,Plt;0.05). PICU length of stay was 2.05±0.87 d (18 h-3 d). One patient was reintubated due to lung infection, which had nothing to do with the FT treatment. During the followup which lasted for 3 to 6 months, 21 patients had good heart function with no reoperation or death. Conclusion FT treatment is safe and feasible to some CAVSD patients associated with PAH, and shorter PICU length of stay can be achieved. The validation of FT model for the CAVSD patients with severe PAH needs research with large sample.
Abstract： Objective To evaluate clinical outcomes of pulmonary artery banding for morphologic left ventricular training in corrected transposition of the great arteries.?Methods?A total of 89 patients with corrected transposition of the great arteries underwent surgical repair in Shanghai Children’s Medical Center from January 2007 to December 2011 year. Among them, 11 patients underwent pulmonary artery banding, whose clinical records were retrospectively analyzed. Except that one patient was 12 years, all other patients were 3 to 42 （16.40±11.67） months old and had a body weight of 6 to 32 （11.70±7.20）kg. All the patients were diagnosed by echocardiogram and angiocardiogram.?Results?There was no postoperative death after pulmonary artery banding in 11 patients. The pulmonary arterial pressure/systemic blood pressure ratio （Pp/Ps） was 0.3 to 0.6 （0.44±0.09） preoperatively and 0.6 to 0.8 （0.70±0.04） postoperatively with statistical difference （P＜0.01）. Tricuspid regurgitation was mild in 2 （18.2%） patients, moderate in 5 （45.4%）, severe in 4 （36.4%）preoperatively，and none in 2（18.2%）patients, mild in 7 （63.6%），and mild to moderate in 2 （18.2%）postoperatively. Five patients underwent staged double-switch operation after pulmonary artery banding at 15.20±8.31 months, and 1 patient died. The other 6 patients were followed up for 18.83±3.43 months, and echocardiogram showed tricuspid regurgitation as trivial in 2 （33.3%）, mild in 3 （50.0%）, and moderate in 1 （16.7%）patient.?Conclusions?In patients with corrected transposition of the great arteries, pulmonary artery banding is helpful to reduce tricuspid regurgitation, and morphologic left ventricle can be trained for staged double-switch operation with good clinical outcomes. It is important to follow up these patients regularly to evaluate their morphologic left ventricular function and tricuspid regurgitation after pulmonary artery banding.
Objective To confirm the changes of pulmonary artery pressure, neo pulmonary artery stenosis and reoperation in children with unilateral absence of pulmonary artery (UAPA) undergoing pulmonary artery reconstruction. Methods The clinical data of the infants with UAPA undergoing pulmonary artery reconstruction in our hospital from February 19, 2019 to April 15, 2021 were analyzed. Changes in pulmonary artery pressure, neo pulmonary artery stenosis and reoperation were followed up. Results Finally 5 patients were collected, including 4 males and 1 female. The operation age ranged from 13 days to 2.7 years. Cardiac contrast-enhanced CT scans were performed in all children, and 2 patients underwent pulmonary vein wedge angiography to confirm the diagnosis and preoperative evaluation. Preoperative transthoracic echocardiography and intraoperative direct pulmonary arterial pressure measurement indicated that all 5 children had pulmonary hypertension, with a mean pulmonary arterial pressure of 31.3±16.0 mm Hg. Pulmonary arterial pressure decreased immediately after pulmonary artery reconstruction to 16.8±4.2 mm Hg. The mean follow-up time was 18.9±4.7 months. All 5 patients survived during the follow-up period, and 1 patient had neo pulmonary artery stenosis or even occlusion and was re-operated. Conclusion Pulmonary artery reconstruction can effectively alleviate the pulmonary hypertension in children with UAPA. The patency of the neo pulmonary artery should be closely followed up after surgery, and re-pulmonary angioplasty should be performed if necessary.