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European Journal of Cardio-thoracic... Feb 2022Common Arterial Trunk (CAT) continues to have a very poor prognosis globally. To address that, we have developed a novel technique targeting key concepts for the...
OBJECTIVES
Common Arterial Trunk (CAT) continues to have a very poor prognosis globally. To address that, we have developed a novel technique targeting key concepts for the correction of all components of the anomaly, using autologous arterial tissue. This aims to enhance results, availability worldwide, and importantly to avoid the need for repeated reoperations.
METHODS
From January 2019 to 4 January 2021, all patients with isolated CAT had repair of the defect using autologous arterial trunk tissue with direct right ventricle (RV) to pulmonary artery (PA) connection. Clinical outcomes, follow-up which included multi-slice computed tomography 3D segmentation and 4D cardiovascular magnetic resonance flow, are presented.
RESULTS
Twenty patients were included in the study (median age 4.5 months). There were 2 hospital deaths due to systemic infection and pulmonary hypertensive crisis, respectively. Following discharge all patients remained asymptomatic with no signs of heart failure and improved pattern of growth (median follow-up: 8 months). Early postoperative 3D segmentation showed a conical shaped neo-right ventricular outflow chamber connecting the body of the RV to the main PA through a valveless ostium, and normal crossing of PA and neo-aorta. 4D cardiovascular magnetic resonance pattern of flow showed normal rapid laminar flow through the atrioventricular valves followed by a vortex towards the outflow tracts. There was laminar flow through the neo-aorta and neo-PA with velocity not exceeding 2.5 m/s. The PA regurgitant fraction was 25 ± 5% and was limited to early diastole.
CONCLUSIONS
The initial results of utilizing the key concepts, using autologous arterial tissue for the repair of CAT, are encouraging, both clinically and by multimodality imaging.
Topics: Heart Defects, Congenital; Heart Ventricles; Humans; Pulmonary Artery; Reoperation; Truncus Arteriosus, Persistent
PubMed: 34347066
DOI: 10.1093/ejcts/ezab336 -
Biomedicine & Pharmacotherapy =... Sep 2020Pulmonary arterial hypertension (PAH) is a progressive disease with limited effective therapies that can be lethal in the terminal stage. Since the universal use of... (Review)
Review
Pulmonary arterial hypertension (PAH) is a progressive disease with limited effective therapies that can be lethal in the terminal stage. Since the universal use of traditional PAH-specific drugs affecting the nitric oxide pathway, endothelin pathway and prostacyclin pathway, major advances to treat this devastating condition have been made. For example, it has been recently demonstrated that PAH is associated with various dysfunctional genes and molecular signaling pathways, abnormal metabolism and endocrine systems, inflammation and immune dysfunction. Numerous descriptions of novel mechanisms in PAH have been presented, and some new drugs targeted to ease, reverse or cure PAH have been developed at the laboratory-level and/or tested in clinical trials. There are also some investigated drugs that have shown promising efficacy and safety in animal models but have failed in human beings. Inspiringly, immunotherapies also seem to be valuable therapies for PAH patients. In this review, we update the advances in investigational pharmacotherapy and immunotherapy of PAH. Hopefully this work can help promote more non-invasive treatment for PAH patients.
Topics: Animals; Antihypertensive Agents; Arterial Pressure; Humans; Immunosuppressive Agents; Immunotherapy; Molecular Targeted Therapy; Pulmonary Arterial Hypertension; Pulmonary Artery; Signal Transduction; Vaccines
PubMed: 32559622
DOI: 10.1016/j.biopha.2020.110355 -
Cardiology in the Young Jul 2023The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the...
INTRODUCTION
The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature.
METHODS
The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed.
RESULTS
Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later.
CONCLUSIONS
Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.
Topics: Pregnancy; Male; Humans; Female; Infant; Univentricular Heart; Heart Defects, Congenital; Heart Bypass, Right; Pulmonary Artery; Tricuspid Atresia; Truncus Arteriosus, Persistent; Fontan Procedure; Retrospective Studies; Heart Ventricles; Treatment Outcome
PubMed: 35815558
DOI: 10.1017/S1047951122002128 -
Surgery Today Apr 2022The bronchopulmonary vascular bifurcation patterns in the upper lobe of the left lung are diverse. Therefore, it is important for general thoracic surgeons to understand...
PURPOSES
The bronchopulmonary vascular bifurcation patterns in the upper lobe of the left lung are diverse. Therefore, it is important for general thoracic surgeons to understand the detailed anatomy of the pulmonary segments when performing thoracoscopic anatomical pulmonary resection. This study aimed to analyze the bronchovascular patterns of the left upper lobe and summarize the anatomical information associated with pulmonary anatomical pulmonary resection.
METHODS
We reviewed the anatomical patterns of pulmonary vessels and the left lung bronchus of 539 patients using computed tomography imaging data including those obtained using three-dimensional computed tomography. We herein report the anatomic structure in the left upper lobe.
RESULTS
Regarding the superior division bronchi, a pattern of trifurcation into B, B, lingular division bronchus was observed in nine patients (1.7%). A pattern of proximal bifurcation of B was found in eight patients (1.5%). Regarding the lingular veins (LV), patterns of LV drainage into the left lower pulmonary vein were observed in 22 patients (4.1%). Regarding the pulmonary artery, mediastinal lingular arteries (MLA) were found in 161 patients (29.9%).
CONCLUSION
The bifurcation patterns of the bronchovascular region in the upper lobe of the left lung were clarified. These results should be carefully noted when performing anatomical pulmonary resection.
Topics: Bronchi; Humans; Lung; Mediastinum; Pulmonary Artery; Pulmonary Veins
PubMed: 35179645
DOI: 10.1007/s00595-022-02471-1 -
The Heart Surgery Forum Feb 2021Percutaneous balloon pulmonary valvuloplasty has proven to be a standard of care for neonates with severe pulmonary valve disease. However, the peripheral vessel injury,...
BACKGROUND
Percutaneous balloon pulmonary valvuloplasty has proven to be a standard of care for neonates with severe pulmonary valve disease. However, the peripheral vessel injury, tricuspid chordae tendineae rupture, and cardiac tamponade could occur. Recently, we performed balloon valvuloplasty through pulmonary artery trunk. To date, the obtained outcome was promising.
METHODS
Between January 2018 and December 2018, three neonates with critical pulmonary stenosis and two with membranous pulmonary atresia with intact ventricular septum were enrolled in our center. Balloon valvuloplasty through pulmonary artery trunk was performed in all patients. A 2-cm parasternal incision was made in the left third intercostal space. A guidewire was used to advance or perforate the pulmonary valve from the pulmonary artery trunk into the right ventricle, followed by balloon dilation of the valve.
RESULTS
The procedure was successful in all patients. The oxygen saturation increased immediately after the balloon dilation, while the right ventricular systolic pressure and the gradient across the pulmonary valve decreased. No severe complications occurred.
CONCLUSIONS
Balloon valvuloplasty through the pulmonary artery trunk is a safe and feasible alternative procedure. Thus, it could serve as a supplementary choice for treating severe pulmonary valve disease.
Topics: Abnormalities, Multiple; Balloon Valvuloplasty; Cardiac Surgical Procedures; Echocardiography; Female; Follow-Up Studies; Humans; Infant, Newborn; Male; Pulmonary Artery; Pulmonary Atresia; Pulmonary Valve; Pulmonary Valve Stenosis; Retrospective Studies; Severity of Illness Index; Treatment Outcome
PubMed: 33635246
DOI: 10.1532/hsf.3423 -
Journal of Cardiac Surgery Mar 2022We report a case of a 27-day-old girl with an unusual association of crossed pulmonary arteries with common arterial trunk showing pulmonary dominance with an...
We report a case of a 27-day-old girl with an unusual association of crossed pulmonary arteries with common arterial trunk showing pulmonary dominance with an interruption of aortic arch between the origin of the left common carotid artery and left subclavian artery. This case highlights the potential airway-related implications of this variant pulmonary arterial anatomy in the setting of the common arterial trunk and the role of computed tomography angiography in providing not only accurate and three-dimensional visualization of complex cardiovascular anatomy, but also a comprehensive evaluation of associated airway abnormalities with great precision.
Topics: Angiography; Aorta, Thoracic; Female; Humans; Pulmonary Artery; Subclavian Artery; Truncus Arteriosus, Persistent
PubMed: 34994486
DOI: 10.1111/jocs.16213 -
European Journal of Cardio-thoracic... May 2022This study aimed to investigate whether computed tomographic indices support surgical decision-making in patients with pulmonary artery sling, congenital tracheal...
OBJECTIVES
This study aimed to investigate whether computed tomographic indices support surgical decision-making in patients with pulmonary artery sling, congenital tracheal stenosis and right lung underdevelopment.
METHODS
A total of 38 patients with pulmonary artery sling and congenital tracheal stenosis underwent tracheoplasty. Patients were divided into 3 groups based on anatomical categorization: group normal lung (NL; n = 21), group H (right lung hypoplasia, n = 12) and group A (right lung agenesis or aplasia, n = 5). Using preoperative computed tomographic images, the severity of the tracheal bending due to the right posterior deviation of the aortic arch and the relative length of the left pulmonary artery for reimplantation was evaluated.
RESULTS
Slide tracheoplasty posterior to the aortic arch with pulmonary artery reimplantation was performed in 32 patients (21, 10 and 1 in groups NL, H and A, respectively). Slide tracheoplasty anterior to the aortic arch was performed in 4 patients (2 patients each in groups H and A). Among the 6 patients with severe tracheal bending who underwent slide tracheoplasty posterior to the aortic arch, 5 required aortopexy for tracheomalacia. The overall mortality rate was 3% (group NL, n = 1). The relative length of the left pulmonary artery in group H (0.85) was significantly shorter than that in group NL (1.36, P < 0.0001).
CONCLUSIONS
In patients with right lung underdevelopment, preoperative computed tomography elucidated the tracheal bending due to right posterior deviation of the aortic arch, which compromised tracheoplasty and shortness of the left pulmonary artery for pulmonary artery sling repair.
Topics: Constriction, Pathologic; Heart Defects, Congenital; Humans; Infant; Lung; Pulmonary Artery; Retrospective Studies; Trachea; Tracheal Stenosis; Treatment Outcome; Vascular Malformations
PubMed: 35134910
DOI: 10.1093/ejcts/ezac070 -
Advances in Experimental Medicine and... 2024Truncus arteriosus (TA, also known as common arterial trunk) consists of only one great artery ("the truncus") with a semilunar valve (truncus valve) arising from the...
Truncus arteriosus (TA, also known as common arterial trunk) consists of only one great artery ("the truncus") with a semilunar valve (truncus valve) arising from the heart and an additional ventricular septal defect and (Fig. 50.1). This great artery is positioned above the ventricular septal defect and gives rise to the coronary arteries, the pulmonary arteries, and the aortic arch. Historically, TA has been classified by Collet and Edwards in three types, where in type I there was a common pulmonary artery truncus, in type II the left and right PA arise separately but close to each other, in type III both PA arise independently; in addition, there was a type IV that was later characterized as pulmonary atresia with VSD and major aortopulmonary collateral arteries arising from the descending aorta.
Topics: Humans; Pulmonary Artery; Pulmonary Atresia; Truncus Arteriosus; Truncus Arteriosus, Persistent
PubMed: 38884752
DOI: 10.1007/978-3-031-44087-8_50 -
Annals of Saudi Medicine 2019Pulmonary hypertension (PH) is an idiopathic or secondary disorder associated with many systemic illnesses. Long-term survival in PH depends on the severity and...
BACKGROUND
Pulmonary hypertension (PH) is an idiopathic or secondary disorder associated with many systemic illnesses. Long-term survival in PH depends on the severity and functional class. Several new drugs are now available to treat PH, but their impact on clinical outcome and survival are not well established.
OBJECTIVES
Evaluate severity parameters and the impact of current recommended therapy on survival in PH.
DESIGN
Cross-sectional.
SETTINGS
Tertiary care center.
PATIENTS AND METHODS
The study included adult patients who had undergone right heart catheterization since 2012 and were diagnosed with pulmonary hypertension. Survival was recorded after 6 years, at the end of the study. Nine severity variables for PH were assessed including right ventricular size by echocardiogram and pulmonary artery diameter (PA diameter) and the ratio of pulmonary artery diameter to ascending aorta diameter (PA/Ao ratio) by CT.
MAIN OUTCOME MEASURES
Evaluation of severity parameters.
SAMPLE SIZE
30 patients.
RESULTS
Twenty-five patients were positive for 8/9 severity parameters. Eight of 30 (26.6%) patients died. In nonsurvivors, right ventricular size was increased by 25% ( P=.427), pulmonary vascular resistance increased by 29.4% in nonsurvivors ( P=.302), the 6-minute walk distance decreased by 21% ( P=.875), median brain natriuretic peptide increased by 96% ( P=.890), median GGT and alkaline phosphatase were 3 times higher in nonsurvivors ( P=.893 and P=.047, respectively) and PA/Ao was nonsignificantly decreased in nonsurvivors ( P=.373), Survival was decreased by a median of 2.3 years in nonsurvivors.
CONCLUSION
Our study identified a subgroup of PH patients with NYHA functional class III and above with worsening severity indicators who were labeled as a high-risk group. These patients showed continuous deterioration in their clinical status despite escalation of therapy with current guidelines. We recommend these high-risk group patients be referred for early lung transplantation.
LIMITATIONS
Low sample size and only a single center. Needs confirmation with a larger multicenter trial.
CONFLICT OF INTEREST
None.
Topics: Adult; Echocardiography; Female; Humans; Hypertension, Pulmonary; Male; Pulmonary Artery; Severity of Illness Index; Survival Analysis; Treatment Outcome
PubMed: 31804134
DOI: 10.5144/0256-4947.2019.426 -
Journal of Cardiac Surgery Dec 2019A pulmonary artery sling is formed when the left pulmonary artery originates from the right pulmonary artery and encircles the distal trachea, coursing between the...
A pulmonary artery sling is formed when the left pulmonary artery originates from the right pulmonary artery and encircles the distal trachea, coursing between the trachea and esophagus to reach the hilum of the left lung. Pulmonary artery slings are often associated with distal tracheal narrowing, due to either intrinsic stenosis or secondary compression by the anomaly itself. We report a very rare case in which the left superior pulmonary artery originated from the right pulmonary artery and then acted as a sling, the left inferior pulmonary artery originated from the pulmonary trunk.
Topics: Humans; Infant; Male; Pulmonary Artery; Vascular Malformations
PubMed: 31557345
DOI: 10.1111/jocs.14268