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Background Branch 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. Methods This 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 VO 2 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 VO 2 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. Discussion This 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 registration ClinicalTrials.gov NCT05809310. Registered on March 15, 2023.

The arterial switch operation (ASO) is the standard surgical treatment for transposition of the great arteries (TGA). Postoperative complications such as branch pulmonary artery (PA) stenosis are commonly observed. This study aimed to investigate the possible potential anatomical mechanisms contributing to isolated branch PA stenosis using cardiac computed tomography (CCT). A retrospective, single-center study was conducted on pediatric patients under 18 years of age diagnosed with TGA, with or without ventricular septal defect (VSD), who underwent ASO and cardiac CCT between January 2004 and October 2022. Baseline characteristics, echocardiographic data, and CCT findings were compared between patients with and without isolated branch PA stenosis. Special attention was given to the angle between the PA bifurcation and the ascending aorta (AAO). Among 30 patients enrolled, 46.67% (14/30) had isolated branch PA stenosis, while 53.33% (16/30) had no stenosis. Baseline and echocardiographic variables showed no significant differences between groups, except for the PA bifurcation angle relative to the AAO. Patients with isolated right PA (RPA) stenosis demonstrated significantly greater leftward angulation (median angle: 25° [IQR: 24, 30]) compared to the no-stenosis group (median angle: -2° [IQR: -8, 6.5]). Similarly, isolated left PA (LPA) stenosis was associated with greater rightward angulation (median angle: -22° [IQR: -32, -20]) compared to the no-stenosis group (P = 0.042). Alterations in the PA bifurcation angle relative to the AAO may contribute to the development of isolated branch PA stenosis following ASO. Preoperative planning to optimize the PA bifurcation angle to within ±20 degrees may help reduce the risk of postoperative stenosis. A larger study incorporating advanced cardiac imaging into routine ASO follow-up is warranted.

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 2.5 mm in diameter). Seventeen (12 male) patients were considered for the procedure. The median age and weight were 27 days (range 2–94) and 2.6 kg (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.

Branch pulmonary artery (PA) stenosis due to ductus arteriosus (DA) tissue (DA-PS) contributes to the morbidity associated with pulmonary atresia (PAtr). We sought to identify preoperative echocardiographic features predictive of DA-PS. Patients consecutively encountered with PAtr and a DA-dependent pulmonary circulation at birth who underwent intervention in our program over a 5-year period were identified and records reviewed. Preoperative echocardiograms were reviewed to identify features that predicted postoperative DA-PS. Seventy patients with PAtr met inclusion criteria and 36 (51%) had DA-PS. At preoperative echocardiography, the proximal diameter of the PA ipsilateral to the DA was smaller in those with versus without DA-PS ( Z -score − 4.8 ± 1.7 vs − 1.1 ± 1.7, respectively p  < 0.001). PA origins could not be imaged on the same axial plane in 21/36 (58%) with versus 2/34 (6%) without DA-PS. Patients with DA-PS had an obtuse posterior angle of the PA bifurcation compared to those without (128 ± 17° and 87 ± 21°, p  < 0.001), and a posterior angle of > 100° best predicted DA-PS with a sensitivity of 97% and specificity of 76%. An abnormal PA relationship and/or an obtuse posterior bifurcation angle had a sensitivity, specificity, positive and negative predictive value for DA-PS of 78%, 94%, 90% and 86%, respectively. Finally, DA insertion was into the ipsilateral PA in 26/36 (72%) of cases with DA-PS. A smaller proximal ipsilateral PA diameter, inability to image the PAs in the same plane, a posterior PA bifurcation angle of > 100°, and insertion of the DA in the ipsilateral PA demonstrated by echo are useful in identifying patients at risk for DA-PS.

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 8 mGy-cm and the mean calculated effective radiation dose was 0.312 mSv. 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.

A 9-day-old male neonate was found to have a systolic murmur during a routine follow-up for skin jaundice. Imaging revealed a large mass at the bifurcation of the main pulmonary artery, causing significant bilateral stenosis. The patient underwent emergency surgery due to critically compromised pulmonary blood flow. Intraoperative exploration demonstrated a large ductus arteriosus aneurysm (DAA) with extensive thrombosis. The DAA was completely resected under deep hypothermic circulatory arrest, followed by reconstruction of the descending aorta and repair of the pulmonary artery bifurcation defect. The patient was discharged on the 20th postoperative day and remained asymptomatic at the 3-month follow-up, exhibiting normal growth and cardiac function.

We report the case of an 11-year-old boy with situs inversus, interrupted inferior vena cava with azygos continuation, persistent left superior vena cava with absent bridging vein, congenitally corrected transposition of the great arteries, pulmonary atresia, and ventricular septal defect. He underwent a Kawashima shunt at age 1 and fenestrated extracardiac conduit Fontan completion at age 9, delayed due to loss to follow-up while living abroad. Fifteen months post-op, the patient was diagnosed with plastic bronchitis. Cardiac catheterization revealed elevated Fontan pressures and proximal left pulmonary artery stenosis, which was stented (AndraTec 38 mm Optimus-XL). Although fenestration creation was considered, the procedure was initially delayed allowing for a trial of medical management. Four months later, a 6 mm Occlutech atrial flow regulator (AFR) was placed transjugularly, but thrombosis occurred 48 h later. Transjugular catheter-based clot aspiration (Penumbra Lightning ® 7) and stenting (10/20 mm Cook Formula stent) followed by sequential high-pressure ballooning increased the fenestration diameter, improving symptoms and hemodynamics. He had a favorable recovery with significant improvements and remained stable at 13 weeks of follow-up.

Granulomatosis with polyangiitis (GPA) is a rare disease that belongs to the group of necrotizing systemic vasculitis, which is characterized by the involvement of small and medium-sized blood vessels, the formation of granulomas, and the presence of proteinase 3-anti-neutrophil cytoplasmic antibodies (PR3-ANCA). The disease typically involves the upper and lower respiratory tract and kidneys, but other organs and systems may also be involved. A rare manifestation of the disease is the involvement of large vessels, which may occur in the form of aneurysms, dissection and stenosis. We present the case of a 39-year-old male patient who was diagnosed with GPA several years ago, with the involvement of lungs, kidneys, skin and inflammation of the ascending aorta. The patient currently reported fever, cough and dyspnea. The imaging studies showed stenosis of the right and left pulmonary arteries. The treatment included glucocorticosteroids and rituximab. A review of the literature on pulmonary artery involvement in granulomatosis with polyangiitis was performed, seven case reports meeting such criteria were found.

Background Maldistribution of pulmonary artery blood flow (MPBF) is a potential complication in patients who have undergone single ventricle palliation culminating in the Fontan procedure. Cardiovascular magnetic resonance (CMR) is the best modality that can evaluate MPBF in this population. The purpose of this study is to identify the prevalence and associations of MPBF and to determine the impact of MPBF on exercise capacity after the Fontan operation. Methods This retrospective single-center study included all patients after Fontan operation who had maximal cardiopulmonary exercise test (CPET) and CMR with flow measurements of the branch pulmonary arteries. MPBF was defined as > 20% difference in branch pulmonary artery flow. Exercise capacity was measured as percent of predicted oxygen consumption at peak exercise (% predicted VO 2 ). Linear and logistic regression models were used to determine univariate and multivariable predictors of exercise capacity and correlates of MPBF, respectively. Results A total of 147 patients who had CMR between 1999 and 2017 were included (median age at CMR 21.8 years [interquartile range (IQR) 16.5–30.6]) and the median time between CMR and CPET was 2.8 months [IQR 0–13.8]. Fifty-three patients (36%) had MPBF (95% CI 29–45%). The mean % predicted VO 2 was 63 ± 16%. Patients with MPBF had lower mean % predicted VO 2 compared to patients without MPBF (60 ± 14% versus 65 ± 16%, p  = 0.04). On multivariable analysis, a lower % predicted VO 2 was independently associated with longer time since Fontan, higher ventricular mass-to-volume ratio, and MPBF. On multivariable analysis, only compression of the branch pulmonary arteries by the ascending aorta or aortic root was associated with MPBF (OR 6.5, 95% CI 5.6–7.4, p  < 0.001). Conclusion In patients after the Fontan operation, MPBF is common and is independently associated with lower exercise capacity. MPBF was most likely to be caused by pulmonary artery compression by the aortic root or the ascending aorta. This study identifies MPBF as an important risk factor and as a potential target for therapeutic interventions in this fragile patient population.

To investigate the long-term efficacy of percutaneous transluminal pulmonary angioplasty (PTPA) in patients with Takayasu arteritis (TA) and pulmonary artery stenosis and pulmonary hypertension (PH). Data from 183 lesions from 79 surgeries performed on 32 patients with TA and PH were analyzed. Symptoms, laboratory investigation results, World Health Organization (WHO) functional class, 6-min walk distance (6 MWD), hemodynamic parameters, and prognosis were analyzed at baseline and follow-up. The mean (± SD) age of the 32 patients (28 female, 4 male) was 42.8 ± 11.9 years, and the median follow-up was 49.5 months (interquartile range, 26-71 months). Compared with baseline, changes in total bilirubin, N-terminal pro-brain natriuretic peptide (NT-proBNP) level, 6 MWD, and WHO score functional class demonstrated significant differences ( <0.001). Echocardiography findings, right and left ventricular diameter, tricuspid annular plane systolic excursion, and estimated pulmonary artery systolic pressure were all improved ( =0.016, <0.001, <0.001, =0.005, respectively). Importantly, repeat right heart catheterization revealed that mean pulmonary artery pressure, pulmonary vascular resistance, and cardiac index also improved significantly at follow-up ( <0.001, <0.001, and =0.011, respectively). Pulmonary angiography revealed post-procedure restenosis in 64 (35.0%) lesions underwent PTPA within three to six months. Among three patients who underwent stent implantation, one experienced restenosis. Two patients died during the follow-up period, one from aggravation of right heart failure after lung infection, and the other in a traffic accident. Results of this study indicated that PTPA significantly improved clinical symptoms, exercise tolerance, and hemodynamic parameters in patients with TA pulmonary artery stenosis and PH. More importantly, reperfusion pulmonary edema significantly decreased, and no patient died of PTPA-related complications with guidance from the pressure wire.