Circumflex Coronary Artery Fistulae Draining into Right Atrium: A Case Report

Coronary artery fistula (CAF) is an anomaly making a coronary artery communicate with a heart cavity or a great vessel, thus bypassing the myocardial capillary bed. CAF is frequently diagnosed as an incidental finding. Herein, we present the case of a 4-year-old boy. He was referred for a pediatric cardiology assessment due to a continuous murmur at the middle sternal border. Echocardiogram showed dilated left coronary artery and an abnormal diastolic flow in the right atrium. The right-sided chambers were slightly enlarged, but ventricular contractility was normal. CAF was suspected. Angiography and CT confirmed the diagnosis of coronary fistula from the circumflex coronary artery to the right atrium. Successful transcatheter closure with an Amplatzer PiccoloOccluder was performed with complete occlusion.

Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases

AIM To compare the behavior of pulmonary hypertension(PHT) associated with coronary artery fistulas(CAFs) between the Asian and Caucasian subjects.METHODS CAFs may be complicated with PHT secondary to leftto-right shunt. Literature review limited to the English language. A total of 211 reviewed patients were collected. Of those, 111 were of Asian and 100 were of Caucasian ethnic origin. The mean age of the Asian and the Caucasian groups of patients were 48.9(range 19-83) and 49.9 years(range 16-85), respectively. In both groups, right heart catheterization was the most commonly(95%) used method for determining pulmonary artery pressure. RESULTS From all of the reviewed subjects, PHT was found in 49 patients(23%), of which 15 were Asian and 34 were Caucasian. In 75% of PHT subjects, mild to moderate PHT was reported and 76% of the fistulas had a vascular mode of termination. Treatment was surgical in 61%, followed by percutaneous therapeutic embolization(27%) and finally conservative medical management in 12% of PHT subjects. PHT was associated with a slight female gender predominance. The majority demonstrated mild to moderate PHT. PHT was reported more frequent in the Caucasian compared with the Asian ethnicity group. The majority of fistulas in patients with PHT had a vascular mode of termination. The results of this review are intended to be indicative and require cautious interpretation.CONCLUSION The likelihood for a CAF patient to develop PHT is presented when possessing the following features, with a Caucasian female having a fistula with a vascular mode of termination.

Giant Aneurysm of a Coronary-Pulmonary Artery Fistula:A Rare Cause of a Diastolic Murmur

A coronary-pulmonary artery fistula with giant aneurysmal dilatation is an extremely rare clinical constellation.The natural course of this disease and the incidence of complications are unknown.Hence,optimal treatment,particularly in asymptomatic patients,is still a matter of debate.Here we report a case of a 71-year-old asymptomatic woman with a diastolic murmur.Comprehensive cardiovascular assessments including cardiac computed tomography and invasive coronary angiography revealed a coronary-pulmonary artery fi stula with giant aneurysmal dilatation.The patient was managed conservatively and has now been followed up for 5 years without any events.

Coil embolization of bronchial artery aneurysm with high flow bronchial artery-pulmonary artery fistula

We report a case of an asymptomatic 36-year-old man with a bronchial artery aneurysm in the right hilum. Selective angiography revealed a 25mmsaccular aneurysm and an efferent artery of the aneurysm forming a high flow bronchial artery-pulmonary artery fistula. Because of dilatation and tortuosity of the bronchial artery, the microcatheter could reach the efferent artery but not the fistula. Therefore, we embolized the fistula by sending microcoils through the bloodstream from the efferent artery to the fistula (the “flow-dependent” coil embolization technique), and further embolized the aneurysm by coil isolation and packing technique.

Device closure of fistula from left lower pulmonary artery to left atrium using a vascular plug:A case report

BACKGROUND Pulmonary artery-to-left atrial fistula is a variant of pulmonary arteriovenous fistula and is a developmental anomaly.Delayed presentation,cyanosis and effort intolerance are some of the important features.The diagnosis is confirmed by computed tomography or pulmonary artery angiography.Catheter-based closure is preferred to surgery.CASE SUMMARY Left pulmonary artery-to-left atrial fistula is rare.A 40-year-old male presented with effort intolerance,central cyanosis,and recurrent seizures.He had a large and highly tortuous left pulmonary artery-to-left atrial fistula associated with a large aneurysmal sac in the course.Catheter-based closure was performed using a vascular plug.CONCLUSION Left pulmonary artery-to-left atrial fistula is relatively uncommon compared to right pulmonary artery-to-left atrial fistula.Percutaneous closure by either a transeptal technique or guide wire insertion into the pulmonary vein through the pulmonary artery is preferred.The need for an arteriovenous loop depends on the tortuosity of the course of the fistula and the size of the device to be implanted because a larger device needs a larger sheath,necessitating firm guide wire support to facilitate negotiation of the stiff combination of the delivery sheath and dilator.

Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital heart disease(PAH-CHD)require further validation.This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up.Additionally,new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients.Methods:This retrospective study included 126 PAH-CHD patients.Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact tests.Indepen-dent risk factors were identified using ordered logistic regression,while Kaplan-Meier and Cox proportional hazards regression analyses evaluated their impact on all-cause mortality.A new stratification model for the PAH-CHD population was constructed based on these analyses.Results:Significant survival differences across stratified risk groups were observed(p<0.001),validating the effectiveness of the simplified risk stratification method in PAH-CHD patients.Prothrombin activity was a strong independent predictor of adverse outcomes of PAH-CHD patients(Hazard ratio 0.95,p<0.001,C-index 0.70).A model combining N-terminal pro-brain natriuretic peptide,prothrombin activity,albumin,and right atrial area achieved an area under the curve of 0.89 and a C-index of 0.85.Conclusions:The simplified risk stratification method is applicable to PAH-CHD patients.Prothrombin activity is a strong independent predictor of adverse outcomes.A comprehensive risk stratification approach,incorporating both established and novel biomarkers,enhances accessibility and offers predictive efficacy during follow-up for PAH-CHD patients,comparable to established models.

Current characteristics of congenital coronary artery fistulas in adults:A decade of global experience

AIM:To describe the characteristics of coronary artery fistulas(CAFs) in adults,including donor vessels and whether termination was cameral or vascular. METHODS:A PubMed search was performed for articles between 2000 and 2010 to describe the current characteristics of congenital CAFs in adults.A group of 304 adults was collected.Clinical data,presentations,diagnostic modalities,angiographic fistula findings and treatment strategies were gathered and analyzed.With regard to CAF origin,the subjects were tabulated into unilateral,bilateral or multilateral fistulas and compared.The group was stratified into two major subsets according to the mode of termination;coronary-cameral fistulas(CCFs) and coronary-vascular fistulas(CVFs) . A comparison was made between the two subsets. Fistula-related major complications[aneurysm formation,infective endocarditis(IE) ,myocardial infarction(MI) ,rupture,pericardial effusion(PE) and tamponade] were described.Coronary artery-ventricular multiple micro-fistulas and acquired CAFs were excluded as well as anomalous origin of the coronary arteries from the pulmonary artery(PA) . RESULTS:A total of 304 adult subjects(47%male) with congenital CAFs were included.The mean age was51.4 years(range,18-86 years) ,with 20%older than 65 years of age.Dyspnea(31%) ,chest pain(23%) and angina pectoris(21%) were the prevalent clinical presentations.Continuous cardiac murmur was heard in 82%of the subjects.Of the applied diagnostic modalities,chest X-ray showed an abnormal shadow in 4%of the subjects.The cornerstone in establishing the diagnosis was echocardiography(68%) ,and conventional contrast coronary angiography(97%) .However,multislice detector computed tomography was performed in 16%.The unilateral fistula originated from the left in 69%and from the right coronary artery in 31%of the subjects.Most patients(80%) had unilateral fistulas,18%presented with bilateral fistulas and 2%with multilateral fistulas.Termination into the PA was reported in unilateral(44%) ,bilateral(73%) and multilateral(75%) fistulas.Fistulas with multiple origins(bilateral and multilateral) terminated more frequently into the PA(29%) than into other sites(10.6%)(P=0.000) . Aneurysmal formation was found in 14%of all subjects.Spontaneous rupture,PE and tamponade were reported in 2%of all subjects.In CCFs,the mean age was 46.2 years whereas in CVFs mean age was 55.6 years(P=0.003) .IE(4%) was exclusively associated with CCFs,while MI(2%) was only found in subjects with CVFs.Surgical ligation was frequently chosen for unilateral(57%) ,bilateral(51%) and multilateral fistulas(66%) ,but percutaneous therapeutic embolization(PTE) was increasingly reported(23%,17%and 17%,respectively) . CONCLUSION:Congenital CAFs are currently detected in elderly patients.Bilateral fistulas are more frequently reported and PTE is more frequently applied as a therapeutic strategy in adults.

Congenital solitary coronary artery fistulas characterized by their drainage sites

Last centuries have witnessed tremendous sophistication and progress in the detection,diagnosis and treatment of coronary artery fistulas(CAFs).In many countries,CAFs were reported to be visualized and treated using several imaging techniques and different management strategies.Reports from nearly all continents of the globe have contributed to the description of CAFs,not only in Asia and Europe but also throughout North and Latin America.However,these reports have to be cautiously analyzed as many of them were published as a case report and careful interpretation is warranted due to possible publication bias.A literature search was performed using PubMed search interface to select papers dealing with congenital CAFs in adult population between 2000-2009.A total of 233 subjects were collected,and analysed according to their drainage site and treatment modality.They were divided into two subgroups:percutaneous transluminal embolization group(PTE group,n=122)and surgical ligation group(SL group,n=111).In the SL group,atherosclerotic coronary artery disease(19%)and associated congenital lesions(23%)were more prevalent compared with the PTE group(9%and 8%),respectively.Infective endocarditis was more frequently seen in the SL group besides syncope,congestive heart failure and hemopericardium.In both groups multimodality diagnostic workup composed of several non-invasive and invasive imaging techniques for fistula visualization were performed and drainage sites into the different cardiac chambers and intrathoracic great vessels were similarly distributed in the two groups.

Tuberculous esophagomediastinal fistula with concomitant mediastinal bronchial artery aneurysm-acute upper gastrointestinal bleeding: A case report

BACKGROUND Esophagomediastinal fistula is a very rare complication of tuberculosis in otherwise healthy adults, and mediastinal bronchial artery aneurysm is even rarer. In this case report, we describe a rare case of tuberculosis complication that presented with acute upper gastrointestinal(GI) bleeding. It also highlights the benefits of chest computed tomography(CT) as an excellent adjunct diagnostic tool to endoscopy and bronchoscopy and the role of trans-arterial embolization as a minimal invasive therapy alternative to surgery.CASE SUMMARY A 19-year-old medically free male patient presented with acute multiple episodes of hematemesis for 1 d. Upper GI endoscopy, bronchoscopy, and chest CT with IV contrast confirmed esophagomediastinal fistula with mediastinal bronchial artery aneurysm. After resuscitating patient with IV fluid and blood product transfusion, trans catheter embolization was performed for mediastinal bronchial artery aneurysm.CONCLUSION We successfully treated a patient with acute upper GI bleeding due to tuberculous esophagomediastinal fistula and mediastinal bronchial artery aneurysm using transcatheter coil embolization.

Incidental congenital coronary artery vascular fistulas in adults:Evaluation with adenosine-13N-ammonia PET-CT

AIM To assess the functionality of congenital coronary artery fistulas(CAFs) using adenosine stress ^(13)N-ammonia positron emission tomography computed tomography(PET-CT).METHODS Congenital CAFs were incidentally detected during coronary angiography(CAG) procedures in 11 adult patients(six males and five females) with a mean age of 64.3 years(range 41-81). Patients were collected from three institutes in the Netherlands. The characteristics of the fistulas(origin, pathway and termination), multiplicity of the origins and pathways of the fistulous vessels were assessed by CAG. Five patients underwent adenosine pharmacologic stress ^(13)N-ammonia PET-CT to assess myocardial perfusion and the functional behavior of the fistula. RESULTS Eleven patients with 12 CAFs, 10 unilateral and one bilateral, originating from the left anterior descending coronary artery(n = 8), right coronary artery(n = 2) and circumflex(n = 2). All fistulas were of the vascular type, terminating into either the pulmonary artery(n = 11) or coronary sinus(n = 1). The CAG delineated the characteristics of the fistula(origin, pathway and termination). Multiplicity of the origins and pathways of the fistulous vessels were common in most fistulas(8/12, 67% and 9/12, 75%, respectively). Multiplicity was common among the different fistula components(23/36, 64%). Adenosine pharmacologic stress ^(13)N-ammonia PET-CT revealed normal myocardial perfusion and ejection fraction in all but one patient, who showed a reduced ejection fraction.CONCLUSION PET-CT may be helpful for assessing the functional status of congenital CAFs in selected patients regarding clinical decision-making. Studies with a larger patient series are warranted.

Acute upper gastrointestinal bleeding caused by esophageal right bronchial artery fistula:A case report

BACKGROUND Fistula between the esophagus and bronchial artery is an extremely rare and potentially life-threatening cause of acute upper gastrointestinal bleeding.Here,we report a case of fistula formation between the esophagus and a nonaneurysmal right bronchial artery(RBA).CASE SUMMARY An 80-year-old woman with previous left pneumonectomy and recent placement of an uncovered self-expandable metallic stent for esophageal adenocarcinoma was admitted due to hematemesis.Emergent computed tomography showed indirect signs of fistulization between the esophagus and a nonaneurysmal RBA,in the absence of active bleeding.Endoscopy revealed the esophageal stent correctly placed and a moderate amount of red blood within the stomach,in the absence of active bleeding or tumor ingrowth/overgrowth.After prompt multidisciplinary evaluation,a step-up approach was planned.The bleeding was successfully controlled by esophageal restenting followed by RBA embolization.No signs of rebleeding were observed and the patient was discharged home with stable hemoglobin level on postoperative day 7.CONCLUSION This was a previously unreported case of an esophageal RBA fistula successfully managed by esophageal restenting followed by RBA embolization.

Surgical Correction of Coronary Artery Ectasia Combining Congenital Coronary Artery Fistula

Background:Coronary artery ectasia(CAE)complicated with concomitant congenital coronary artery fistula(CCAF)is rare.This study characterizes the clinical characteristics of CAE combining CCAF,and reports a single-institution experience with surgical correction of CAE combining CCAF.Methods:A total of 24 symptomatic patients(8 males,median 52.5 years old)who underwent surgical correction of CAE combining CCAF in this center were reviewed.Based on the size of ectatic segment,the CAE were classified as a giant CAE(>20 mm,n=14)and a non-giant CAE(≤20 mm,n=10).Individualized surgical approaches were chosen.The patients were followed up for a median of 3.8 years.Results:The overwhelming majority of CAEs were solitary,and only 4.2%of CAEs were associated with multiple lesions.CAEs were predominantly located in the right coronary artery with predilection to women more than to men(2:1).95.8%of patients with the CCAF had single fistula defect.The right atrium was the most frequent drainage site(33.3%)followed by the left ventricle(25.0%).Surgical mortality was 4.2%.All 22 follow-up patients survived with recovery from symptoms and New York Heart Association(NYHA)functional class I-II.In 10 patients with non-giant CAEs undergoing closure of fistula alone,favorable in-hospital outcomes were recorded,but residual fistula(one patient)and acute inferior wall myocardial infarction related to intracoronary thrombosis(one patient)were observed at follow-up.In 11 patients with giant CAEs undergoing aneurysm resection plus distal bypass grafting at the time of closure of fistula,favorable in-hospital outcomes and encouraging midterm results were recorded.Additionally,in 3 patients with giant CAEs undergoing closure of fistula plus aneurysmal plication,adverse events occurred,including surgical death related to rupture of the ectatic segment(one patient),perioperative myocardial infarction caused by acute thromboembolism(one patient),nonfatal inferior wall myocardial infarction related to intracoronary thrombosis(one patient)at follow-up.Conclusion:Individualized surgical approaches based on the size and the location of ectatic coronary artery as well as fistula should be offered to symptomatic patients with CAE combining CCAF.

Congenital Coronary Artery Fistula in Children:A Review of 28 Cases with Clinical and Imaging Outcomes

Background:Congenital coronary artery fistula(CCAF)is a rare anomaly.Treatment strategies tend to close the defect with a symptomatic and significant shunt,primarily based on expert consensus and case series.Results for long-term follow-up in children are limited Methods:We conducted a retrospective study to assess clinical and imaging outcomes of children with CCAF at Siriraj Hospital,Thailand during 2000–2020.Patients with single ventricle were excluded.Treatment strategies[surgical closure(SC),and percutaneous closure(PC)]were classified and the clinical outcomes at the follow-up in 2021,including coronary thrombosis,myocardial ischemia,and the results of cardiovascular imaging were reviewed.Results:Twenty-eight children with CCAF were included in the study.The median age at diagnosis was 2.5 years(2 days–18 years).Presenting symptoms were audible murmur(82%)and heart failure(35%).Most of fistulae arose from the right coronary artery(12/28)and exited at the right atrium(11/28).In recent visits(0.5–14 years follow-up),six patients with asymptomatic small CCAF were managed by watchful follow-up without complications.PC was primarily treated in 11 children:7 underwent successful procedures;1 had a residual shunt and required re-intervention;1 had ischemic symptoms immediately after the procedure with left coronary occlusion that required device removal plus SC and 2 were technically unable to place the device,requiring SC.Four patients were waiting for interventions(1 PC and 3 SC).Cardiovascular imaging surveillance that followed closure demonstrated asymptomatic thrombus formation in three patients(1 PC and 2 SC).No mortality presented.Conclusion:CCAF with significant shunt is indicated to close either SC or PC.Ischemic events are rare but have been reported after closure.In addition,thrombus formation should be watched for post-intervention.Surveillance with cardiovascular imaging is recommended after defect closure(ideally 1–5 years post closure),or at interval follow-ups in patients with symptoms to evaluate possible recanalization,thrombus,or ischemia.Life-long clinical and echocardiographic follow-up is warranted.Watchful follow-up is acceptable for hemodynamically insignificant fistula without complication in the series.

Bilateral coronary-pulmonary artery fistula with paroxysmal atrial arrhythmia:a case report

Coronary-pulmonary artery fistula(CPAF)is a relatively rare type of congenital coronary artery variation,which is mostly asymptomatic and is discovered incidentally by coronary artery CT imaging or angiography.However,CPAF can cause a phenomenon of inter-artery steal and hemodynamic disorders,leading to myocardial ischemia,arrhythmia,heart failure,syncope and even cardiac sudden death in severe cases.Here we reported a case of a 53-year-old female who complained of intermittent palpitations and had a history of paroxysmal atrial fibrillation.The coronary angiography revealed bilateral coronary artery fistulae to pulmonary artery.In this case,the multiple fistulas were very rare and significant.Multidisciplinary treatment should be considered,including cardiologists,surgeons,anesthesiologists and nurses,etc.Regular follow-up is essential for long-term disease management after diagnosis or surgery.

Transgelin as a potential target in the reversibility of pulmonary arterial hypertension second to congenital heart disease

Background The reversibility of pulmonary arterial hypertension(PAH)in congenital heart disease(CHD)is of great importance for the operability of CHD.Proteomics analysis found that transgelin was significantly upregulated in the lung tissue of CHD-PAH patients,especially in the irreversible group.However,how exactly it participated in CHD-PAH development is unknown.

Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction:A Rare Case Report

Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.

Bosentan Is Associated with a Reduction in Right Ventricular Systolic Pressure N-Terminal Pro-Hormone B-Type Natriuretic Peptide Levels in Young Patients with Pulmonary Hypertension

Pulmonary hypertension is a rare and potentially fatal disease in children if left untreated. Emerging therapies, including Bosentan, a dual endothelin receptor antagonist, have shown significant benefits in the adult pulmonary hypertension population;however, few studies have assessed the efficacy and safety of endothelin receptor antagonists in infants and young children. Our study was a single-center retrospective analysis of patients less than two years of age with a confirmed diagnosis of pulmonary hypertension and initiated on Bosentan therapy between 2017 and 2020. Twelve cases met eligibility criteria. Demographic data, laboratory data, echocardiographic, and cardiac catheterization data were analyzed. With treatment, there was a statistically significant decrease in mean right ventricular systolic pressure estimated by the tricuspid regurgitation jet (79 ± 23 mmHg reduced to 52 ± 25 mmHg;p < 0.001) N-terminal pro-hormone B-type natriuretic peptide levels (21,071 reduced to 2,037;p < 0.001). Additionally, improvement and eventual normalization of right ventricular function and septal geometry was seen within the first four months of therapy. Patients who underwent cardiac catheterization after therapy initiation (n = 4) demonstrated hemodynamic improvements;however, only the decrease in diastolic pulmonary artery pressure was statistically significant (p = 0.018). No significant differences in hemoglobin, platelet count, or liver function tests were observed between groups. In conclusion, these data suggest that Bosentan may be an effective and relatively safe treatment option for children less than two years of age with pulmonary hypertension. Further long-term randomized control studies are necessary to validate the potential clinical benefit of utilizing this drug therapy in young children.

Intralobar Pulmonary Sequestration with Rete Mirabile as Part of Its Systemic Artery

Intralobar sequestration (ILS) is a complex lung lesion where part of a lower lobe lacks communication with the tracheobronchial tree and receives an aberrant arterial blood supply from the systemic circulation. That systemic artery is usually large, originating from the thoracic aorta or the abdominal aorta and its upper branches. We describe a case of ILS where a large systemic artery coming up from below the diaphragm is formed by the convergence of many very small serpiginous arteries coming up from the area of the celiac trunk and common hepatic artery: a kind of “rete mirabile”. This peculiar morphology was not described before.

Congenital coronary artery flstnlae in adults: diagnosis and surgical treatment

Objective To summarize the clinical characteristics, diagnosis and surgical treatment results of congenital coronary artery fistulas (CAF) in adults. Methods Fourteen patients (8 men,6 women) ,aged from 18 to 60 years with a mean of 32 ±12 years, underwent surgical correction of CAF between March 1985 and April 2002. Eleven of the 14 patients (78. 57% ) were symptomatic. The diagnosis of CAF was made by echocardiography or angiocardioraphy preoperatiely. The fistulae originated from the right, left and double coronary arteries in 10 (71%),3(21%) and 1(7%) patient (s),respectively. The fistulae drained into the right ventricle (8 patients), left ventricle (4),right atrium (1) and pulmonary artery (1), respectively. The diameter of fistulae ranged from 0.30 tol.80 cm with a mean of (1.16±0.49)cm.There were 6 CAF patients associated with coronary artery aneurysms and 4 CAF patients with other coexisting cardiac defects. The distal fistulae were closed in 10 patients with cardiopulmonary bypass (CPB)

Treatment of patients with bosentan in postoperation of congenital heart disease with pulmonary arterial hypertension:a double-blind,randomized controlled trial

Objective Endothelin is a key role in the pathogenic of pulmonary arterial hypertension. High concentrations of endothelin 1 have been recorded in plasma and lungs of patients with pulmonary artery hypertension associated with congenital heart disease,and the concentrations of endothelin-1 was correlated with severity degree