Pulmonary Artery Interventions Research Articles

Pulmonary artery pseudoaneurysm in young male with pulmonary hypertension

Pulmonary artery aneurysms and pseudoaneurysms are rare abnormalities but associated morbidity causes recognition importance. Here we report a clinical case of a 15-year-old male who arrived at our clinic complaining of hemoptysis and dry cough. Diagnosis revealed the mass lesion of the left lung. CT-angiography and intervention of pulmonary arteries confirmed pulmonary artery aneurysms, which explained hemoptysis caused by aneurysm rupture. The pulmonary artery intervention confirmed the condition.

Comparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation

ObjectiveWe aimed to compare the pulmonary artery (PA) growth between infants with univentricular hearts who underwent a ductus stenting (DS) and those who received a systemic to pulmonary shunt (SPS) as an initial palliation. MethodsAll infants with ductal-dependent pulmonary blood flow who underwent initial palliation with either a DS or SPS between 2009 and 2022 in our institution were reviewed. PA development was compared between the groups using the PA index and the symmetry index. ResultsA total of 130 patients were evaluated including 49 patients after DS and 81 after SPS. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19. At stage II palliation, PA index (P = .926), right PA index (P = .692), left PA index (P = .297), and the symmetry index (P = .650) were similar between the groups. At stage III Fontan completion, PA index (P = .115), right PA index (P = .868), and the symmetry index (P = .144) were similar between the groups. However, left PA index (60 vs 74 mm2/m2; P = .015) was lower, and the incidence of venovenous collaterals (24.2% vs 7.8%; P = .036) was higher in the DS group compared with the SPS group. Freedom from PA interventions between stage II and III palliation was lower in the DS group compared with the SPS group (P = .009). ConclusionsIn infants with univentricular heart after DS, freedom from PA interventions after stage II was lower and the left PA index was smaller, compared with those after SPS. Long-term outcomes after the Fontan procedure should be addressed in patients after DS.

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.

Simultaneous stent implantation for pulmonary artery bifurcation stenosis in infants.

Pulmonary artery (PA) bifurcation stenosis often requires simultaneous stent placement, which may be technically challenging. Limited data exist regarding this practice in infants. We aim to report the procedural outcomes and safety of bifurcation stent placement in infants. We performed a single-center retrospective review of infants younger than 12 months who underwent simultaneous stent placement for PA bifurcation stenosis from January 1, 2001 through December 31, 2019. Seventeen infants underwent simultaneous PA bifurcation stent placement. The median age was 6.4 months (1.1-10.1 months), and weight was 5.8 kg (3-10.6 kg). Nine (52.9%) patients had had prior PA intervention. Most stents were placed in central PAs (28, 82.4%), followed by lobar branches (6, 17.6%). All patients received pre-mounted stents. The peak gradient across each branch decreased from 47.4 ± 16 to 18.7 ± 13 mm Hg (P less than .0001). The right ventricle to systemic systolic pressure ratio decreased from systemic (1.0 ± 0.3) to just over half systemic (0.58 ± 0.2) (P = .0001). The minimum vessel diameter increased from 3.6 ± 1.5 to 6.0 ± 1.9 mm (P less than .0001). There were 4 (23.5%) patients with high severity adverse events. There were no procedure-related deaths. The median follow-up period was 83.8 months (5.3 months-19.4 years). All patients had subsequent PA re-intervention at a median time of 8.1 months (2.9 months-8.8 years), and median time to re-operation was 19.1 months (2.9 months-7.5 years). Simultaneous PA stent placement is an effective strategy for relief of bifurcation stenosis in infants. Future transcatheter interventions are necessary to account for patient growth, but may delay the need for re-operation.

Comparing palliation strategies for single-ventricle anatomy with transposed great arteries and systemic outflow obstruction.

Patients with complex single-ventricle anatomy with transposed great arteries and systemic outflow obstruction (SV-TGA-SOO) undergo varied initial palliation with ultimate goal of Fontan circulation. We examine a longitudinal experience with multiple techniques, including the largest published cohort following palliative arterial switch operation (pASO), to describe outcomes and decision-making factors. Neonates with SV-TGA-SOO who underwent initial surgical palliation from 1995 to 2022 at a single institution were retrospectively reviewed. In total, 71 neonates with SV-TGA-SOO underwent index surgical palliation at a median age of 7days (interquartile range, 6-10) by pASO (n=23), pulmonary artery band (PAB) with or without arch repair (n=25), or modified Norwood with Damus-Kaye-Stansel aortopulmonary amalgamation (n=23). Single-ventricle pathology included double-inlet left ventricle (n=37, 52%), tricuspid atresia (n=27, 38%), and others (n=7, 10%). All mortalities (n=5, 7%) occurred in the first interstage period after PAB (n=3) and Norwood (n=2). Subaortic obstruction in the PAB group was addressed by operative resection (n=10 total, 7 at index operation) and/or delayed aortopulmonary amalgamation (n=13, 52%). Two patients with pASO (9%) had early postoperative coronary complications, 1 requiring operative revision. Median follow-up for survivors was 10.4years (interquartile range, 4.5-16.6years). Comparing patients by their initial palliation type, notable significant differences included size of bulboventricular foramen, weight at initial operation, operation duration, postoperative length of stay, time to second-stage palliation, multiple pulmonary artery reinterventions, and left pulmonary artery interventions. There were no significant differences in overall survival, Fontan completion, reintervention-free survival in the first interstage period, pulmonary artery reintervention-free survival, long-term systemic valve competency, or ventricular dysfunction. Excellent mid- to long-term outcomes are achievable following neonatal palliation for SV-TGA-SOO via pASO, PAB, and modified Norwood, with comparable survival and Fontan completion. Initial palliation strategy should be individualized to optimize anatomy and physiology for successful Fontan by ensuring an unobstructed subaortic pathway and accessible pulmonary arteries. pASO is a reasonable strategy to consider for these heterogeneous lesions.

Pre-Glenn aorto-pulmonary collaterals in single-ventricle patients.

In single-ventricle patients undergoing staged-bidirectional Glenn, 36-59% have aorto-pulmonary collateral flow, but risk factors and clinical outcomes are unknown. We hypothesise that shunt type and catheter haemodynamics may predict pre-bidirectional Glenn aorto-pulmonary collateral burden, which may predict death/transplantation, pulmonary artery or aorto-pulmonary collateral intervention. Retrospective cohort study of patients undergoing a Norwood procedure for single-ventricle anatomy. Covariates included clinical and haemodynamic characteristics up to/including pre-bidirectional Glenn catheterisation and aorto-pulmonary collateral burden at pre-bidirectional Glenn catheterisation. Multivariable models used to evaluate relationships between risk factors and outcomes. From January 2011 to March 2016, 104 patients underwent Norwood intervention. Male sex (odds ratio 3.36, 95% confidence interval 1.17-11.4), age at pre-bidirectional Glenn assessment (2.12, 1.33-3.39 per month), and pulmonary to systemic flow ratio (1.23, 1.08-1.41 per 0.1 unit) were associated with aorto-pulmonary collateral burden. Aorto-pulmonary collateral burden was not associated with death/transplantation (hazard ratio 1.19, 95% confidence interval 0.37-3.85), pulmonary artery (sub-hazard ratio 1.38, 0.32-2.61), or aorto-pulmonary collateral interventions (sub-hazard ratio 1.11, 0.21-5.76). Longer post-Norwood length of stay was associated with greater risk of death/transplantation (hazard ratio 1.22 per week, 95% confidence interval 1.08-1.38), but lower risk of aorto-pulmonary collateral intervention (sub-hazard ratio 0.86 per week, 95% confidence interval 0.75-0.98). Time to pre-bidirectional Glenn catheterisation was associated with lower risk of pulmonary artery (sub-hazard ratio 0.80 per month, 95% confidence interval 0.65-0.98) and aorto-pulmonary collateral intervention (sub-hazard ratio 0.79, 0.63-0.99). Probability of moderate/severe aorto-pulmonary collateral burden increased with left-to-right shunt (22.5% at <1.0, 57.6% at >1.4) and the age at pre-bidirectional Glenn catheterisation (10.6% at <2 months, 56.9% at >5 months). Aorto-pulmonary collateral burden is common after Norwood procedure and increases as age at bidirectional Glenn increases. As expected, higher pulmonary to systemic flow ratio is a marker for greater aorto-pulmonary collateral burden pre-bi-directional Glenn; aorto-pulmonary collateral burden does not confer risk of death/transplantation or pulmonary artery intervention.

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.

Branch pulmonary artery stent angioplasty in infants less than 10 kg

BackgroundBranch pulmonary artery (PA) Stenting is an established strategy for PA stenosis in older children and adults. Its use in infants is less well established. We describe our experience of branch PA Stenting in infants <10 kg. MethodRetrospective chart review of infants <10 kg who underwent PA Stenting at The Children's Hospital, Westmead between 2010 and 2020. Pre and post-procedural angiograms were reviewed to determine PA size. Technical procedural success was defined as >50% increase in PA diameter. Procedural complications and need for PA re-intervention were ascertained. ResultsForty-one children (age 7.6 months, IQR 2.4–9.8 months, weight 5.9 ± 2.2 kg) had 43 primary Stent implantations (10/43 intraoperative versus 33/43 transcatheter; left pulmonary artery [LPA] 25/43, right pulmonary artery [RPA] 8/43 and bilateral 10/43). Diagnoses were tetralogy of Fallot (27%), hypoplastic left heart syndrome (24%), truncus arteriosus (12%), transposition of the great arteries (7%), other single ventricle (10%) and other biventricular (20%). 40/41 (98%) had undergone a cardiac intervention in the preceding 31 days, [IQR 1–181], with 17/41 having prior branch PA intervention. 14/41 patients had urgent Stenting. There were 2 minor and 1 major complications with no procedural mortality. LPA (LPApre 2.3 ± 1.0 × 2.2 ± 1.2 mm versus LPApost 5.2 ± 1.3 × 5.0 ± 1.7 mm, p < 0.01) and RPA (RPApre 2.5 ± 0.8 × 1.9 ± 0.8 mm versus RPApost 4.9 ± 1.0 × 4.1 ± 1.0 mm, p < 0.01) calibre increased post Stenting. 20/41 required branch PA reintervention (time to reintervention 13.6 months [IQR 8.2–29.3]. ConclusionsBranch PA Stenting is effective and safe in infants <10 kg with expected high rates of reintervention. Urgent PA Stenting provides relief of early post-operative haemodynamic compromise.

Intermediate-term outcomes of deferred Norwood strategy.

The aim of this study was to assess outcomes of the deferred Norwood strategy, i.e. planned Norwood following routine bilateral pulmonary artery banding and ductal stenting or continuous prostaglandin E1 administration. Forty-five patients with hypoplastic left heart syndrome and its variants treated with the deferred Norwood strategy between 2012 and 2021 were enrolled. Mid-term outcomes were retrospectively reviewed. The median follow-up period after Norwood in hospital survivors was 4.6 years (interquartile range: 1.9, 6.8). Fourteen patients (31.1%) had no risk factors. The median age and weight at Norwood were 1.8 months (0.9, 3.5) and 3.1 kg (2.7, 3.6). Transplant-free survival at 6 years was 84.5%. Birth weight ≤2.5 kg and systemic atrioventricular valve (SAVV) regurgitation ≥ moderate after birth were not risk factors for mortality; body weight at Norwood ≤2.5 kg, however, was a risk factor [hazard ratio (HR), 11.3; 95% confidence interval (CI), 1.2-11; P = 0.036]. Twenty-two (48.9%) underwent Fontan with no mortalities, and 7 (15.5%) are awaiting Fontan. Freedom from SAVV surgery at 5 years was 53.1%. SAVV regurgitation ≥ moderate after birth was a risk factor for SAVV surgery (HR, 16; 95% CI, 3.6-71; P < 0.001); however, ductal stenting had a protective effect against SAVV surgery (HR, 0.09; 95% CI, 0.01-0.68; P = 0.019). Freedom from both surgical and catheter-based pulmonary artery intervention at 3 years was 27.1. Although deferred Norwood provided acceptable intermediate-term survival, the Fontan completion rate was unsatisfactory. SAVV surgery and pulmonary artery intervention were frequently required.

CT-guided transthoracic pulmonary artery catheterization: an experimental study in a porcine model.

We investigated the safety and feasibility of CT-guided transthoracic pulmonary artery catheterization (TPAC) in a porcine model. Procedures were conducted on ten mature Bama miniature pigs. After anesthesia, chest CT was performed in the left lateral decubitus position to determine the puncture route. Under the guidance of multiple CT scans, the introducer sheath was inserted from the right chest wall of the pig into the right pulmonary artery using the Seldinger technique. Then, a catheter connected with a transducer was inserted into the sheath to measure the pulmonary artery pressure. Finally, an active approximator was used to close the puncture site on the pulmonary artery. The pigs were followed up for 8weeks to evaluate the operation-related complications and survival. Ten of 11 CT-guided TPAC procedures were successfully performed on ten pigs, rendering a technical success rate of 90.9%. One pig had hemoptysis while the needle was being inserted during the first operation, and a second procedure was successfully conducted 17days later. Other complications, including pulmonary bleeding along the needle track (3 of 11; 27.3%), unclosed pulmonary artery puncture sites (3 of 10; 30%), pneumothorax (1 of 11; 9.1%), and hemopericardium (1 of 11; 9.1%), spontaneously resolved without complication-specific treatment. The mean pulmonary arterial pressure was 32 ± 17.6mmHg. All animals survived the procedure and reached the end of the follow-up period. CT-guided TPAC is feasible and safe in a porcine model, serving as a potential alternative pathway for pulmonary artery intervention. • TPAC is feasible and safe in a porcine model, serving as a potential alternative pathway for pulmonary artery intervention. • This novel approach allows for faster access to the pulmonary artery, and it might be easier to operate the tip of the catheter to super-select the intent branch of the pulmonary artery. • TPAC can be an alternative pulmonary artery intervention pathway in patients with mechanical right-heart valves, great-vessel transposition, and other obstacles.

A gray zone in the literature: indications, options, and outcomes for surgical repair of pulmonary artery aneurysms.

Pulmonary artery aneurysms are rare. They are characterised by an aneurysmatic dilatation of the pulmonary vascular bed, including the main pulmonary artery or the accompanying pulmonary artery branches. Increases in pulmonary flow and pulmonary artery pressure increase the risk of rupture: when these conditions are detected, surgical intervention is necessary.This study is a retrospective analysis of 33 patients treated in our paediatric cardiac surgery clinic from 2012 to 2020. Aneurysms and pseudoaneurysms in patients who were patched for right ventricular outflow tract reconstruction and corrected with a conduit were excluded from the study. Seventeen (51.5%) of the patients included in the study were female and 16 (48.5%) were male. The patients were aged between 23 and 61 years (mean 30.66 ± 12.72 years). Graft interpositions were performed in 10 patients (30.3%) and pulmonary artery plications were performed in 23 patients (69.7%) to repair aneurysms. There was no significant difference in mortality between the two groups (p > 0.05).Pulmonary artery aneurysm interventions are safe, life-saving treatments that prevent fatal complications such as ruptures, but at present there is no clear guidance regarding surgical timing or treatment strategies. Pulmonary artery interventions should be performed in symptomatic patients with dilations ≥5 cm or asymptomatic patients with dilations ≥8 cm; pulmonary artery pressure, right ventricular systolic pressure, and pulmonary artery aneurysm diameter must be considered when planning surgeries, their timing, and making decisions regarding indications. Experienced surgical teams can achieve satisfactory results using one of the following surgical techniques: reduction pulmonary arterioplasty, plication, or graft replacement.

Abstract 12078: Pre-Glenn Aorto-Pulmonary Collaterals and Its Impact on Outcomes in Single Ventricle Patients

Introduction: In single-ventricle patients undergoing bi-directional Glenn (BDG), 36-59% have angiographically detectable aorto-pulmonary collateral (APC) flow. However, predictors, hemodynamic and clinical outcomes are unknown. Hypothesis: We hypothesize that (a) shunt type, hemodynamic findings at the pre-Glenn catheterization predict pre-BDG APC burden and (b) APC burden at BDG catheterization may predict post BDG death/transplantation, pulmonary artery (PA) or APC intervention. Methods: Retrospective cohort study of patients following Norwood Procedure for single ventricle anatomy. Covariates included demographics; clinical and hemodynamic at Pre-BDG catheterization. APC burden at pre-BDG catheterization was assessed dichotomously by a single reader (moderate/severe vs. none/mild). Logistic regression was used to identify predictors of APC burden and Cox regression for time to clinical outcomes. Results: Among 104 patients, 89.4% (n=93/104) underwent pre-BDG catherization, of which 55% (n=51/93) had APC intervention and 91% (n=85/93) progressed to BDG. Post-BDG, 60% (n=51/85) had no/mild and 40% (n=34/85) had moderate/severe APC burden. Within 36 months, APC intervention occurred in 74% (n=69/63) with no difference between groups (84% vs. 79%, Logrank 0.75). Predictors of APC burden were male sex (OR 3.59; 95% CI 1.18-10.98), older age at BDG (1.02 per year; 1.01-1.04), PA saturations (1.80;1.18-2.75), and Qp:Qs ratio (OR 1.23 per 0.10 unit increase;1.08-1.41). APC burden is not predictive of death/transplantation or future APC intervention but may show a trend towards future PA intervention (HR 2.11, 95% CI 0.98-4.52, p=0.056)(Figure 1). Sano shunt is a predictor of APC intervention following BDG (HR 2.07, 95%CI 1.09-3.90, p=0.03). Conclusions: Moderate or severe APC burden was present in 40% after stage I Norwood and QP:QS is a strong predictor for APC burden; patients with moderate/severe APC’s may be at higher risk of PA interventions.

Impact of Transcatheter Pulmonary Artery Intervention Following Superior Cavopulmonary Connection on Pulmonary Artery Growth.

Balloon and stent angioplasty of the pulmonary arteries (PAs) are frequently performed following superior cavopulmonary connection (SCPC), not only to normalize the caliber of the affected PA but also in hopes of maximizing downstream growth over time. There are limited data on the impact on subsequent PA growth prior to total cavopulmonary connection (TCPC). A single-center, retrospective cohort study was performed on children who underwent transcatheter (TC) PA intervention following SCPC between January 1, 2010, and December 31, 2018. Growth of treated and contralateral PAs was measured at the lobar bifurcation (distal branch PA [DBPA]) and in the proximal lower lobe (lower lobe branch [LLB]) on serial angiograms. Growth rate was evaluated using a mixed-effect model clustered by individual patient with an interaction term for treated PA and time to evaluate for differential growth rates between treated and contralateral PAs. Thirty-five patients underwent TC PA intervention following SCPC, at a median of 70 days (interquartile range: 19-297 days) postoperatively. Significant growth was seen at both DBPA and LLB for raw (0.8 mm/year, 95% CI: 0.6-1.0, P < .001 for both) and body surface area (BSA) adjusted measures (8.4mm/m2/year, 95% CI: 5.6-11.2, P < .001; 7.9 mm/m2/year, 95% CI: 5.5-10.2, P < .001). The growth rate of the treated vessel was not significantly different from that of the contralateral vessel at the DBPA or LLB positions for raw (P = .71, .70) or BSA-adjusted measurements (P = .86, .64). Transcatheter PA intervention was associated with normal distal PA growth rate relative to the untreated side.

Pulmonary Artery Intervention for Submassive Pulmonary Embolism From an Arm Superficial Venous Access.

Pulmonary embolism (PE) endovascular interventions are often approached from an internal jugular or femoral venous access. There are multiple advantages of right basilic vein (RBV) access for both patient and operator, especially in the setting of morbid obesity. We hereby describe the case of a 48-year-old, morbidly obese man who presented with acute respiratory insufficiency and was found to have bilateral submassive subocclusive PE, worse on the right. The right ventricular to left ventricular ratio was 2.1 and troponin was elevated. A 7 Fr sheath was placed in the RBV under ultrasound guidance. Selective bilateral pulmonary arteriography was then performed. A 106 x 12 cm EKOS catheter was placed in the segment of highest thrombotic burden for a 6-hour protocol of catheter-directed ultrasound-facilitated thrombolytic therapy. The patient recovered well on a direct oral anticoagulant and his acute symptoms resolved. Treating massive/submassive PE from a RBV access offers the convenience and safety of superficial venous access (for patient and operator), better patient comfort, less venous stasis during therapy with ability to ambulate, less potential for bleeding and vascular complications, less potential for operator radiation exposure when compared with the jugular approach, and better operator ergonomics.

Postoperative pulmonary artery stenosis in lung transplantation recipients: review of case series

Background: Postoperative pulmonary artery stenosis (PAS) is rare complication that can appear lung transplant recipients due to inappropriate donor to recipient lung size matching. In this study, we retrospectively reviewed patients who were diagnosed PAS after lung transplantation. Methods: Medical records were retrospectively reviewed in the group of lung transplant recipients from 2012 to 2019 in Sever ance Hospital, Korea. Results: Among 267 recipients who underwent lung transplantation from October 2012 to December 2019, 12 patients were diagnosed PAS and treated accordingly. Four patients received surgical treatment. Six patients were successfully received in terventional therapy: pulmonary angioplasty or pulmonary artery (PA) stent insertion. Two patients were under observation due to benign progress. Mortality were observed in three patients, and one death was related to PAS. Three patients had either left or right donor-to-recipient PA mismatch and four patients had donor lung size mismatch. After excluding the mortality and ob servant cases, eight patients had relief of symptoms and five patients showed increased outcome in before-and-after perfusion scan. Most common initial manifestation was progressive dyspnea and chest discomfort (seven cases), while median time of diagnosis-to-treatment was 36 days. Seven patients were diagnosed PAS in 2 weeks from day of lung transplantation. In this group, most common clue for diagnosis was acute change of chest X-ray (increased haziness) and hypoxemia. On contrary, five patients who were diagnosed after 2 weeks tended to not show acute change in chest X-ray, rather most common phenomenon was progressive dyspnea, chest discomfort, and prolonged pneumonia. Conclusions: PAS after lung transplantation can be managed by either PA intervention or surgical approach depending on cir cumstance of patients. Mortality were most likely associated with underlying patient’s condition such as septic shock. Progres sive dyspnea, chest discomfort, hypoxemia, and left lung haziness in chest X-ray may indicate PAS. Early diagnosis-to-treatment is vital for favorable outcome.

Abstract 16377: Cardiovascular Computed Tomography Can Predict Branch Pulmonary Artery Stenosis in Neonates With Ductal Dependent Pulmonary Blood Flow

Background: A subpopulation of neonates with ductal dependent pulmonary blood flow (PBF) are at risk for branch pulmonary artery (PA) stenosis in the PA that receives the patent ductus arteriosus (PDA). Identification of this population is important in planning initial and future interventions. The objective of this study was to identify novel cardiovascular computed tomography (CCT) measures in the preoperative period that are associated with the need for future branch PA surgical or catheter-based intervention. Methods: In this single center retrospective study, we identified neonates (&lt; 28 days) with ductal dependent PBF who underwent CCT prior to initial intervention from 11/2003-12/2019. A single reviewer made conventional and novel measurements of the branch PAs as well as PDA size and configuration. The primary outcome was reached if there was any initial or subsequent surgical or catheter-based intervention on the branch PA that received the PDA. Results: A total of 47 neonates (16 female, 31 male) were included. 30 (64%) reached the primary outcome. Upon multivariable regression analysis, PDA origin at the aortic isthmus [OR 7.13 (95% CI 1.01-50.4), p = 0.049] and distance to the largest diameter (DLD) (Figure 1) [OR 1.24 (95% CI 1.01-1.51), p = 0.040] were independently associated with the primary outcome. DLD is the distance of the largest diameter of the PA with the PDA from the bifurcation in confluent PAs or from the insertion of the PDA in discontinuous PAs. On receiver operating characteristic analysis, area under the curve for DLD = 0.75, p = 0.01. A DLD &gt; 6 mm displayed 82% sensitivity and 67% specificity in identifying patients who met the primary outcome. Conclusions: PDA origin at the aortic isthmus and DLD can risk stratify patients at high risk for requiring surgical or catheterization-based branch PA interventions. Further analysis is needed to determine if intervention strategies based on these measurements improve patient outcomes.

Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease

To assess outcomes in a large cohort of patients with Alagille syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex pulmonary artery disease. Patients with ALGS who underwent pulmonary artery reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch pulmonary artery stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs. Fifty-one patients with ALGS underwent pulmonary artery surgery at our center, including 22 with severe branch pulmonary artery stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) underwent complete repair at the first surgery, compared with 8 (89%) with TOF without MAPCAs and 19 (86%) with isolated branch pulmonary artery stenosis. At a median follow-up of 1.7years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median pulmonary artery:aortic systolic pressure of 0.38. Nine patients (18%), including 8 with isolated branch pulmonary artery stenosis, underwent liver transplantation. Most patients with ALGS and complex pulmonary artery disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcomes, with higher mortality and more frequent pulmonary artery interventions compared with patients with TOF without MAPCAs or isolated branch pulmonary artery stenosis. Complex pulmonary artery disease is not a contraindication to liver transplantation in patients with ALGS.

Cardiac catheter intervention complexity and safety outcomes in adult congenital heart disease

ObjectiveTo describe the intervention spectrum, complexity, and safety outcomes of catheter-based interventions in a contemporary adult congenital heart disease (ACHD) tertiary cohort.MethodsAll patients over 16 years who underwent a catheter-based...

Early Infant Symptomatic Patients With Tetralogy of Fallot With Absent Pulmonary Valve: Pulmonary Artery Management and Airway Stabilization

Tracheobronchomalacia and airway obstruction from severely dilated pulmonary arteries in tetralogy of Fallot with absent pulmonary valve (TOF-APV) has been associated with high rates of respiratory failure and mortality (15% to 25%). It is not known whether aggressive pulmonary artery (PA) or direct airway intervention during early definitive cardiac repair improves outcomes. A retrospective observational study was made of all patients undergoing surgical repair for TOF-APV at our center between 2006 and2018. Twenty patients underwent repair at a median age of 51 days and PA Z-scores of 8.1. Twelve patients had a valve implanted, 6 of whom required reoperation for valve replacement at a median of 9 months (range, 3 to 28) compared with 8 who had initial transannular patch, and only 1 patient required subsequent valve replacement (P < .05). Seven patients had central PAs replaced with thin-walled Gore-Tex (WL Gore, Flagstaff, AZ) grafts; none of these required PA reoperation during a median follow-up of 26.5 months, whereas 3 of 13 patients who did not have PA replacement with Gore-Tex required subsequent PA reoperation (P < .05). Concomitant airway interventions (eg, tracheobronchopexy/plasty) were performed in 4 patients and none required subsequent airway interventions, whereas 2 patients not having initial airway intervention required subsequent tracheopexy (P < .05). Three patients in the cohort eventually required tracheostomy (15%), and 2 patients died (10%; on postoperative days 30 and 326); none had received initial airway intervention. Pulmonary artery replacement and aggressive direct airway management at initial definitive repair of cardiac TOF-APV can be performed safely with acceptable survival outcomes and low rates of airway and PA reintervention.

Recurrent Pulmonary Artery Interventions Following the Norwood Procedure Are Not Associated With Conduit Type

Given pulmonary artery interventions following the Norwood procedure can recur, the average number of occurrences per patient over time is likely more informative than the crude percentage of patients who required an intervention. Pulmonary artery intervention was defined as any surgical or catheter-based procedure after the Norwood procedure. The number of pulmonary artery interventions for patients with hypoplastic left heart syndrome were compared between patients with modified Blalock-Taussig Shunts (MBTS) and right ventricle-to-pulmonary artery conduits (RVPA) at a single institution from 2011 to 2018. The comparison was replicated using data from the Single Ventricle Reconstruction Trial (SVR), a nonoverlapping dataset. The mean number of pulmonary artery interventions per patient over time (mean cumulative function, MCF) is described using Nelson-Aalen estimates and compared using the pseudo-score test. The number of patients requiring intervention was compared using the chi-square test. Using our institutional dataset, the Norwood operation was performed on 117 patients (59 MBTS, 58 RVPA). In total, 73 patients had a pulmonary artery intervention, including 32 of 58 (55%) after MBTS and 41 of 59 (69%) after RVPA (P= 0.11). The MCF did not vary between cohorts (P = 0.55). Using the SVR trial dataset, 140 of 549 patients required pulmonary artery intervention, including 55 (21%) after MBTS and 85 (30%) after RVPA (P = 0.0090). The MCF did not vary between cohorts (P = 0.067). Although more patients with RVPA than MBTS require pulmonary artery interventions after the Norwood procedure, the MCFs are not different, which may be of greater importance to patients and families.