Superior vena cava syndrome and pulmonary artery stenosis in a patient with lung metastases of bladder cancer / 复旦学报（医学版）

Superior vena cava syndrome(SVCS)is a group of clinical syndromes caused by obstruction of the superior vena cava and its major branches from various causes.Pulmonary artery stenosis(PS)is a complication of lung cancer or mediastinal tumours.SVCS combined with PS due to pulmonary metastases from bladder cancer is extremely rare and has not been reported in the literature.Here we reported an old male patient with pulmonary metastases from bladder cancer presenting with swelling of the head,neck and both upper limbs.SVCS combined with PS was clarified by pulmonary artery computed tomography angiography(CTA)and digital subtraction angiography(DSA).Endovascular stenting was used to treat SVCS.Angiography also showed that PS had not caused pulmonary hypertension and did not need to be treated.The swelling of the patient's head,neck and upper limbs was gradually reduced after the procedure.

The effect of LeCompte maneuver on mid-to-long term reintervention after arterial switch operation in children with side-by-side Taussig-Bing anomaly / 中国胸心血管外科临床杂志

@#Objective    To explore the effect of LeCompte maneuver on in-hospital mortality and mid-to-long term reintervention after single-stage arterial switch operation in children with side-by-side Taussig-Bing anomaly. Methods     Clinical data of patients diagnosed with side-by-side Taussig-Bing anomaly and undergoing single-stage arterial switch operation in Shanghai Children’s Medical Center from 2006 to 2017 were retrospectively analyzed. Patients were divided into two groups based on whether LeCompte maneuver was performed: a LeCompte maneuver group and a non LeCompte maneuver group. The clinical data of two groups were compared. Results    Finally 92 patients were collected. LeCompte maneuver was performed in 32 out of 92 patients with a median age of 65.0 days and an average weight of 4.3 kg, among whom 24 (75.0%) were male. Fifteen (46.9%) patients received concomitant aortic arch repair while 12 (37.5%) patients were associated with coronary artery malformation. LeCompte maneuver was not performed in 60 patients with a median age of 81.0 days and an average weight of 4.8 kg, among whom 45 (75.0%) were male. Twenty-two (36.7%) patients received concomitant aortic arch repair while 35 (58.3%) patients were associated with coronary artery malformation. The average cardiopulmonary bypass duration of the LeCompte maneuver group showed no statistical difference from the non LeCompte maneuver group (179.0±60.0 min vs. 203.0±74.0 min, P=0.093). The in-hospital mortality of the two groups were 6 (18.8%) and 7 (11.7%), respectively, which also showed no statistical difference (P=0.364). The median follow-up period was 4.1 (1.6, 7.5) years for 79 patients with 8 lost to follow-up, and no death was observed. Kaplan-Meier curve and log-rank test showed no statistical difference in overall mid-to-long term reintervention rate (P=0.850) as well as right ventricular outflow tract and pulmonary artery reintervention rate (P=0.240) with or without LeCompte maneuver. Conclusion    Whether or not to perform LeCompte maneuver shows no statistical impact on in-hospital mortality and mid-to-long term reintervention rate of single-stage arterial switch operation for side-by-side Taussig-Bing anomaly.

Difficulty in Weaning from Cardio-Pulmonary Bypass due to Pulmonary Artery Extrinsic Stenosis Caused by a Hematoma—a Case of Ruptured Aortic Arch Aneurysm / 日本心臓血管外科学会雑誌

During aortic arch replacement in response to an aortic arch aneurysm sealed rupture, we experienced a case in which weaning from cardio-pulmonary bypass (CPB) became difficult, and pulmonary artery stenosis developed due to expanded hematoma. A 77-year-old man was raced to our hospital due to subjective symptom of chest/back pain. With a recognition of aortic arch aneurysm and hematoma around the aneurysm, it was diagnosed as an aortic arch aneurysm sealed rupture. Even though an elective aortic arch replacement was implemented using an open stent graft, reduction in blood pressure and poor oxygenation was observed at the process of CPB weaning. As a result of intraoperative pulmonary arteriography, severe stenosis was revealed on both left and right pulmonary arteries. With placement of a self-expanding stent, weaning from CPB was successfully completed. Being transferred to other hospital on day 60 after the surgery in order to continue rehabilitation, the man visits our hospital as an outpatient on his own as of now. As it is considered to be a rare case that weaning from CPB was successfully performed by pulmonary stenting in response to progressed intraoperative pulmonary artery stenosis caused by expanded hematoma after heparin administration, the details are reported here.

The application of Z-scores in diagnosis of fetal pulmonary artery stenosis / 中国医师杂志

Objective The purpose of this study was to produce cut-off values of fetal pulmonary artery stenosis (PS),allowing the application of Z-scores to evaluate fetal pulmonary artery.Methods A total of 214 normal singleton fetuses and 107 singleton fetuses with PS were included in this study.They were measured for standard biometry:biparietal diameter (BPD) and femoral diaphysis length (FL) and an assessment of gestation age (GA).The standardized fetal echocardiographic measurements included diameters of pulmonary valve annulus (PV),pulmonary artery (PA),ductus arteriosus (DA) and aorta (AO).Then we calculated the Z-scores from parameters of fetal size.The Z-scores differences of PV,PA,DA and AO in PS group were compared.The receiver operator characteristic (ROC) curves of PV and PA Z-scores were obtained by statistical analysis.Results Compared with the normal group,the Z-scores of PV,PA and DA decreased in the PS group,whereas the Z-scores of AO increased (P ＜0.01 for all).The critical values of Z-score for PV in diagnosing PS with BPD,FL and GA as variables were-1.13,-1.33,-1.28,with sensitivity 94.2％,90.3％,93.5％,specificity 60.0％,65.0％,62.5％,respectively.The critical values of Z-score for PA with BPD,FL and GA as variables were-1.01,-0.98,-1.01,with sensitivity 96.8％,96.8％,96.1％,specificity 65.0％,65.0％,62.5％,respectively.Conclusions In the fetus with pulmonary artery stenosis,PV,PA and DA Z-scores decreased,while AO Z-scores increased.The pulmonary artery Z-scores had important application value in quantitative evaluation and diagnosis of mild fetal pulmonary artery stenosis.

Computed Tomography Pulmonary Vascular Volume Ratio Can Be Used to Evaluate the Effectiveness of Pulmonary Angioplasty in Peripheral Pulmonary Artery Stenosis

OBJECTIVE: To explore whether computed tomography (CT) pulmonary vascular volume ratio can be used to evaluate the effectiveness of pulmonary artery angioplasty in patients with peripheral pulmonary artery stenosis. MATERIALS AND METHODS: Changes in CT pulmonary vascular volume ratio between serial cardiothoracic CT examinations were calculated in 38 patients. Fifteen patients underwent interim pulmonary artery angioplasty (group 1), while 23 did not (group 2). According to the effectiveness of pulmonary artery angioplasty, patients in group 1 were further divided into group 1A (improved or aggravated) and group 1B (ineffective). Changes in the pulmonary vascular volume percentages among the three groups (group 1A, group 1B, and group 2) on serial CT examinations were compared. RESULTS: Pulmonary artery angioplasty on serial CT examinations was successful in seven patients, ineffective in seven patients, and aggravated in one patient. As a result, eight patients were included in group 1A and seven were included in group 1B. Changes in the CT pulmonary vascular volume percentages in group 1A were statistically significantly greater than those in group 1B (11.6 ± 5.6% vs. 2.7 ± 1.6%, p 0.1). CONCLUSION: CT pulmonary vascular volume ratio can be used to evaluate the effectiveness of pulmonary artery angioplasty in patients with peripheral pulmonary artery stenosis.

Pulmonary Artery Stenosis due to Lung Carcinoma: A Rare Cause of Dyspnea

Acquired bilateral pulmonary artery stenosis in adults due to lung malignancy is infrequently reported. We describe an adult male who presented to us with chief complaints of dyspnea on exertion and one episode of hemoptysis. Two dimensional transthoracic echocardiography with color Doppler showed presence of an extra cardiac mass causing severe extrinsic compression of both the right and left pulmonary artery leading to high pressure severe tricuspid regurgitation and extension of the mass into the left atrium. Three dimensional transthoracic echocardiography clearly delineated the anatomy of the left atrial mass and its surrounding anatomical relationship. The diagnosis of non small cell lung carcinoma was confirmed by multidetector computed tomography (MDCT) and with MDCT guided biopsy with histopathology. Patient succumbed one month later due to an episode of massive hemoptysis.

Severe Pulmonary Hypertension Caused by Pulmonary Artery Stenosis in a Patient with Behcet's Disease Treated by Balloon Angioplasty / 대한내과학회지

Pulmonary artery stenosis that causes pulmonary hypertension is usually observed in congenital anomalies and is seldom observed in adults. A 47-year-old female patient came to the hospital for aggravated dyspnea and peripheral edema. Severe pulmonary hypertension was found on an echocardiography examination, along with dilation of the main pulmonary artery and alias flow at the bifurcation sites of both pulmonary arteries. Under coronary angio-computed tomography, 70% high grade stenosis was observed on both sides of the proximal pulmonary arteries without showing atherosclerotic plaque or a thrombus. The patient's symptoms improved after performing pulmonary artery angiography and angioplasty. Thus, we report our experience of a patient with Behcet's disease and severe pulmonary hypertension caused by pulmonary artery stenosis, which was treated with percutaneous pulmonary angioplasty.

The radical surgery for tetralogy of Fallot combined with right pulmonary artery stenosis / 中国综合临床

Objective To explore the radical surgery for tetralogy of Fallot combined with right pulmonary artery stenosis.Methods Thirteen cases with tetralogy of Fallot combined with right pulmonary artery stenosis accepted radical surgery,among them 7 cases who-had a long right pulmonary artery stenosis accepted aortic transaction for radical surgery of tetralogy of Fallot;5 cases who had the entrance stenosis of the right pulmonary artery accepted right pulmonary artery patch expansion for radical surgery of tetralogy of Fallot;One case who had the distortion and stenosis after pulmonary shunt accepted the radical surgery for tetralogy of Fallot after seperation and expansion.Results There were no operation-related deaths,post-operative low cardic output and perfusion lung occurred for all the cases.Ten cases had grade 1 cardiac function and 3 cases had grade 2,respectively.Conclusion Right pulmonary artery stenosis increased the risks during the radical surgery for tetralogy of Fallot,but it is still the primary operation choice.

Changes of the Biventricular Outflow Tract after a Half Turned Truncal Switch Operation in Patients with Transposition of the Great Arteries, a Ventricular Septal Defect and Pulmonary Stenosis: 2 case reports / 대한흉부외과학회지

Rastelli repair has been considered the procedure of choice for surgically repairing transposition of the great arteries combined with ventricular septal defect and pulmonary stenosis. However, the long term results have been less than optimal and these patients who ungo this procedure can eventually display conduit obstruction, left ventricular outflow tract obstruction and arrhythmias. Many new procedures are now available and they are technically challenging for making a more normal anatomic repair. In our hospital, two patients who had a TGA with VSD and PS have been repaired with a Half Turned Truncal Switch Operation and a Lecompte maneuver in 2003 and 2006, respectively. We report on our two experiences with performing a Half Turned Truncal Switch Operation, and we discuss the changes of the biventricular outflow tract.

Numerical analysis on the mechanism causing the left pulmonary artery stenosis after repair by tetralogy of fallot / 医用生物力学

Objective To investigate the influence from the left pulmonary artery (LPA) with different stenosises (50%,20%,0%) after complete repair of tetralogy of fallot (TOF) on the hemodynamic features using computational fluid dynamics(CFD). Method The 3D models were reconstructed by the computer tomography (CT) images. CFD simulations were performed on these three models to describe the flow characteristics of the main pulmonary artery and the bifurcations such as average speed in LPA and right pulmonary artery(RPA), flow patterns, static pressure and wall shear stress. Results In the unsteady state simulation, different regurgitation and flow velocity distribution can be easily found in the start of LPA due to the stenosis rate of LPA, while the flow velocity distribution is uniform in the RPA. The flow velocity distribution, static pressure, pressure drop and wall shear stress are also different in these three models. Conclusions It is important to expand the branch pulmonary arteries during the TOF operation. LPA stenosis may be an early and important cause of pulmonary artery regurgitation. The use of CFD simulation can provide the early reference information for the effect after TOF operation.

Modified Nikaidoh Procedure for Patient with TGA, Restrictive VSD, and PS / 대한흉부외과학회지

The surgical management of complete transposition of the great arteries, ventricular septal defect, and pulmonary stenosis still remain a significant challenge. The Rastelli (REV procedure) remains the most widely applied procedure for surgical repair of these lesions. Although the Rastelli procedure can be performed with good early results, the intermediate- and long-term results have been less than satisfactory because of deterioration of the hemodynamic performance of the LVOT or RVOT. We performed a modified Nikaidoh procedure as an alternative surgical procedure in a 19-month-old boy weighing 10.4 kg with this anomaly. Aortic translocation with biventricular outflow tract reconstruction resulted in a more "normal" anatomic repair and postoperative echocardiography showed straight, direct, and unobstructed ventricular outflow.

Aortic Translocation for Complete Transposition of the Great Arteries with a Ventricular Septal Defect and Pulmonic Stenosis / 대한흉부외과학회지

The Rastelli operation has been a standard procedure for repairing complete transposition of the great arteries combined with a ventricular septal defect and pulmonary stenosis. Yet this procedure has several shortcomings, including the risk of incurring left ventricular outflow tract obstruction on long-term follow-up. In this regard, aortic translocation has recently been regarded as a potent alternative to Rastelli's operation. We report here on a case of complete transposition of the great arteries that was combined with an inlet-extended perimembranous ventricular septal defect and pulmonary stenosis in a 2-year-old boy. All the problems were successfully repaired using the aortic translocation technique. Postoperative echocardiography showed a straight and wide left ventricular outflow tract.

Half-turned Truncal Switch Operation for Transposition of Great Arteries, Ventricular Septal Defect and Pulmonic Stenosis / 대한흉부외과학회지

The surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis remains a challenge. The Rastelli operation or Lecompte operation is the preferred surgical procedure, but its long-term results are not optimal because of a warped left ventricular outflow tract through a space-occupied intraventricular tunnel and a contrived right ventricular outflow tract. We performed a half-turned truncal switch operation as an alternative surgical procedure in a 3-year-old boy (weighing 9.6 kg) with this anomaly. Postoperative echocardiography showed laminar flow through straight and nonobstructive aortic and pulmonary ventricular outflow tracts.

Long-term Results of Modified Lecompte Procedure for the Anomalies of Ventriculoarterial Connection / 대한흉부외과학회지

Background: The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2+/-20.3 (range: 3~83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2+/-6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3+/-4.2%, and 87.0+/-5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6+/-4.5%, 73.9+/-7.3%, and 54.0+/-10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

Morphologic Changes of the Pulmonary Arteries after Stent Implantation on Branch Pulmonary Artery Stenosis: Impact of Pulmonary Insufficiency

PURPOSE: Intravascular stent implantation for the treatment of postoperative branch pulmonary artery(PA) stenosis has been used successfully. However, the cross sectional area of contralateral branch PA does not regress in spite of the successful dilation of the stenotic branch PA after stent implantation. We analyzed the morphologic and hemodynamic factors on the size of branch PA after successful stent implantation. METHODS: The subjects in our study were 23 children who had undergone stent implantaion from Jan. 1995 to Jul. 2002 in the Division of Yonsei Pediatric Cardiology. We evaluated the cross sectional area index(CSAI) of branch PA before and after stent implantation at follow-up catheterization. We also investigated factors such as residual pulmonary stenosis, pulmonary regurgitation(PR), systolic pressure of right ventricle, and lung perfusion scan. RESULTS: The CSAI of the RPA without stenosis changed from mean 238+/-17 mm2/BSA to mean 249+/-20 mm2/BSA(P=0.47), but didn't regress. The CSAI of the LPA with stenosis was increased effectively by stent implantation from the mean 102+/-12 mm2/BSA to mean 125+/-11 mm2/BSA(P< 0.05). At follow up after stent implantation, the CSAI of PA is correlated with the residual PR fraction after stent implantation. CONCLUSION: In a group with increased residual PR, CSAI of RPA was found to be significantly increased between the pre- and post-stages of stent implantation. So, we suspect that the pulmonary regurgitation remaining after right ventricle outlet tract(RVOT) dilatation surgery is correlated with the increase in CSAI of RPA.

Absent Pulmonary Valve with Intact Ventricular Septum, PDA, ASD / 대한흉부외과학회지

Absent pulmonary valve syndrome with intact ventricular septum(APVS with IVS) is a rare congenital anomaly. The severe form of this syndrome, characterized by severe respiratory distress presented soon after birth, has been attributed to the compression of the airways caused by aneurysmal dilatation of pulmonary artery. Several operative treatments such as pulmonary valve insertion, or reductive angioplasty of pulmonary artery have been applied. We present a 3-day-old male who showed improvement after PDA ligation, reductive angioplasty of pulmonary artery, pulmonary valve reconstruction, and ASD closure.

Main Pulmonary Artery Stenosis Caused by Fibrocalcified Mass in a Young Infant

We present a rare case of main pulmonary artery stenosis secondary to protruding fibrous material in the main pulmonary artery associated with patent ductus arteriosus. A 1-month-old baby boy manifested cardiac murmur. Echocardiogram showed circumferential high echogenic mass inside the main pulmonary artery with pressure gradient of 49 mmHg and patent ductus arteriosus. The mass did not regress during 3 months' follow-up period. Angiographic images showed that the circular filling defect was located at the main pulmonary artery distal to pulmonary valve, and pulmonary valve and both pulmonary arteries were normal. After surgical removal of the circumferential material and ductus ligation, the pressure gradient became negligible. The material was consisted of scarcely cellular fibrous tissue, abundant coagulum of fibrinous material and dense calcification.

Corrective Surgery of Congenital Cardiac Anomalies in the Noonan syndrome: Report of two cases / 대한흉부외과학회지

Noonan syndrome is characterized by a Turner-like phenotype and a normal karyotype associated with congenital abnormalities, such as short stature, variable mental retardation, hypertelorism, webbed neck, low posterior hair line, skeletal malformation and congenital cardiovascular defect. Two third of Noonan syndrome have cardiac anormalies, half with pulmonary stenosis. We have experienced two cases of pulmonary stenosis associated with other cardiac anomalies in Noonan syndrome. The first 31-year-old male patient had characteristic appearance of Noonan syndrome with severe infundibular pulmonic stenosis and patent foramen ovale. The second 28-year-old male patient had valvular and subvalvular pulmonary stenosis with typical Noonan's face and stature. Pulmonary valvotomy and hypertrophied muscle bundles in the right ventricular cavity were resected in both cases. Patent foramen ovale was closed directly in the first case. Postoperative follow-up examinations revealed no symptoms and successful outcome.

Early Results of Stent Implantation in Branch Pulmonary Artery Stenosis after Tetralogy of Fallot Repair

PURPOSE: Pulmonary artery stenosis is mainly complicated by aortopulmonary shunt or total correction of tetralogy of Fallot(TOF). Results of surgical angioplasty are poor and the success rate of balloon angioplasty is 53-72%. Endovascular stents have been applied to these lesions. The purpose of this study is to evaluate the early results of stent implantation in postoperative pulmonary artery stenosis in TOF. METHODS: Nineteen children with postoperative pulmonary artery stenosis were selected and balloon-expandable stent implantation was attempted between May 1994 and Feb 2000. For assessment of the results of stent implantation, the intraluminal diameter and the pressure gradient across the narrowest point were measured, as were the ratio of a right ventricle and aorta pressure, and a radionuclide lung perfusion scan was done before and after the procedure. RESULTS: Nineteen stents were implanted in 19 children, aged 2.3 years to 25,5 years. The increase of vessel diarneter ranged from 5.0+/-1.4mm to 10.7+/-2.1mm, pulmonary perfusion ratio from 16.7+/-5.3% to 29.2+/-10.0% and pressure gradients from 28.0+/-18.0 to 11.0+/-11.0mmHg with stent implantation. The systolic pressure ratio of the right ventricle and aorta decreased from 0.55+/-0.16 to 0.45+/-0.14. All above changes were statistically significant. Implantation failure occurred in 1 case and complications arose in 4 cases. CONCLUSIONS: The success rate of stent implantation is 94.296. Stent implantation is an effective and safe treatment method for postoperative pulmonary artery stenosis of TOF in short-term follow-up.

A Case of Takayasu's Arteritis Presented by Stenosis of Main Pulmonary Artery and Obstruction of the Right Coronary Artery / 대한내과학회지

Takayasu's arteritis is generally recognized as a chronic, idiopathic, inflammatory disease, which affects the aorta and the proximal portions of its major branches. We experienced a patient with Takayasu's arteritis who was presented with main pulmonary artery stenosis and right coronary ostial occlusion without involvement of aorta nor its major branches. She was managed with pulmonary arterioplasty and coronary artery bypass graft. This case emphasize that the disease cannot affect the aorta.