Branch Pulmonary Artery Stenosis Research Articles

The effects of percutaneous branch pulmonary artery interventions in biventricular congenital heart disease: study protocol for a randomized controlled Dutch multicenter interventional trial

BackgroundBranch pulmonary artery (PA) stenosis is one of the most common indications for percutaneous interventions in patients with transposition of the great arteries (TGA), tetralogy of Fallot (ToF), and truncus arteriosus (TA). However, the effects of percutaneous branch PA interventions on exercise capacity remains largely unknown. In addition, there is no consensus about the optimal timing of the intervention for asymptomatic patients according to international guidelines. This trial aims to identify the effects of percutaneous interventions for branch PA stenosis on exercise capacity in patients with TGA, ToF, and TA. In addition, it aims to assess the effects on RV function and to define early markers for RV adaptation and RV dysfunction to improve timing of these interventions.MethodsThis is a randomized multicenter interventional trial. TGA, ToF, and TA patients ≥ 8 years with a class IIa indication for percutaneous branch PA intervention according to international guidelines are eligible to participate. Patients will be randomized into the intervention group or the control group (conservative management for 6 months). All patients will undergo transthoracic echocardiography, cardiac magnetic resonance (CMR) imaging, and cardiopulmonary exercise testing at baseline, 6 months, and 2–4 years follow-up. Quality of life (QoL) questionnaires will be obtained at baseline, 2 weeks post intervention or a similar range for the control group, and 6 months follow-up. The primary outcome is exercise capacity expressed as maximum oxygen uptake (peak VO2 as percentage of predicted). A total of 56 patients (intervention group n = 28, control group n = 28) is required to demonstrate a 14% increase in maximum oxygen uptake (peak VO2 as percentage of predicted) in the interventional group compared to the control group (power 80%, overall type 1 error controlled at 5%). Secondary outcomes include various parameters for RV systolic function, RV functionality, RV remodeling, procedural success, complications, lung perfusion, and QoL.DiscussionThis trial will investigate the effects of percutaneous branch PA interventions on exercise capacity in patients with TGA, ToF, and TA and will identify early markers for RV adaptation and RV dysfunction to improve timing of the interventions.Trial registrationClinicalTrials.gov NCT05809310. Registered on March 15, 2023.

Percutaneous dual intervention in a child with Alagille syndrome –A case report.

Alagille syndrome(ALGS) is a rare autosomal dominant (AD) genetic disorder .ALGS involves multisystem which includes liver ,heart, skeleton, Eyes, kidneys with facial abnormalities. ALGS may involve a particular system like facial dysmorphism, cardiac defect or vertebral defect. Some individual may not have liver anomalies or typical features. We report a case of 9 year old male child with large ostium secundum ASD(16 mm) and branch pulmonary artery stenosis(LPA)diagnosed incidentally at 8 year of age. The child had facial dysmorphism (broad forehead, frontal bossing , triangular facies, hypertelorism, prominent ear and pointed chin, straight nose with bulbous tip. The mental development of the child was borderline. He also had butterfly vertebrae in thoracic and lumber region. He was treated successfully with ASD device closure for ASD and balloon pulmonary valvuloplasty for LPA stensis. Both the procedure was performed simultaneously without any complications. There is no curative management for the disorder so symptomatic management is the only treatment option.

Novel mutation in ELN gene causes cardiac abnormalities and inguinal hernia: case report

BackgroundElastin-driven genetic diseases are a group of complex diseases driven by elastin protein insufficiency and dominant-negative production of aberrant protein, including supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Here, a Chinese boy with a novel nonsense mutation in the ELN gene is reported.Case presentationWe report a 1-year-old boy who presented with exercise intolerance, weight growth restriction with age, a 1-year history of heart murmur, and inguinal hernia. Gene sequencing revealed a novel nonsense mutation in the ELN gene (c.757 C > T (p.Gln253Ter), NM_000501.4). Due to severe branch pulmonary artery stenosis, the reconstruction of the branch pulmonary artery with autologous pericardium was performed. The inguinal hernia repair was performed 3 months postoperatively. After six months of outpatient follow-up, the child recovered well, gained weight with age, and had no special clinical symptoms.ConclusionWe identified a de novo nonsense mutation in the ELN gene leading to mild SVAS and severe branch pulmonary artery stenosis. A new phenotype of inguinal hernia was also needed to be considered for possible association with the ELN gene. Still, further confirmation will be necessary.

Results of Transcatheter Therapy with Balloon Versus Stent for Treating Isolated Branch Pulmonary Artery Stenosis in Children and Adolescents: A Midterm Study

Background: Branch pulmonary artery stenosis presents as either an isolated disease or in association with congenital heart diseases (in 2 - 3% of cases). Balloon angioplasty (BA) and stent implantation (SI) are two percutaneous methods that are commonly used to treat this condition. We compared the outcomes of these 2 methods in children and adolescents with isolated branch pulmonary artery stenosis. Methods: Pediatric patients with severe branch pulmonary artery stenosis who were referred to our center and underwent transcatheter insertion via either BA or SI between 2010 and 2019 were studied. Patients’ demographic data, including age, gender, and body weight, were recorded. Standard procedures for BA and SI were applied. Post-procedure angiography and echocardiographic parameters were determined and compared between the 2 groups. Patients who were followed up for at least 2 years were selected for this study, and comparative evaluations were performed during the follow-up. Results: Forty patients with a median age of 2.1 years and a median weight of 10 kg were enrolled in the study, of whom 25 and 15 underwent BA and SI, respectively. The trans-stenotic pulmonary artery pressure gradient significantly reduced immediately after both procedures, but there was no significant difference between the BA and SI groups in terms of pulmonary artery pressure gradient early after the procedures (P-value = 0.014). There was a significant decline in the peak right ventricular pressure after both procedures. Restenosis occurred more frequently in the BA group. In the BA group, patients under one year old and weight lower than 10 kg had a lower restenosis recurrence rate, evidenced by echocardiographic measurements, compared to those with a weight over 10 kg. Three patients (12%) in the BA group needed balloon reinsertion. Conclusions: Immediate therapeutic success was similar between BA and SI in patients with branch pulmonary artery stenosis. Re-stenosis occurred more often in the BA group; however, the chance of re-stenosis was lower among patients weighing less than 10 kg over a 2-year follow-up.

Stent Strut Dilation in Branch Pulmonary Artery Stenosis Following Stenting of Arterial Duct in Duct-dependent Pulmonary Circulation.

To assess the feasibility and outcome of stent strut dilation after arterial duct stenting with associated branch pulmonary artery (BPA) stenosis. Stenting of arterial duct in infants with duct-dependent pulmonary circulation is technically challenging. The presence of BPA stenosis is a relative contraindication for stent implantation. Infants with duct-dependent pulmonary circulation and associated BPA stenosis were assessed either by transthoracic echocardiogram alone or additional computerized tomography angiogram when required. Following ductal stenting, the stent struts of the stenosed BPA were crossed with an additional 0.014″ coronary guide wire and dilated using coronary balloons (2.0 or 2.5mm in diameter). Seventeen (12 male) patients were considered for the procedure. The median age and weight were 27days (range 2-94) and 2.6kg (range 2.2-5), respectively. Fourteen patients (82.4%) underwent stent strut dilation after arterial duct stenting. Struts to left pulmonary artery was opened in 9 (64.3%) and right pulmonary artery in 5 (35.7%). The mean systemic oxygen saturation increased from 66.23 ± 8.9% at baseline to 86 ± 2.2% immediately after the stent deployment and final saturations after stent strut dilation were 89.29 ± 4.3%. Angiographic pulmonary flow improved in all cases. Stent strut dilation could not be done in 3 patients due to unfavorable anatomy. One patient had acute stent thrombosis and died in the hospital. Two others died during follow-up, during an acute febrile illness and gastroenteritis. All survivors underwent cardiac surgery and were on regular follow-up. Strut dilation of BPA stenosis is feasible to augment pulmonary blood flow, following arterial duct stenting. This procedure may be useful in selected patients with BPA stenosis to have uniform growth of pulmonary arteries.

Modified Aortic Arch Reconstruction Combined With Pulmonary Artery Reconstruction in the Comprehensive Stage II Operation Following a Hybrid Stage I for Hypoplastic Left Heart Syndrome

The hybrid approach for hypoplastic left heart syndrome is a well-accepted alternative strategy to the conventional staged surgical approach. However, in the typical comprehensive stage II operation, bilateral branch pulmonary artery stenosis is a risk factor for postoperative catheter and/or surgical interventions. In addition to addressing the stenosis from the bands, the branch pulmonary arteries are at risk of compression from the dilated main pulmonary trunk and the resultant large neo-aorta. Here, we present a new aortic arch reconstruction technique combined with the pulmonary artery reconstruction in the comprehensive stage II operation to address these issues. In our experience of 11 patients who underwent hybrid strategy, Fontan completion was achieved in 8 cases (73%) during a median follow-up duration of 84 months (range; 45-161 months), and the 3-year, 5-year, and 10-year survival rates were 73% each. While 6 of 8 cases (75%) required catheter interventions to the branch pulmonary arteries, the Nakata Index was 204±11 before the stage II and 185±19 before Fontan completion, indicating that good pulmonary artery growth. Our modified comprehensive stage II technique optimizes the pulmonary arteries for the Fontan and beyond by addressing both anatomic stenoses and external compression for future growth.

Frequency of various cardiac complications in children with repaired tetralogy of Fallot identified by computer tomography

BACKGROUND: Tetralogy of Fallot represents 710% of all cases of congenital heart disease, as it occurs in approximately 0.5 per 1,000 live births and is the second most common form of complex congenital heart disease. Advances in diagnosis, surgical techniques, and postoperative treatment have led to an increasing number of patients reaching adulthood, with a dramatic increase in the survival rate to almost 90% at 30 years, thereby creating a need for long-term monitoring of certain anatomic parameters to identify complications in a timely manner. This study aimed to investigate the frequency of computed tomography detected complications after radical correction of Tetralogy of Fallot in pediatric patients.
 AIM: to identify markers between the most frequency computed tomography detected complications after repair of Tetralogy of Fallot in pediatric patients.
 MATERIALS AND METHODS: A retrospective analysis was conducted on 613 patients with Tetralogy of Fallot from October 2011 to June 2020. The study included a total of 116 patients (69 men and 47 women) who experienced complications after a repair of Tetralogy of Fallot, as identified by computed tomography. At the time of repair of Tetralogy of Fallot, the patients average age ranged from 10 to 36 months (mean: 12 months), average body weight was 21 kg, average height was 105.4 cm, and average body surface area was 0.74 m2. The patients median age at the time of the computed tomography examination was 17.5 years (age range: 736 years).
 RESULTS: Among the 116 patients who exhibited complications after an repair of Tetralogy of Fallot, 49 had a pulmonary artery stenosis, 92 had a pulmonary artery branch stenosis (56 of them of the left main pulmonary artery branch, and 36 of them of the right main pulmonary artery branch), 8 had a right ventricular outflow tract stenosis, 32 had a ventricular septal defect, 1 had a shunt thrombosis, 12 had a postoperative deformation of the pulmonary artery, 10 exhibited a marked right ventricular dilatation, 2 had an right ventricular outflow tract aneurysm, and 6 suffered from conduit calcification and stenosis. Moreover, patients with left main pulmonary artery branch stenosis had a 6.5 times greater chance of developing an right main pulmonary artery branch stenosis in (p 0.001).
 CONCLUSION: The most frequently computed tomography detected complications after a repair of Tetralogy of Fallot were pulmonary artery stenosis and pulmonary artery branch stenosis. Patients with pulmonary artery stenosis and pulmonary artery branch stenosis exhibit no significant differences in terms of age, anthropometric parameters (height, weight, and body surface area), and gender distribution in the presence or absence of different stenosis types (pulmonary artery, right main pulmonary artery branch, or left main pulmonary artery branch). However, an right main pulmonary artery branch stenosis increases the chances of developing an left main pulmonary artery branch stenosis.

Characterization of an induced pluripotent stem cell line NCHi011-A from a 23-year-old female with Alagille Syndrome harboring a heterozygous JAG1 pathogenic variant

Alagille syndrome (ALGS) is a multisystem disease with high variability in clinical features. ALGS is predominantly caused by pathogenic variants in the Notch ligand JAG1. An iPSC line, NCHi011-A, was generated from a ALGS patient with complex cardiac phenotypes consisting of pulmonic valve and branch pulmonary artery stenosis. NCHi011-A is heterozygous for a single base duplication causing a frameshift in the JAG1 gene. This iPSC line demonstrates normal cellular morphology, expression of pluripotency markers, trilineage differentiation potential, and identity to the source patient. NCHi011-A provides a resource for modeling ALGS and investigating the role of Notch signaling in the disease.

Role of Cardiac CTA to Evaluate Branch Pulmonary Artery Stenosis and Ductal Insertion Pattern in Right-Sided Congenital Heart Defects.

There is limited experience in evaluating abnormal ductus arteriosus (DA) insertion pattern by contrast-enhanced cardiac computed tomography (cardiac CT) in patients with right-sided obstructive cardiac defects. Retrospective review of 38 infants with right-sided obstructive cardiac defects who underwent a preoperative cardiac CT between 2016 and 2021. We reviewed the types of cardiac lesions, patterns of ductal insertion, frequency of pulmonary artery (PA) stenosis requiring intervention, total dose length product (DLP), and effective radiation dose. Of 38 infants, 45% were female, the median gestational age and weight were 37 (range 34-40) weeks and 2.95 (range 2-4) kg. The most common pathologies were pulmonary atresia with ventricular septal defect (24%) and tetralogy of Fallot (24%). The abnormal ductal insertion patterns were DA inserting into the left PA in 39%, DA bifurcating into branch PA in 32%, and DA inserting into the right PA in 13%. Of the 38 infants, 76% developed branch PA stenosis requiring intervention. Among patients with abnormal DA insertion, 44% required branch PA arterioplasty during their index surgery compared to 17% without abnormal DA insertion. Regardless of the type of abnormal DA insertion, 67% developed bilateral branch PA stenosis over time. The mean DLP was 8mGy-cm and the mean calculated effective radiation dose was 0.312mSv. The utilization of contrast-enhanced cardiac CT in infants with right-sided obstructive heart defects can offer crucial insights into abnormal ductus arteriosus insertion patterns. This information is valuable for effective procedure planning and for monitoring the development of branch pulmonary artery stenosis.

Three-dimensional/four-dimensional spatiotemporal image correlation morphology of the ductus arteriosus in fetuses with pulmonary atresia undergoing neonatal ductal stenting.

The value of prenatal identification of morphology of ductus arteriosus in fetuses with congenital heart defects (CHD) with pulmonary atresia and duct-dependent pulmonary circulation (DDPC) in planning neonatal ductal stenting procedure is untested. The aim of the study is to analyze the utility of three-dimensional/four-dimensional (3D/4D) spatiotemporal image correlation (STIC) fetal echocardiography in delineating the morphology of ductus arteriosus in fetuses with DDPC undergoing neonatal ductal stenting. In this retrospective study (2017-22), prenatal imaging of pulmonary artery (PA) anatomy, aortic arch sidedness, and morphology of ductus arteriosus (ductal origin was classified as vertical/horizontal and ductal course as tortuous/straight) was done using 3D/4D STIC imaging and volume datasets. Prenatal findings were correlated with angiographic findings during stenting and the degree of agreement was calculated. We included 27 fetuses with a prenatal diagnosis of CHD with DDPC who underwent neonatal ductal stenting. The accuracy of prenatal assessment of PA anatomy, branch PA stenosis, and arch sidedness was 100%, 92.6%, and 88.9%, respectively. The accuracy of prenatal assessment of ductal origin and course, compared with angiography, was 85.2% and 88.9%, respectively. Prenatal imaging had a diagnostic accuracy of 100% for vertical straight and horizontal tortuous ducts, 84.6% for vertical tortuous, and 67% for horizontal straight ducts. Duct stenting was successful in 25 (92.6%) babies; two died after the procedure from stent occlusion. Fetal echocardiography using 3D/4D STIC imaging enables accurate delineation of the morphology of ductus arteriosus in fetuses with DDPC, thereby aiding parental counseling and planning neonatal ductal stenting.

Surgical and transcatheter pulmonary valve replacement in patients with repaired tetralogy of Fallot: cardiac magnetic resonance imaging characteristics and morphology of right ventricular outflow tract.

Pulmonary valve replacement is recommended in patients with repaired tetralogy of Fallot based on cardiac magnetic resonance imaging (MRI) criteria. This procedure is performed by surgical or transcatheter approaches. We aimed to investigate the differences in preprocedural MRI characteristics (volume, function, strain) and morphology of the right ventricular outflow tract and branch pulmonary arteries in patients for whom surgical or transcatheter pulmonary valve replacementwas planned. Cardiac MRI of 166 patients with tetralogy of Fallot were analyzed. Of these, 36 patients for whompulmonary valve replacement was plannedwere included. Magnetic resonance imaging characteristics, right ventricular outflow tract morphology, branch pulmonary artery flow distribution and diameter were compared between surgical and transcatheter groups. Spearman correlation and Kruskal-Wallis tests were performed. Circumferential and radial MRI strain for the right ventricle were lower in thesurgical group (P=0.045and P=0.046, respectively). The diameter of the left pulmonary artery was significantly lower (P=0.021) and branch pulmonary artery flow and diameter ratio were higher (P=0.044and P = 0.002, respectively) in the transcatheter group. There was a significant correlation between right ventricular outflow tract morphology and right ventricular end-diastolic volume indexand global circumferential and radial MRI strain (P=0.046, P=0.046and P= 0.049, respectively). Preprocedural MRI strain, right-to-left pulmonary artery flow, diameter ratio and morphological features of the right ventricular outflow tract were significantly different betweenthe two groups. A transcatheter approach may be recommended for patients with branch pulmonary artery stenosis, since both pulmonary valve replacement and branch pulmonary artery stenting can be performed in the same session.

Preoperative Computed Tomographic Angiography Can Predict Need for Branch Pulmonary Artery Intervention in Patients With Ductal-Dependent Pulmonary Blood Flow.

Neonates with ductal-dependent pulmonary blood flow (DD-PBF) are at risk for pulmonary artery (PA) stenosis. The objective of this study was to identify preoperative cardiovascular computed tomography angiography (CTA) measures that are associated with the need for branch PA intervention. We identified neonates with DD-PBF who underwent preoperative CTA at our center and were followed for 24 months. The primary outcome was requiring intervention for branch PA stenosis at the initial or subsequent procedure. Patients were divided into three groups: 1) No PA intervention, 2) Initial PA intervention, and 3) Remote PA intervention. Measurements of the branch PAs and patent ductus arteriosus (PDA) were made prospectively. Forty patients were included, 7 (18%) did not receive a PA intervention, 23 (58%) were in the initial PA intervention group, and 10 (25%) were in the remote PA intervention group. The distance from PA bifurcation to the largest diameter of the PA that receives the PDA showed a difference between the no-intervention group versus the initial and remote intervention groups (0.8 mm [IQR 0.7, 2.0], 8.2 mm [IQR 1.9, 13.7], 8.5 mm [IQR 6.5, 11.1], respectively, P = .02). The receiver operating characteristic curve showed a distance >2.2 mm had a sensitivity = 91% and specificity = 86% in predicting the need for PA intervention. The distance from the PA bifurcation to the largest diameter of the branch PA that accepts the PDA on preoperative CTA is highly predictive of the need for initial or remote PA intervention in this group. Preoperative CTA should be considered for risk stratification in neonates undergoing intervention for DD-PBF.

Stenting of branch pulmonary artery stenosis in children: initial experience and mid-term follow-up of the pul-stent

Not all stents are suitable for children. For instance, premounted stents can be used in infants and small children but cannot dilate with age to accommodate adult-sized pulmonary arteries. Conversely, the Pul-Stent adapts to somatic growth. Thus, our hospital implemented the Pul-Stent in pediatric patients with branch pulmonary artery stenosis. This study summarizes our initial experience with Pul-Stents in this patient population, including the efficacy and safety. We implanted 37 Pul-Stents in 35 patients between August 2014 and June 2015. The patients’ mean age and weight at stent implantation were 6.7 ± 3.0 years and 20.9 ± 8.7 kg, respectively. Bench testing revealed that axial shortening of the Pul-Stent was minimal with further dilation, and the radial strength did not change. The stents were successfully deployed in all cases, except two with minor malpositioning. Primarily, 8–12 mm mounting balloons were used for the initial implantation, and a long sheath (8–10 F) was used for delivery. After stent implantation, the minimal lumen diameter in the stenosed segment increased by 50% in 97% (34/35) of patients. Furthermore, the pressure gradient across the stenosed segment decreased by 50% in 77% (23/30) of biventricular patients. One stent fracture and one stent restenosis were noted during the follow-up visits (mean follow-up time: 4.6 ± 1.7 years). Eighteen patients (51%) underwent repeat catheterization; ten had successful redilation. No aneurysms or stent fractures were observed. Our initial results indicate that the Pul-Stent is safe and effective in pediatric patients and can be further dilated over time to accommodate somatic growth. Moreover, the Pul-Stent has good compliance and adequate radial strength to treat pulmonary artery stenosis effectively.

Ductus arteriosus morphology in duct-dependent pulmonary circulation: CT classification and pattern in different ventricular morphology.

The objective was to study the ductus arteriosus morphology in duct-dependent pulmonary circulation and its pattern in different ventricle morphology using CT angiography. From January 2013 to December 2015, patients aged 6 months and below with duct-dependent pulmonary circulation underwent CT angiography to delineate the ductus arteriosus origin, tortuosity, site of insertion, and pulmonary artery anatomy. The ductus arteriosus were classified into type I, IIa, IIb, and III based on its site of origin, either from descending aorta, distal arch, proximal arch, or subclavian artery, respectively. A total of 114 patients and 116 ductus arteriosus (two had bilateral ductus arteriosus) were analysed. Type I, IIa, IIb, and III ductus arteriosus were seen in 13 (11.2 %), 71 (61.2%), 21 (18.1%), and 11 (9.5%), respectively. Tortuous ductus arteriosus was found in 38 (32.7%), which was commonly seen in single ventricular lesions. Ipsilateral and bilateral branch pulmonary artery stenosis was seen in 68 (59.6%) and 6 (5.3%) patients, respectively. The majority of patients with pulmonary atresia intact ventricular septum had type I (54.4%) and non-tortuous ductus arteriosus, while those with single and biventricular lesions had type II ductus arteriosus (84.9% and 89.7%, respectively). Type III ductus arteriosus was more common in biventricular lesions (77.8%). Ductus arteriosus in duct-dependent pulmonary circulation has a diverse morphology with a distinct origin and tortuosity pattern in different types of ventricular morphology. CT may serve as an important tool in case selection and pre-procedural planning for ductal stenting.

Branch Pulmonary Artery Stenosis in Chronic Myeloid Leukaemia: A Rare Presentation

26-year-old lady admitted with shortness of breath and chest discomfort for last one and half months. She had no history of chest pain. She was not suffering from any major illness neither she was taking any medications on regular basis.

Incidence of branch pulmonary artery stenosis requiring intervention based on surgical repair type for tetralogy of Fallot

BackgroundTetralogy of Fallot is a common form of cyanotic congenital heart disease. The right ventricular outflow tract obstruction and pulmonary stenosis are typically repaired using either a transannular patch or valve sparing repair. Following repair, one recognized complication is branch pulmonary artery stenosis. ObjectiveThis study sought to evaluate if there was a higher incidence of branch pulmonary artery stenosis requiring intervention in patients with tetralogy of Fallot who underwent transannular patch repair as compared to valve sparing repair. MethodsIn this retrospective study, 62 pediatric patients with tetralogy of Fallot underwent surgical repair at our institution between January 1, 2006 and August 2, 2016. Data, including echocardiographic measurements, surgical repair details, and post-operative follow up was recorded. Statistical analysis was performed, including chi-square test, Student t-test, multiple logistic regression, and Kaplan-Meier analysis. ResultsMedian length of follow up was 7.3 years (IQR 4.7–9.1). Of the 62 patients, 8 (13 %) developed branch pulmonary artery stenosis requiring intervention. A significantly higher incidence was seen in the transannular patch group (n = 7, 37 %) compared to the valve sparing repair patients (n = 1, 2 %) (p = 0.0002). Most patients required intervention within one year following repair. The effect of repair type persisted after controlling for multiple potential confounding variables, including Blalock-Taussig shunt placement and patch augmentation of the branch pulmonary arteries. ConclusionThere is a significantly higher incidence of branch pulmonary artery stenosis requiring intervention in patients with classic tetralogy of Fallot who underwent transannular patch repair as compared to valve sparing repair.

Automated analysis method to assess pulmonary blood flow distribution using conventional X-ray angiography

Quantitative assessment of the right-to-left ratio of pulmonary blood flow distribution is important for determining the clinical indications for treating pulmonary arterial branch stenosis. A novel theory was recently proposed that can be used to quantitatively assess the right-to-left ratio on conventional X-ray angiography images. In the proposal, further developments were indicated, especially automated calculation. In this study, a new automated algorithm was developed. In the X-ray image, regions of interest were set in right and left lung, and time-signal intensity curves were measured. The new automated algorithm is applied to determine the optimal time window for the analysis of the time-signal intensity curve and to calculate the slope of the curve in the optimized time window. The right-to-left ratios in seven consecutive patients calculated by the new automated algorithm were compared to those calculated by lung perfusion scintigraphy. The ratios were in good agreement with linear regression with a slope of 1.27 and a Pearson correlation coefficient of 0.95. The processing time was less than 10 s, which is one-eighth of the manual processing time. The new automated algorithm is accurate, stable, and fast enough for clinical use in the real world.

Initial status and 3-month results relating to the use of biodegradable nitride iron stents in children and the evaluation of right ventricular function.

BackgroundPulmonary artery stenosis is often associated with congenital heart disease. The aim of the study was to evaluate the efficacy and safety of stenting for branch pulmonary artery stenosis using a biodegradable nitride iron stent (IBS® Angel™) and right ventricular systolic and diastolic function.MethodsFrom July 2021 to February 2022, a total of 11 cases (ages ranged from 36 to 86 months old) were included in this pre and post-intervention, prospective, cohort and preclinical study. All cases underwent transthoracic echocardiographic (TTE), chest radiography, along with computed tomography (256-slice scanner, multiple-detector) and right heart catheterization. Different types of biodegradable nitride iron stents were implanted. TTE was performed serially 1 day, 1 month and 3 months after the procedure to evaluate the rate of restenosis and right ventricular function.ResultsStenting was successful in 11 patients. There were no major adverse cardiovascular events related to the device or to the procedure. Blood perfusion in the branch pulmonary artery was improved immediately. At follow-up, there was no significant restenosis that required re-intervention. None of the patients suffered from in-stent thrombosis, vascular embolism, stent displacement or heart failure. Compared with normal values, there were statistical with regards to FAC, E/A and E′/A′. Furthermore, we found that TAPSE correlated significantly with pulsed Doppler S wave (p = 0.008) and left ventricular ejection fraction (p < 0.01). The early trans-tricuspid inflow velocities E/E′ (tissue doppler at the lateral tricuspid annulus) correlated significantly with E′/A′ (p = 0.009). FAC and E′/A′ were statistically different from those prior to stenting (p = 0.041 and p = 0.035) when tested one month postoperatively. At three months postoperatively, only E/A showed a statistical difference (p = 0.015).ConclusionOur analysis suggests that biodegradable nitride iron stents are feasible, safe, and effective in children. Some small improvements were observed in right ventricular systolic and diastolic function after successful transcatheter intervention, although change was not statistically significant due to the small sample number. (A clinical Trial to Evaluate the Safety and Efficacy of IBS Angel in Patients With Pulmonary Artery Stenosis (IRIS); NCT04973540).

Pulmonary Artery Angioplasty (Main Pulmonary Artery Rotation Flap Technique) in Tetralogy of Fallot Patients With Acute-Angle Branch Pulmonary Artery Stenosis

Despite excellent outcomes of tetralogy of Fallot (TOF) repair, reinterventions for recurrent or residual stenosis of branch pulmonary arteries are still required in many patients. The development of these lesions may be related to 2 inherent characteristic morphologies of TOF: Pulmonary ductal coarctation and acute angulation of branch pulmonary artery, especially the left pulmonary artery. I developed a main pulmonary artery rotation flap angioplasty technique to deal with acute-angle branch pulmonary artery stenosis without using any patch. Since 1998, 68 TOF patients have undergone this technique. During a median follow-up of 13.5 years, 20 (29%) patients underwent catheter or surgical-based intervention for branch pulmonary stenosis. Overall freedom from any intervention was 87%, 76%, 70%, and 59% at 1-year, 5-year, 10-year, and 20-year periods, respectively. I believe that this technique is reproducible and provides excellent intermediate and long-term outcomes in TOF patients with complicated branch pulmonary artery stenosis. Despite excellent outcomes of tetralogy of Fallot (TOF) repair, reinterventions for recurrent or residual stenosis of branch pulmonary arteries are still required in many patients. The development of these lesions may be related to 2 inherent characteristic morphologies of TOF: Pulmonary ductal coarctation and acute angulation of branch pulmonary artery, especially the left pulmonary artery. I developed a main pulmonary artery rotation flap angioplasty technique to deal with acute-angle branch pulmonary artery stenosis without using any patch. Since 1998, 68 TOF patients have undergone this technique. During a median follow-up of 13.5 years, 20 (29%) patients underwent catheter or surgical-based intervention for branch pulmonary stenosis. Overall freedom from any intervention was 87%, 76%, 70%, and 59% at 1-year, 5-year, 10-year, and 20-year periods, respectively. I believe that this technique is reproducible and provides excellent intermediate and long-term outcomes in TOF patients with complicated branch pulmonary artery stenosis.

Elastin Insufficiency Confers Proximal and Distal Pulmonary Vasculopathy in Mice, Partially Remedied by the KATP Channel Opener Minoxidil: Considerations and Cautions for the Treatment of People With Williams-Beuren Syndrome.

BackgroundWilliams Beuren syndrome (WBS) is a recurrent microdeletion disorder that removes one copy of elastin (ELN), resulting in large artery vasculopathy. Early stenosis of the pulmonary vascular tree is common, but few data are available on longer-term implications of the condition.MethodsComputed tomography (CT) angiogram (n = 11) and echocardiogram (n = 20) were performed in children with WBS aged 3.4–17.8 years. Controls (n = 11, aged 4.4–16.8 years) also underwent echocardiogram. Eln+/− mice were analyzed by invasive catheter, echocardiogram, micro-CT (μCT), histology, and pressure myography. We subsequently tested whether minoxidil resulted in improved pulmonary vascular endpoints.ResultsWBS participants with a history of main or branch pulmonary artery (PA) stenosis requiring intervention continued to exhibit increased right ventricular systolic pressure (RVSP, echocardiogram) relative to their peers without intervention (p < 0.01), with no clear difference in PA size. Untreated Eln+/− mice also show elevated RVSP by invasive catheterization (p < 0.0001), increased normalized right heart mass (p < 0.01) and reduced caliber branch PAs by pressure myography (p < 0.0001). Eln+/− main PA medias are thickened histologically relative to Eln+/+ (p < 0.0001). Most Eln+/− phenotypes are shared by both sexes, but PA medial thickness is substantially greater in Eln+/− males (p < 0.001). Eln+/− mice showed more acute proximal branching angles (p < 0.0001) and longer vascular segment lengths (p < 0.0001) (μCT), with genotype differences emerging by P7. Diminished PA acceleration time (p < 0.001) and systolic notching (p < 0.0001) were also observed in Eln+/− echocardiography. Vascular casting plus μCT revealed longer generation-specific PA arcade length (p < 0.0001), with increased PA branching detectable by P90 (p < 0.0001). Post-weaning minoxidil decreased RVSP (p < 0.01) and normalized PA caliber (p < 0.0001) but not early-onset proximal branching angle or segment length, nor later-developing peripheral branch number.ConclusionsVascular deficiencies beyond arterial caliber persist in individuals with WBS who have undergone PA stenosis intervention. Evaluation of Eln+/− mice reveals complex vascular changes that affect the proximal and distal vasculatures. Minoxidil, given post-weaning, decreases RVSP and improves lumen diameter, but does not alter other earlier-onset vascular patterns. Our data suggest additional therapies including minoxidil could be a useful adjunct to surgical therapy, and future trials should be considered.