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Background: Pulmonary Atresia, Ventricular Deptal Defect, and Major Aortopulmonary Collateral Arteries (PA-VSD-MAPCAs) is a congenital cyanotic heart defect with poor prognosis. Due to its complex and highly variable anatomy, the best treatment plan is not clear. We aimed (1) to investigate the survival of PA-VSD-MAPCAs patients according to the underlying original anatomy and treatment strategy, and (2) to evaluate life expectancy between patients with or without severe hypoplastic native pulmonary arteries (NPAs) after surgical versus non-surgical treatment., Methods: A prospectively established database of 169 PA-VSD-MAPCAs patients treated and followed up at University Hospitals Leuven was accessed. Patients were divided into three groups according to the treatment strategy. Kaplan-Meier survival curves were plotted, and Log Rank tests were used for comparison., Results: The overall mean survival for patients with PA-VSD-MAPCAs was 38.5 years (95%-CI: 33.1-43.9). Patients with complete intracardiac repair had the longest mean survival of 43.8 years (95%-CI: 38.1-49.6) versus the other groups (p < 0.001). A longer mean event-free survival time was found in patients with normal, well-developed NPAs (p = 0.047). Finally, patients with poorly developed or absent NPAs had worse survival rates when a surgical approach was followed. Systemic-pulmonary shunt placement or unifocalisation had limited effect on prognosis in the absence of total repair (p = 0.167)., Conclusions: Patients with PA-VSD-MAPCAs who underwent complete intracardiac repair and/or with well-developed native pulmonary arteries had the best prognosis. Our analyzed data suggest that incomplete surgical repair resulted in survival rates comparable to those seen with a non-surgical approach., Competing Interests: Declaration of competing interest These authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation; no grant support; no conflict of interest., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Background: The benefit of additional pulmonary blood flow (APBF) in Fontan pathway remains controversial. The aim of the study was to analyze the systemic arterial saturation, ventricular and atrioventricular (AV) valve function, development of pulmonary arteries and postoperative course after Fontan operation in children with preserved or eliminated APBF between stages 2 and 3 of the Fontan pathway., Methods: A group of 180 consecutive children (median age: 2.92(1.83-16.83) years) with single ventricle underwent extracardiac conduit Fontan operation. The patients were retrospectively analyzed with respect to the presence of APBF between stages 2 and 3., Results: Preoperative echocardiography showed no differences in ventricular function (good - 105[95%] vs. 65[98.5%]; reduced - 9[5%] vs 1[1.5%]; p = 0.07) or AV valve function (p = 0.78). There was no difference in oxygen saturation at admission (p = 0.48), in preoperative blood gas analysis (p = 0.62), or at the discharge (p = 0.73). There was no difference in size of the left pulmonary artery (9.5(0-16.2) vs 10.6 (4.9-21.7) mm; p = 0.13), however the right pulmonary artery was significantly larger (11.2(6.5-19.6) vs. 12.8(7.8-2.5) mm; p = 0.048) in APBF group. In this group the mean pressure in the pulmonary artery was significantly higher as well (8[4-17.5] vs 9.5[4-17.3] mmHg; p = 0.03)., Conclusions: APBF does not result in higher oxygen saturation, both before and after Fontan completion. APBF can stimulate the growth of the pulmonary arteries and increase the pulmonary artery pressure. The presence of APBF does not affect the post-operative course after Fontan operation, however it can increase the need of AV valve reconstruction., (© 2024. The Author(s).)

Pulmonary arteriovenous malformations (PAVMs) universally develop in patients with single ventricle congenital heart disease. Single ventricle PAVMs have been recognized for over 50 years but remain poorly understood. To improve our understanding, we developed a surgical rat model of Glenn circulation and characterized PAVM physiology over multiple time points. We performed a left thoracotomy and end-to-end anastomosis of the left superior vena cava to the left pulmonary artery (unilateral Glenn), or sham surgical control. To assess PAVM physiology, we quantified intrapulmonary shunting using two independent methods (bubble echocardiography and fluorescent microsphere injection). Additionally, we performed arterial blood gas measurements to assess oxygenation and plethysmography to assess ventilation. We identified pathologic intrapulmonary shunting by bubble echocardiography as early as 2 weeks post-Glenn, and shunting continued at 2- and 6-months post-Glenn. Shunting also progressed over time, demonstrated by increased shunting of 10 μm microspheres at 6 months. Shunting was accompanied by mildly decreased oxygenation but no differences in ventilation. Our surgical animal model of unilateral Glenn circulation recreates the clinical condition of single ventricle PAVMs with early and progressive intrapulmonary shunting. This model is poised to characterize single ventricle PAVM pathophysiology and lead to mechanistic and therapeutic discovery., (© 2024 The Author(s). Physiological Reports published by Wiley Periodicals LLC on behalf of The Physiological Society and the American Physiological Society.)

Objective: In our study, we aim to explore the structural differences between the aortic root and the pulmonary artery to better understand the process of pulmonary autograft dilatation during the Ross procedure., Materials and Methods: We studied twenty human fetuses (aged 14-36 weeks of gestation) and four adults (one female and three males, aged 30-45 years, mean age = 37 ± 16 years). Samples of aortic root and pulmonary artery were obtained through dissection. Histological examinations, including hematoxylin-eosin, Masson's trichrome, and orcein staining, as well as immunohistochemical technique with caldesmon staining, were performed. Microscopic counting was conducted to assess the number of elastic laminae and smooth muscle cells in each arterial wall. Statistical analyses were performed using R software. Means and standard deviations were used to present central tendencies and data dispersion for elastic laminae and smooth muscle., Results: Significant histological differences were observed between the aortic root and pulmonary artery in both adults and fetuses. In fetuses, no difference was found between the two vessels in terms of elastic laminae (p = 0.26) and smooth muscle cells (p = 0.69). However, in adults, significant differences were found for elastic laminae (p < 0.001) and smooth muscle cells (p < 0.001) between the aorta and pulmonary artery., Conclusions: The microscopic vascular structure impacts the mechanical properties of the pulmonary autograft wall and explains its observed dilatation remote from the Ross procedure due to wall stresses related to systemic pressure., (© 2024. The Author(s).)

Neonates with congenital heart disease and ductal-dependent pulmonary blood flow (DD-PBF) require early intervention. Historically, this intervention was most often a surgical systemic-to-pulmonary shunt (SPS; eg, Blalock-Thomas-Taussig shunt). However, over the past two decades, an alternative to SPS has emerged in the form of transcatheter ductal artery stenting (DAS). While many reports have indicated safety and durability of the DAS approach, few studies compare outcomes between DAS and SPS. The reports that do exist are comprised primarily of small-cohort single-center reviews. Two multicenter retrospective studies suggest that DAS is associated with similar or superior survival compared with SPS. These studies offer the best evidence to-date, and yet both have important limitations. The authors describe herein the rationale and design of the COMPASS (COmparison of Methods for Pulmonary blood flow Augmentation: Shunt vs Stent [COMPASS]) Trial (NCT05268094, IDE G210212). The COMPASS Trial aims to randomize 236 neonates with DD-PBF to either DAS or SPS across approximately 27 pediatric centers in North America. The goal of this trial is to compare important clinical outcomes between DAS and SPS over the first year of life in a cohort of neonates balanced by randomization in order to assess whether one method of palliation demonstrates therapeutic superiority., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Unilateral chronic thromboembolism pulmonary disease (CTEPD) is very rare. There is limited information on the safety and efficacy of pulmonary endarterectomy (PEA) in this population. This study investigated the effectiveness of PEA in this unique disease., Methods: This multicentre study included patients with unilateral CTEPD from three referral centres in the United States, Turkey, and Iran. The patients' demographic information, 6-minute walk test distance (6MWD), New York Heart Association (NYHA) functional class, and haemodynamics were evaluated., Result: Of the 1,031 patients who had undergone PEA, 39 patients (3.7%) had pure unilateral involvement, of whom 28 were female (71.8%). There was a significant improvement in the mean pulmonary artery pressure (mPAP, 26 mmHg vs 21 mmHg; p=0.011) and pulmonary vascular resistance (PVR, 202 vs 136 dynes∗sec 1 ∗cm -5 ; p=0.014). There was also a significant improvement in NYHA functional class (p<0.001) and 6MWD (360 vs 409 m; p<0.001). In the nine patients with normal haemodynamic parameters at rest, there was no significant change in median 6MWD (448.5 vs 449 m; p=0.208), mPAP (19 mmHg vs 16.5 mmHg; p=0.397), and PVR (129 vs 84.5 dynes∗sec 1 ∗cm -5 ; p=0.128). The most common postoperative complication was ipsilateral pleural effusion. One patient needed extracorporeal membrane oxygenation support. No patient died within the 1-year follow up., Conclusion: Pulmonary endarterectomy is a safe and effective procedure for improving the symptoms and haemodynamic parameters of patients with unilateral CTEPH. Symptomatic patients with unilateral chronic thromboembolic disease are suitable for PEA., Competing Interests: Conflicts of Interest The authors declare no conflict of interest regarding this study., (Copyright © 2024 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Background: The repair of certain types of complex congenital cardiac defects may require a right ventricle-pulmonary artery (RV-PA) conduit. Using the Ozaki Aortic valve neocuspidization (AVNeo)technique, a valved RV-PA conduit was constructed with an Ozaki valve inside a Dacron graft. This study aims to evaluate the short-term outcome of the Ozaki valved RV-PA conduit., Material/method: A total of 22 patients received the Ozaki valved RV-PA conduit from November 2019 until December 2023. The median age was 12 years (interquartile range [IQR], 5.5-21), median body weight was 35 kg (IQR, 15.8-48.5). The conduit was used in 16 patients (72.7%) under 18 years of age. Indications for conduit placement included: anatomic repair of corrected transposition of the great arteries, ventricular septal defect/pulmonary stenosis, conduit replacement, pulmonary atresia with associated anomalies, pulmonary artery aneurysm with dysplastic pulmonary valve, tetralogy of Fallot with coronary artery crossing the right ventricular outflow tract, bioprosthetic pulmonary valve regurgitation, and rheumatic heart disease. Native pericardium was used for the Ozaki valve in 12 patients and bovine pericardium for 10 patients. Conduit sizes ranged from 18 mm to 30 mm., Result: The median intensive care unit stay was 4 (IQR, 2-6) days and the median hospital stay was 9 (IQR, 5.5-13.5) days. There were two perioperative mortalities (9.1%) both unrelated to the conduit. The median follow-up was 12.3 (IQR, 4.43-21.2) months. There was no infective endocarditis of the conduit. The median peak gradient across the conduit was 22 mm Hg (range 0-44 mm), and all were competent with trivial regurgitation on follow up., Conclusion: Creation of an Ozaki valved conduit is an attractive option due to low cost, reproducibility, and excellent hemodynamics. Longer-term studies are needed to confirm the durability., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: It is well-known that right ventricle-to-pulmonary artery homograft conduit durability is worse for smaller conduits and smaller/younger patients. However, there is limited literature on age and conduit-size specific outcomes, or on the role of conduit oversizing. Methods: Patients diagnosed with tetralogy of Fallot and major aortopulmonary collateral arteries undergoing right ventricular outflow tract (RVOT) reconstruction with a valved aortic homograft conduit from November 2001 through March 2023, at our institution were included. Conduits were grouped and evaluated by diameter, diameter Z-score, and patient age at implant. The primary time-related outcome was freedom from RVOT reintervention. Factors associated with freedom from time-related outcomes were assessed with univariable Cox regression analysis. Results: A total of 863 RVOT conduits were implanted in 722 patients. On multivariable analysis, younger age, male sex, Alagille syndrome, smaller diameter of the conduit, and smaller Z-score were associated with shorter freedom from reintervention. Among patients with smaller diameter conduits, larger Z-scores were associated with longer freedom from conduit reintervention ( P  < .001). Transcatheter interventions were commonly used to extend conduit lifespan across ages and conduit sizes. Conclusions: Larger conduit diameter, older age, and higher conduit Z-score were associated with longer freedom from reintervention in patients undergoing RVOT reconstruction in this cohort. Oversizing of conduits, even beyond a Z-score of 4, is generally appropriate., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Pulmonary artery banding (PAB) is a procedure mainly performed during the neonatal period as an initial stage to definitive palliative reconstruction, a scenario in which the criteria for banding adjustment are well defined. However, the indication for BAP in the adult is extraordinarily rare, even more in patients with single ventricle and unrepaired transposition of the great arteries (TGA), and there are no established criteria for banding adjustment. Due to the small number of these procedures, there is limited experience in their anesthetic management and complications. We describe a case of a 29-year-old patient diagnosed with a cyanotic congenital heart disease of double-inlet left ventricle with TGA and unrepaired mitral stenosis, who underwent to a hybrid procedure of PAB and enlargement of the communication between the two atria., (Publicado por Elsevier España, S.L.U.)

Background: Assessment of the pattern of the RV outflow tract Doppler provides insights into the hemodynamics of chronic thromboembolic pulmonary hypertension (CTEPH). We studied whether pre-operative assessment of timing of the pulmonary flow systolic notch by Doppler echocardiography is associated with long-term survival after pulmonary endarterectomy (PEA) for CTEPH., Methods: Fifty-nine out of 61 consecutive CETPH patients (mean age 53 ± 14 years, 34% male) whom underwent PEA between June 2002 and June 2005 were studied. Clinical, echocardiographic and hemodynamic variables were assessed pre-operatively and repeat echocardiography was performed 3 months after PEA. Notch ratio (NR) was assessed with pulsed Doppler and calculated as the time from onset of pulmonary flow until notch divided by the time from notch until end of pulmonary flow. Long-term follow-up was obtained between May 2021 and February 2022., Results: Pre-operative mean pulmonary artery pressure (mPAP) was 45 ± 15 mmHg and pulmonary vascular resistance (PVR) was 646 ± 454 dynes.s.cm-5. Echocardiography before PEA showed that 7 patients had no notch, 33 had a NR < 1.0 and 19 had a NR > 1.0. Three months after PEA, echocardiography revealed a significant decrease in sPAP in long-term survivors with a NR < 1.0 and a NR > 1.0, while a significant increase in TAPSE/sPAP was only observed in the NR < 1.0 group. Mean long-term clinical follow-up was 14 ± 6 years. NR was significantly different between survivors and non-survivors (0.73 ± 0.25 vs. 1.1 ± 0.44, p < 0.001) but no significant differences were observed in mPAP or PVR. Long-term survival at 14 years was significantly better in patients with a NR < 1.0 compared to patients with a NR > 1.0 (83% vs. 37%, p =  < 0.001)., Conclusion: Pre-operative assessment of NR is a predictor of long-term survival in CTEPH patients undergoing PEA, with low mortality risk in patients with NR < 1.0. Long-term survivors with a NR < 1.0 and NR > 1.0 had a significant decrease in sPAP after PEA. However, the TAPSE/sPAP only significantly increased in the NR < 1.0 group. In the NR < 1.0 group, the 6-min walk test increased significantly between pre-operative and at 1-year post-operative follow-up. NR is a simple echocardiographic parameter that can be used in clinical decision-making for PEA., (© 2024. The Author(s).)

This brief report reviews the clinical, procedural, and imaging data of 7 patients with p.Arg4810Lys variant of the ring finger protein 213 (RNF213) gene-related peripheral pulmonary arterial stenosis who underwent percutaneous transluminal pulmonary angioplasties (PTPAs) for demographics, clinical presentation, indications for angioplasty, and procedural and clinical outcomes. During median follow-up of 64.4 months since the first confirmed diagnosis, PTPA was performed for 62 segmental pulmonary arteries with 38 sessions of the procedure in 7 patients. Vascular stent placement because of resistance to balloon dilation and immediate elastic recoil was performed in 48 of 62 procedures (77%). Except for 1 death, 6 patients showed an improvement in dyspnea and 5 patients showed a decrease in mean pulmonary arterial pressure (mean, 55.5-42.7 mm Hg) and increase in 6-minute walk distance (mean, 415.5-484.3 m). Reperfusion edema occurred in 4 of 7 patients (57%), which was 6 of 38 sessions (16%)., (Copyright © 2024. Published by Elsevier Inc.)

Objectives: The aim of this study was to analyze the risk factors for acute events after systemic-to-pulmonary shunt (SPS) and to investigate the effectiveness of pulmonary blood flow regulation with a metal clip., Methods: The case histories of 116 patients (78 biventricular [BV] and 38 single ventricle [SV] physiology) who underwent SPS between 2010 and 2021 were retrospectively reviewed. Our strategy was to delay SPS until 1 month of age; pulmonary blood flow (PBF) regulation by partial clipping of the graft, if needed. Cases of aortic cross-clamping were excluded from this study., Results: CPB was used in 49 (42%) patients: the median age at SPS was 1 month (2 days to 16 years), and the sternotomy approach in 65. Discharge survival was 98.3% (114/116); hospital death occurred in 1.7% due to coronary ischemia. Inter-stage mortality occurred in 1.7% (shunt thrombosis, 1; pneumonia, 1). Pre-discharge acute events occurred in 7 patients (6.0%): thrombosis 3, pulmonary over-circulation 2, and coronary ischemia 2. Multiple logistic regression analysis revealed that pulmonary atresia with intact ventricular septum (PA/IVS) (p = 0.0253) was an independent risk factor for acute events. Partial clipping of the graft was performed in 24 patients (pulmonary atresia 15) and clip removal was performed by catheter intervention in 9 patients; no coronary ischemic events and graft injury occurred in these patients., Conclusion: Surgical outcomes after SPS were acceptable and metal clip regulation of pulmonary blood flow appears to be safe and effective. PA/IVS was still a significant risk factor for acute events., (© 2024. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.)

Pulmonary thrombotic arteriopathy (PTA) can be an incidental finding in lung resections performed for various indications. Historic studies largely examined PTA in autopsies. Thus, the prevalence in surgical samples, particularly in the modern era of lung cancer screening, is poorly defined. Detection of PTA in surgical samples may provide an opportunity for therapeutic intervention, but the impact of this finding on clinical management is unknown. We retrospectively examined consecutive lung surgical resections containing a report of incidental PTA between 2019 and 2022 in our institution. A retrospective chart review was performed to determine the history of systemic thromboembolism and clinical and radiographic follow-up. All slides were reviewed to morphologically characterize the vascular changes. Among 2930 pulmonary resections, 66 (2.3%) reportedly contained PTA. Twenty-four (36.4%) patients had a clinically recognized thromboembolic event either before or after surgical resection. Patients with clinically recognized thromboembolic disease were significantly more likely to have both acute and organized thrombi affecting large arteries. The presence of infarct, chronic hypertensive vasculopathy, or number of vessels with thrombi were not significantly associated with a clinically detected event. Reporting of incidental PTA led to clinical intervention in six patients and confirmed systemic thromboembolic disease in 2. Moreover, 2 patients with no further workup based on the incidental pathology findings subsequently developed pulmonary embolism. PTA is incidentally detected in 2.3% of surgical lung resections, and in two-thirds of cases, there is no clinical suspicion of thromboembolic disease. Pathologic reporting of PTA rarely led to clinical intervention, suggesting a need for improved communication of incidental pathology findings., Competing Interests: Conflicts of Interest and Source of Funding: L.M.S.: Consulting for Genentech, Lilly; Research support from Genentech, Bristol Myers Squibb. A.G. is supported by a fellowship grant from the Fonds de Recherche du Québec-Santé. For the remaining authors, none were declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Previous reports have demonstrated the safety and efficacy of performing early Kawashima procedure (KP). Despite this, more recent studies have shown that the average age at the time of operation remains greater than one year of age. We report our experience with performing KP at an earlier age than previously reported., Methods: A retrospective review was completed of patients undergoing KP at a single center (January 2000 to June 2020). Clinical outcomes were examined., Results: Initial palliation was performed in 11 out of 12 patients. Age and weight at time of KP were 8.25 months (6.4-9.7) and 7.7 kg (6.5-8.6). Intensive care unit stay was 3.16 days (1-12), overall length of stay was 9.5 days (3-22). There was one unplanned reoperation, and no mortality in the cohort. Discharge oxygen saturation was 88% (80%-98%). Seven patients developed pulmonary arteriovenous malformations (PAVMs) with six proceeding to hepatic vein incorporation (HVI). Interval time to development of PAVMs was 42.3 months (16-121). Four of the 12 patients were left with antegrade pulmonary blood flow (PBF) and three (75%) remain without PAVMs. Median follow-up was 10 years (1.5-22) with 11 of 12 (91.67%) survival for the cohort., Conclusions: The KP can be done at a younger age than previously reported with adequate early and late results. Most patients will go on to develop PAVMs and require HVI but leaving patients with some antegrade PBF is likely protective but will need further investigation to show definitive benefit., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: The European Chronic Thromboembolic Pulmonary Hypertension (CTEPH) registry, conducted between 2007 and 2012, reported the major impact of pulmonary endarterectomy (PEA) on the long-term survival of patients with CTEPH. Since then, 2 additional treatments for inoperable CTEPH have become available: balloon pulmonary angioplasty (BPA), and an approved oral drug therapy with the guanylate cyclase stimulator riociguat. The current registry aimed to evaluate the effect of these new therapeutic approaches in a worldwide context., Methods: Participation in this international global registry included 34 centers in 20 countries. Between February 2015 and September 2016, 1009 newly diagnosed, consecutive patients were included and followed until September 2019., Results: Overall, 605 patients (60%) underwent PEA and 185 (18%) underwent BPA; 76% of the 219 remaining patients not receiving mechanical intervention (ie, neither PEA nor BPA) were treated with pulmonary hypertension drugs. Of patients undergoing PEA and BPA, 38% and 78% also received drugs for pulmonary hypertension, respectively. Median age at diagnosis was higher in the BPA and No PEA/BPA groups than in the PEA group: 66 and 69, respectively, versus 60 years. Pulmonary vascular resistance (PVR) was similar in all groups, with an average of 643 dynes.s.cm -5 . During the observation period (>3 years; ≤5.6 years), death was reported in 7%, 11%, and 27% of patients treated by PEA and BPA, and those receiving no mechanical intervention ( P <0.001). In Kaplan-Meier analysis, 3-year survival was 94%, 92%, and 71% in the 3 groups, respectively. PEA 3-year survival improved by 5% from that observed between 2007 and 2012. There was no survival difference in patients receiving vitamin K antagonists and non-vitamin K oral anticoagulants ( P =0.756). In Cox regression, reduced mortality was associated with: PEA and BPA in the global cohort; history of venous thromboembolism and lower PVR in the PEA group; lower right atrial pressure in the BPA group; and use of pulmonary hypertension drugs, oxygen therapy, and lower right atrial pressure, as well as functional class in the group receiving no mechanical intervention., Conclusions: This second international CTEPH registry reveals important improvement in patient survival since the introduction of BPA and an approved drug for pulmonary hypertension. The type of anticoagulation regimen did not influence survival., Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT02656238., Competing Interests: M.D. reports research grants from Janssen, speaker and consultant fees from Altavant, Acceleron, AOP, Bayer, Ferrer, Gossamer, INARI, Janssen, United Therapeutics, and MSD outside the submitted work, and all paid to her institution. She was holder of a Janssen chair for pulmonary hypertension at the KU Leuven. J.P.Z. reports research grants from MSD and consultant fees from Ferrer, Gossamer, Janssen outside submitted work. S.G. reports speaker fees or consultant honoraria from Actelion/Janssen, Bayer AG, MSD, and Pfizer. D.P.J. reports speaker fees and consultancy fees from Janssen outside this submitted work. D.G.K. reports speaker fees, consultancy fees, or funding to attend meetings from Acceleron, Altavant, Ferrer, Janssen, Gossamer, MSD and United Therapeutics and research support from Ferrer and Janssen, all outside of the submitted work. N.H.K. reports receiving speaker fees from Bayer and Janssen; consultancy fees from Bayer, Janssen, Merck, Polarean, Pulnovo, and United Therapeutics; and research support from Altavant and Gossamer Bio. All disclosed fees and support are outside the submitted work. M.M.M. reports consultancy fees and royalties from Wexler Surgical and consultancy fees from Actelion/Janssen and Johnson & Johnson. H.M. reports research grants from Nippon Shinyaku; speaker and consultant fees from Bayer, Janssen, and MSD; speaker fees from Kaneka Medix, Mochida, Nippon Shinyaku, and Nipro, all outside of the submitted work. J.S.O.A. reports personal fees from Bayer and MSD. R.S.K. reports having received speaker fees from AOP Health, Actelion/Janssen, and MSD, all outside of the submitted work. GS reports receiving advisory board and speaker fees from Acceleron, Bayer, Janssen, MSD, and Merck. C.B.W. reports speaker fees or consultant honoraria from Actelion/Janssen, AOP Orphan Pharmaceuticals AG, Bayer AG, BTG, MSD, OrphaCare, and Pfizer. E.M. reports receiving consultancy or speaker fees from Actelion/Janssen, Bayer, and MSD. I.M.L. has relationships with drug companies including AOP Health, Actelion/Janssen, MSD, United Therapeutics, Pulnovo, Medtronic, Neutrolis, and Sanofi; in addition to being investigator in trials involving these companies, relationships include consultancy services, research grants, and membership of scientific advisory boards. The other authors report no conflicts.

Introduction: The indications for concominant tricuspid valve surgery in patients undergoing mitral valve surgery for rheumatic reasons are limited. The aim of our study was to investigate the effects of severe pulmonary hypertension and low TAPSE values on early-term mortality and morbidity in patients undergoing mitral valve replacement., Methods: The data of all patients who underwent mitral valve replacement between January 2013 and August 2020 were examined retrospectively. Patients were divided into 2 groups according to pulmonary artery pressure (PAP ≥ 50 and PAP < 50). The group with PAP > 50 was then divided into 2 subgroups according to TAPSE (1.5 ≥ or < 1.5) values. The early-term mortality and morbidity rates of these groups were compared., Results: Seventy-nine patients who underwent mitral valve replacement were included in the study. Fifty-four (68%) of them were female, and 25 (32%) were male. During the preoperative period, the TAPSE was 16.8 ± 3.0 mm, and the PAP was 52.1 ± 14.1 mmHg. There were 53 patients with PAP > 50 and 26 patients with PAP < 50. In the PAP > 50 group, the rates of tricuspid regurgitation (p < 0.001), blood transfusion (p < 0.001), intensive care unit stay (p < 0.001), need for CPAP (p = 0.043), reintubation (p = 0.048), acute renal failure (p = 0.028), and mortality (p = 0.026) were found to be significantly different., Conclusion: In conclusion, we believe that in patients with mitral valve pathology, early referral for surgical intervention, before the pulmonary pressures significantly increase and right ventricular function deteriorates, can enhance survival outcomes., (© 2024. The Author(s).)

Background: Patients can develop de novo malignancies following orthotopic heart transplantation. However, vascular tumors are not commonly described in this population., Case Presentation: We present a 69-year-old female with a history of orthotopic heart transplantation for chemotherapy-induced cardiomyopathy who developed an incidental pulmonary artery mass six years after her transplantation. Given concerns for malignancy, the patient underwent an operative excisional biopsy through a left anterior mini-thoracotomy with femoral artery and vein cannulation for cardiopulmonary bypass. The mass was determined to be a non-malignant vascular overgrowth with PIK3CA mutation., Conclusion: We present the case of an unusual pulmonary artery mass with PIK3CA mutation found in a post heart transplant patient. We were able to spare her the morbidity of a redo-sternotomy by excising the mass via a minimally invasive left anterior thoracotomy approach., (© 2024. The Author(s).)

Background: Nowadays, video-assisted thoracic surgery (VATS) lobectomy represents the treatment of choice for early-stage lung cancer. Over the years, different methods for VATS training have evolved. The aim of this study is to present an innovative beating-heart filled-vessel cadaveric model to simulate VATS lobectomies., Methods: Via selective cannulation of the cadaver heart, the pulmonary vessels were filled with a gel to improve their haptic feedback. An endotracheal tube with a balloon on its tip then allowed movement of the heart chambers, transmitting a minimum of flow to the pulmonary vessels. A simulated OR was created, using all instrumentation normally available during surgery on living patients, with trainees constantly mentored by experienced surgeons. At the end of each simulation, the participants were asked 5 questions on a scale of 1 to 10 to evaluate the effectiveness of the training method ("1" being ineffective and "10" being highly effective)., Results: Eight models were set up, each with a median time of 108 min and a cost of €1500. Overall, 50 surgeons were involved, of which 39 (78%) were consultants and 11 (22%) were residents (PGY 3-5). The median scores for the 5 questions were 8.5 (Q1; IQR 1-3 8-9), 8 (Q2; IQR 1-3 7-9), 9 (Q3; IQR 1-3 8-10), 9 (Q4; IQR 1-3 8-10), and 9 (Q5; IQR 1-3 8-10). Overall, the model was most appreciated by young trainees even though positive responses were also provided by senior surgeons., Conclusions: We introduce a new beating-heart filled-vessel cadaveric model to simulate VATS lobectomies. From this initial experience, the model is cost effective, smooth to develop, and realistic for VATS simulation., (© 2024. The Author(s).)

Various surgical approaches address complex heart disease with arch anomalies. Bilateral pulmonary artery banding (bPAB) is a strategy for critically ill patients with complex arch anomalies. Some reports argued the potential effect of bPAB on the growth of the left ventricular outflow tract (LVOT) during inter-stage after bPAB. This study aimed to analyze the LVOT growth for biventricular repair candidates with arch anomaly and systemic ventricular outflow tract (SVOT) for univentricular repair candidates with arch anomaly. This retrospective study analyzed 17 patients undergoing initial bPAB followed by arch repair. The Z-scores of LVOT and SVOT were compared between pre-bPAB and pre-arch repair. Patient characteristics, transthoracic echocardiogram data, and PAB circumferences were reviewed. The diameter of the minimum LVOT for biventricular repair (BVR) candidates, the pulmonary valve (neo-aortic valve, neo-AoV) and the pulmonary trunk (the neo-ascending aorta, neo-AAo) for univentricular repair (UVR) candidates, and the degree of aortic or neo-aortic insufficiency in each candidate was statistically analyzed. 17 patients were divided into the UVR candidates (group U) with 9 patients and the BVR candidates (group B) with 8 patients. In group B, the median value of the Z-score of the minimum LVOT increased from -3.2 (range: - 4.1 ~ - 1.0) at pre-PAB to -2.8 (range: - 3.6 ~ - 0.3) at pre-arch repair with a significant difference (p = 0.012). In group U, the median value of the Z-score of the neo-AoV increased from 0.5 (range: - 1.0 ~ 1.7) at pre-bPAB to 1.2 (range: 0.2 ~ 1.9) at pre-arch repair with a significant difference (p < 0.01). The median value of the Z-score of the neo-AAo was also increased from 3.1 (range: 1.5 ~ 4.6) to 4.3 (range: 3.1 ~ 5.9) with a significant difference (p = 0.028). The growth of the LVOT for BVR candidates and SVOT for UVR candidates during the inter-stage between bPAB and arch repair was observed. These results suggest the potential advantage of bPAB in surgical strategies. Further research is needed to validate these findings and refine surgical approaches., (© 2024. Springer Nature Japan KK, part of Springer Nature.)

Purpose:  Preoperative evaluation of pulmonary vascular and tracheal routes and variations is of great importance to the surgeon. Three-dimensional computed tomography bronchography and angiography (3D-CTBA) has evolved in recent years with the optimization of 3D reconstruction techniques and artificial intelligence. We aim to apply CT angiography and Exoview 3D reconstruction technology to assess patients' pulmonary arterial tree and its anatomical variants and to try to summarize a set of anatomical typing of the pulmonary arterial tree that is relatively easy and conducive to promoting teaching based on surgical habits of lobectomy., Methods:  A total of 358 patients hospitalized in the Department of Thoracic Surgery of the First Affiliated Hospital of Soochow University between July 2020 and August 2021 were included in this study. We carefully analyzed the site of emanation, alignment, and number of branches of the pulmonary artery according to a uniform classification method in conjunction with the two-dimensional CT images and transformed them into 3D reconstruction models., Results:  Different types of pulmonary artery were observed in 358 cases. We evaluated the complete pulmonary artery tree and counted the number and frequency of major arteries of the pulmonary based on the surgical habits of anatomical lobectomy., Conclusion:  The 3D-CTBA technique enables us to adequately assess the anatomy of the pulmonary arteries. Moreover, we provide a practical classification scheme of pulmonary arterial anatomical patterns based on lobectomy and 3D-CTBA. Our data can be used by clinicians in the teaching of pulmonary artery anatomy and the preoperative preparation for anatomical lobectomy., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

The last 40 years have shown dramatic improvement in outcomes for neonatal cardiac surgery for a spectrum of congenital heart disease diagnoses. With more patients surviving into adulthood, the long-term impact of initial management strategies of these patients has come into focus. This is particularly true for patients with pediatric heart valve disease. Many patients born with right ventricular to pulmonary artery (RVPA) discontinuity require placement of a valved conduit in the neonatal period. Valved conduit options are limited in this patient population due to patient size and inability to respond to somatic growth. Genetically engineered porcine (GEP) donors may offer a xenograft conduit alternative that can grow with the patient. We have developed a model utilizing GEP donor RVPA conduits placed in infantile nonhuman primate (NHP) recipients. Our recipient is maintained on single-drug immunosuppression and demonstrates no evidence of pulmonary valve insufficiency or stenosis during short-term follow-up. Further studies and long-term outcomes are necessary to determine the utility of this technology in human application., Competing Interests: Disclosure: J.D.C. and D.C.C. have a working affiliation and collaborate with eGenesis. The other authors have no conflicts of interest., (Copyright © ASAIO 2024.)

The current state of the literature for a bullet embolization to the heart and/or pulmonary vasculature indicates the occurrence is rare but could increase due to the rise in civilian low-kinetic-energy firearm acquisition and use. We present the case of an older teenage male who sustained a gunshot wound to the left flank. The bullet entered the iliac vein, travelled through the heart and lodged in the pulmonary artery. Successful relocation of the bullet to the internal iliac vein was performed by the interventional radiologist. This article highlights the advancement of interventional radiology as a successful non-invasive endovascular technique for bullet embolus removal., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To evaluate the correlation between clot burden and pulmonary artery pressures in patients undergoing suction thromboembolectomy for high-risk and intermediate-high-risk pulmonary embolism with secondary outcomes of 30-day mortality and intensive care unit (ICU) length of stay., Materials and Methods: Institutional review board (IRB) exemption was granted for this retrospective study. The charts of 120 consecutive patients who underwent mechanical thromboembolectomy using the Flowtriever system (Inari Medical, Irvine, California) between February 2020 and August 2022 were retrospectively reviewed, and the following data were collected: (a) preprocedural B-type natriuretic peptide and creatinine levels, (b) echocardiographic findings, (c) preprocedural and postprocedural pulmonary artery pressures, (d) ICU length of stay, and (e) 30-day mortality. Clot burden was scored using Qanadli and Miller indices and correlated with the clinical outcomes., Results: Of the 120 patients who underwent thromboembolectomy, pulmonary artery pressures and diagnostic-quality angiograms were available in 109 patients. In the 109 patients with adequate data, Qanadli, preprocedural Miller, and postprocedural Miller scores correlated with pulmonary artery pressures. Neither was independently associated with ICU length of stay. Freedom from 30-day mortality was 91%, and embolism-specific mortality was 92%. All-risk and high-risk patients who survived demonstrated a significantly lower preprocedural and postprocedural Miller score, respectively., Conclusions: Thrombus burden as measured by the Qanadli and Miller scores appeared to be correlated with pulmonary artery pressures. Furthermore, catheter-directed thromboembolectomy led to a reduction in Miller scores, which appeared to be correlated with a reduction in pulmonary pressures. In high-risk patients, a reduced postprocedural Miller score and pulmonary pressure demonstrated improved 30-day survival. Further investigation into the association between Miller scores and patient mortality is warranted to stratify patients who would benefit from emergency intervention., (Copyright © 2024 SIR. Published by Elsevier Inc. All rights reserved.)

Our objective is to describe our approach for a case of subacute on top of chronic thromboembolic disease and highlight operative learning points. Prior to incision, appropriate monitoring equipment, including an arterial line, Swan-Ganz catheter, brain saturation monitor and bispectral index monitor, is placed for proper management of haemodynamics. Sternotomy was performed, and the ascending aorta was cannulated, followed by bicaval cannulation for venous drainage. The patient was cooled to deep hypothermia. Once target temperature was achieved, circulatory arrest commenced. The left pulmonary artery was opened and the subacute component was removed without disrupting the plane of the chronic thromboembolic disease. An endarterectomy plane was then created proximally and dissected into the distal segmental/subsegmental branches. Once the endarterectomy was completed, the left pulmonary artery was closed. Circulation was resumed for end-organ perfusion. Once the right pulmonary artery was ready for dissection, circulatory arrest was restarted. Similarly to the left side, the subacute component was removed without disrupting the plane of the chronic thromboembolic disease. An endarterectomy plane was then created proximally and dissected into the distal segmental/subsegmental branches. Circulation was then resumed. Once rewarmed to 35.5°C, the patient was decannulated and the sternum was closed., (© The Author 2024. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

An aortopulmonary window is a rare disorder that occurs in 0.1–0.2% of all congenital disorders. Our patient was a 1-month-old boy weighing 4180 g. The patient had heart failure associated with an aortopulmonary window. We used 3-dimensional computer graphic software (Viewtify, SCIEMENT) for diagnosis based on DICOM data from contrast-enhanced computed tomography. This made it easy to identify anatomical landmarks and findings and select the most suitable approach. We avoided stenosis of the right pulmonary artery and aorta. We encountered a case of an aortopulmonary window in which 3-dimensional computer graphic software was helpful in selecting the surgical technique. We report this case using 3-dimensional computer graphic images and present a review of the literature., (© The Author 2024. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: This study aims to present the midterm outcomes of surgical correction of the anomalous left coronary artery from the pulmonary artery (ALCAPA)., Methods: This is a retrospective study of patients undergoing anomalous origin of the LCA from the pulmonary artery repair between 2010 and 2019., Results: Forty-nine patients (20 boys and 29 girls) underwent ALCAPA repair. Patients were divided into two groups based on their age at ALCAPA repair: infant (< 1 year of age: n = 24) and non-infant ( ≧ 1 year of age: n = 25). Median age at time of repair was 23 months(7-60months). LCA reimplantation was performed in 47 patients, and Takeuchi repair was performed in 2 patients. Hospital mortality in the infant group was 8.2% (4 of 49). Infant group had significantly lower LVEF in pre-operation (p < 0.05), but there was not significantly different between the two groups about LVEF at discharge. The median follow-up duration was 43(18-85)months. The freedom from reoperation was not significantly different between two groups (infants vs. non-infants: 68.8% vs. 87.5% at 10 years; p = 0.096)., Conclusions: Surgical treatment of ALCAPA had an excellent early and midterm outcomes. Left ventricular dysfunction in pre-operation was the main risk of mortality in-hospital. The freedom from reoperation did not differ significantly between infant group and non-infant group., (© 2024. The Author(s).)

A 2-month-old female (3.4 kg, 50 cm) with Down syndrome and left-to-right shunting congenital heart defects underwent an unsuccessful transcatheter ductal closure, followed by bilateral implantation of manually modified microvascular plugs (MVP-9Q) from Medtronic (Minneapolis, MN, USA), used as pulmonary flow restrictors. Post-procedure, she developed febrile respiratory distress, leading to admission to the intensive care unit. Despite initial improvement, she was readmitted with respiratory syncytial virus infection, progressing to bilateral pneumonic consolidation. Subsequent complications included pulmonary artery aneurysmal dilatation attributed to pulmonary flow restrictors, which necessitated urgent surgery two months after their implantation. The surgery involved removing the pulmonary flow restrictors and repairing the injuries to the pulmonary artery, followed by management with extracorporeal support and targeted antibiotics. The patient recovered over 12 months., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The patient was an 86-year-old woman. She was brought to the emergency department because of chest pain and respiratory distress symptoms that did not improve. 12-lead electrocardiogram, echocardiography, and blood tests were negative for acute coronary syndrome, but a chest contrast computed tomography( CT) -scan showed pericardial effusion and a coronary artery aneurysm 20 mm in diameter, and punctured pericardial effusion revealed bloody fluid. A cardiac catheterization was performed. He underwent emergent surgery after being diagnosed as having a ruptured coronary aneurysm associated with a coronary pulmonary artery fistula. The aneurysm was dissected using cardiopulmonary bypass, and the coronary artery-pulmonary artery fistula was closed. The patient had a good postoperative course and was discharged in good condition. There have been few reports of rupture when the aneurysm diameter is less than 30 mm. Regardless of the size of the aneurysm, it has a risk of rupture and it is important to consider surgery.

A 60-year-old male patient suffered from frequent episodes of atrial tachycardia (AT), after the index procedure of catheter ablation for paroxysmal atrial fibrillation. During the repeat procedure, the activation map showed that the earliest activation site was located at the roof of left atrium. Multiple ablations at the earliest activation site on the roof failed to terminate the AT; however, ablation within the pulmonary artery at an adjacent anatomical site successfully eliminated the AT, even without recording distinct near-field potential., (© 2024 Wiley Periodicals LLC.)

The replacement of the superior vena cava and thoracic outlet vessels for thoracic malignancies often becomes necessary for radical oncological surgery. The pulmonary artery can be directly infiltrated by the tumor or affected by metastatic hilar lymph nodes. In some cases, it must be resected and reconstructed to achieve oncological radicality and/or avoid pneumonectomy. This study reflects a single-surgeon, retrospective experience spanning 6 years (2017-2023). We reviewed data from patients undergoing early anticoagulant therapy after superior vena cava or thoracic outlet vessels bypass and from patients undergoing early antiaggregation therapy following pulmonary artery reconstruction or resection. This series comprises 41 patients treated by a single surgeon. Fourteen patients underwent superior vena cava and thoracic outlet vessel procedures. Among these, eight patients received superior vena cava replacement (six for thymic malignancies and two for lung cancer), and six patients underwent jugular and subclavian artery/vein resection or replacement (all six had sarcomas). There was one death due to respiratory failure, not associated with bleeding or bypass closure. Additionally, there was one graft closure in a patient with severe coagulopathy and three instances of hemothorax (two patients had undiagnosed complex coagulopathies not evident in pre-operative routine blood tests). Following bleeding incidents, anticoagulation was initiated the next day in one case and based on hematological indications in the two coagulopathic patients. In the pulmonary artery series, 27 patients were involved: 20 underwent direct suture after tangential resection, and 7 received pericardial patch reconstruction. Only one case experienced bleeding necessitating redo-surgery. All these patients received early and chronic antiaggregation therapy after pulmonary artery reconstruction. We conclude that major thoracic oncological vascular surgery is safe and feasible with appropriate technical skills. However, achieving optimal results requires integration with correct early anticoagulant therapy or antiaggregation to maintain the patency of bypasses/grafts and prevent life-threatening risks associated with closure of the "new vessels.", (© 2024. Italian Society of Surgery (SIC).)

Objectives: 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., Methods: 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., Results: 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)., Conclusions: 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.

Background: Stent grafts (SG) have gained the highest level of evidence for superior management over angioplasty of arteriovenous graft (AVG) venous outflow stenosis, which increases their use in hemodialysis patients. Migration to the heart and lungs is the most feared complication of SG of the venous system., Method: We present a previously unreported case of asymptomatic migration of a 10-cm-long Viabahn lodged in a U-shaped configuration in the main left pulmonary artery after AVG intervention in a 50 year old male hemodialysis patient., Results: Through a single femoral access percutaneous retrieval of migrated SG was accomplished. Technique of coaxial buddy snaring technique is described., Conclusion: Our technique may expand the endovascular likelihood of successful stent removal., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: A coronary artery fistula is an abnormal connection between one or more coronary arteries and a cardiac chamber or great vessel, often discovered incidentally through cardiac imaging. Although coronary artery fistulas are typically asymptomatic during the first two decades of life, particularly when small, they can become clinically significant over time., Case Presentation: We present the case of a 71-year-old female patient with a history of exertional dyspnea. Diagnostic coronary angiography revealed a significant coronary artery fistula originating from the proximal right coronary artery and draining into the pulmonary artery trunk. Given the patient's symptoms and the anatomical features of the fistula, she was successfully treated with transcutaneous closure using a liquid embolic agent (Onyx)., Conclusion: Although surgical intervention has historically been the primary treatment for CAF, minimally invasive techniques such as transcutaneous closure are proving to be effective alternatives., Competing Interests: Déclaration de liens d'intérêt Les auteurs déclarent ne pas avoir de lien d'intérêt avec cet article., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Competing Interests: Disclosures The author has no conflicts of interest to disclose.

Competing Interests: Declaration of competing interest None.

Background: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus., Methods: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair., Results: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11)., Conclusions: Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation., Competing Interests: Disclosures Athar M. Qureshi reports a relationship with W. L. Gore & Associates, Inc. that includes: consulting or advisory; with Medtronic PLC that includes: consulting or advisory; and with B. Braun Medical Inc that includes: consulting or advisory. The other authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Surgical implantation of a right ventricle to pulmonary artery (RV-PA) conduit is an important component of congenital heart disease (CHD) surgery, but with limited durability, leading to re-intervention. The present single-center, retrospective, cohort study reports the results of surgically implanted RV-PA conduits in a consecutive series of children and adults with CHD. Patients with CHD referred for RV-PA conduit surgical implantation (from October 1997 to January 2022) were included. The primary outcome was conduit failure, defined as a peak gradient above 64 mm Hg, severe regurgitation, or the need for conduit-related interventions. Longitudinal echocardiographic studies were available for mixed-effects linear regression analysis. A total of 252 patients were initially included; 149 patients were eligible for follow-up data collection. After a median follow-up time of 49 months, the primary study end point occurred in 44 (29%) patients. A multivariable Cox regression model identified adult age (>18 years) at implantation and pulmonary homograft implantation as protective factors (hazard ratio 0.11, 95% confidence interval [CI] 0.02 to 0.47 and hazard ratio 0.34, 95% CI 0.16 to 0.74, respectively). Fever within 7 days of surgical conduit implantation was a risk factor for early (within 24 months) failure (odds ratio 4.29, 95% CI 1.41 to 13.01). Long-term use of oral anticoagulants was independently associated with slower progression of peak echocardiographic gradient across the conduits (mixed-effects linear regression p = 0.027). In patients with CHD, the rate of failure of surgically implanted RV-PA conduits is higher in children and after nonhomograft conduit implantation. Early fever after surgery is a strong risk factor for early failure. Long-term anticoagulation seems to exert a protective effect., Competing Interests: Declaration of competing interest The authors have no competing interests to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: The outcomes of single-ventricle palliation in unbalanced atrioventricular canal defect with coarctation of aorta (uAVC+CoA) have not been well studied. Systemic ventricle outflow tract obstruction has a propensity to develop in these patients after aortic arch repair with pulmonary artery banding (arch-PAB), which may adversely affect survival and Fontan candidacy., Methods: A retrospective review was performed of patients who underwent single-ventricle palliation for uAVC+CoA from 2000 to 2022. Patients were divided into 2 groups based on initial palliation: (1) arch-PAB and (2) Norwood procedure. Demographic and clinical characteristics were analyzed and compared along with survival data., Results: Stage 1 palliation for uAVC+CoA was performed in 41 patients. Arch-PAB was performed in 14 infants and Norwood in 27 infants. Arch-PAB patients had more chromosomal abnormalities (28.6 vs 7.4%, P < .009) and less severe systemic ventricle outflow tract obstruction on baseline echocardiogram (0.0 vs 70.4%, P < .001). Survival to stage 3 palliation was lower for the arch-PAB group (28.6% vs 66.6%, P = .02). Arch-PAB remained a significant risk factor for mortality (hazard ratio, 2.93; 95% CI, 1.05-8.53; P = .04) after adjusting for chromosomal abnormalities and atrioventricular valve regurgitation. After arch-PAB, systemic ventricle outflow tract obstruction was diagnosed in 13 of 14 patients. Echocardiography underestimated the degree of outflow tract obstruction in 10 of 13 arch-PAB patients., Conclusions: Arch-PAB has worse outcomes than Norwood for uAVC+CoA. Systemic ventricle outflow tract obstruction develops in almost all patients after arch-PAB. Outflow tract obstruction is underestimated by the echocardiogram and requires a high index of suspicion, along with advanced imaging, to ensure timely diagnosis and management., Competing Interests: Disclosures Fawwaz R. Shaw is a member of The Annals of Thoracic Surgery Editorial Board. The other authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The optimal approach for supravalvar right ventricular outflow tract obstruction(RVOTO) after arterial switch operation(ASO) is unclear. The results of percutaneous balloon dilatation have been variable. We report the results of simultaneous double balloon dilation for RVOTO after ASO. Sixteen patients (1.3(0.7-3.8) years; 9.8(8.1-15.1) kgs underwent the procedure at 14(8-44.5) months after ASO. Salient technical features included placement of balloons over stiff guide-wires positioned in both branch pulmonary arteries to enable dilation of the distal-most main pulmonary artery (MPA) with high inflation pressures (~ 12-14 atmospheres) and short inflation-deflation cycles. Effective balloon size was based on the PA annulus or MPA distal to the narrowing. The final balloon: narrowest segment diameter ratio was 2.7. Following dilation, the right ventricle to systemic systolic pressure ratio decreased from 0.9 ± 0.18 to 0.52 ± 0.16 (p  < 0.001) and mean RVOT gradient from 78 ± 18 to 34 ± 13.9 mmHg (p < 0.001). Narrowest diameter improved from 5.4 ± 2.2 to 9.2 ± 2.2 mm. There were no major complications. Two patients with inadequate relief (final RV-systemic ratios: 1.03 and 0.7) were referred for surgery. At median follow up of 9 months, IQR 7-22, range 5-73, others are free of re interventions with median RVOT gradient of 42, IQR 27-49, range 21-55 mmHg. The immediate and short-term follow up results of double balloon dilatation for supravalvar RVOTO is encouraging and may avoid the need for repeat surgery in the majority of patients. Further follow up is needed to determine the long-term durability of the results., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: This research evaluates the effect of balloon pulmonary angioplasty (BPA) on cardiac electrophysiological changes in patients with chronic thromboembolic pulmonary hypertension (CTEPH)., Methods: Involving a retrospective analysis of 39 CTEPH patients (average age 61 ± 11), who had at least two BPAs and paired ECGs pre- and post-surgery, we examined changes in ECG indicators of right ventricular hypertrophy and their correlation with hemodynamic results., Results: BPA yielded marked improvements in cardiac function and hemodynamics. ECG parameters, specifically the Lewis criteria and Butler-Leggett score, correlated strongly with hemodynamics and were predictive of a mean pulmonary arterial pressure (mPAP) ≥ 35mmHg. Notably, QRS complex axis normalization was observed in 25 patients, with 14 fully normalizing (range - 30° to + 90°). The qR pattern in V1 vanished in 9 cases, and 75% of the patients in qR pattern in V 1 group had QRS complex electrical axis completely returned to normal range. The qR V1 group had higher mPAP and pulmonary vascular resistance (PVR), and lower cardiac output and index compared to the non-qR V1 group, alongside a higher Butler-Leggett score., Conclusions: BPA enhances cardiac function and hemodynamics in CTEPH patients, with certain ECG measures such as Lewis criteria and Butler-Leggett score reflecting the severity of hemodynamic impairment. The reversal of QRS axis deviation and the disappearance of the qR pattern in lead V 1 may serve as valuable indicators for assessing post-BPA satisfaction in CTEPH patients., (© 2024. The Author(s).)

In pulmonary segmentectomy, the dominant pulmonary arteries are traditionally divided at the fissure. However, this approach sometimes leads to inadvertent injury to the pulmonary artery and prolonged air leak when the fissure is fused. To overcome these problems, by taking advantage of the good visualization provided by robotic surgery, we have adopted the lung-inverted approach without fissure dissection for segmentectomy. We have successfully performed a robotic left S6 and S1+2c segmentectomy using the lung-inverted approach. In addition to a good postoperative course, the console time was 57 minutes, which was considered relatively short. This approach may have contributed to the short operating time because it did not require repeated rotation of the lung. A clear understanding of the anatomy was required to perform this approach properly, because each branch of the pulmonary vessels and bronchi was treated inverted at the hilum. A preoperative 3-dimensional computed tomography broncho-angiographic scan was considered useful because it allowed us to recognize the relative positions of the dominant pulmonary vessels, the bronchi and other structures that were preserved., (© The Author 2024. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

BACKGROUND Pulmonary artery sling (PAS) is an anatomical vascular anomaly due to the origin of the left pulmonary artery from the right pulmonary artery, which runs posteriorly between the esophagus and trachea, resulting in compression of adjacent structures. Accurate evaluation for malformation of the pulmonary artery and severity of airway obstruction is essential to surgical strategy. This report presents the diagnosis and surgical management of pulmonary artery sling in a 12-year-old boy. CASE REPORT A 12-year-old boy had chest tightness and wheezing after exercise for 6 years. He was diagnosed with PSA based on findings from imaging tests, demonstrating the left pulmonary artery originated from the middle of the right pulmonary artery and the tracheal carina was located at the site of the T6 thoracic vertebra. The main bronchus and esophagus were compressed by the left pulmonary artery due to its ectopic origin. Then, after comprehensive preoperative assessment, the patient underwent surgical repair of PAS. CONCLUSIONS This report highlights the importance of pulmonary artery sling diagnosis, imaging, and surgical planning, and the role of a multidisciplinary team in preoperative and postoperative patient management. An individualized strategy based on the preoperative assessment, intraoperative coordination among cardiologists, surgeons, and perfusionists, and careful postoperative management are the core elements for successful PAS repair.

Pulmonary artery rupture is a rare complication of right heart catheterization characterized by a rapid clinical deterioration and high mortality rate. We present the case of an 89-year-old woman with severe symptomatic aortic stenosis who underwent cardiac catheterization prior to aortic valve replacement. The patient had acute cardiopulmonary deterioration due to pulmonary artery rupture at the time of right heart catheterization, that was successfully sealed by balloon tamponade., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Displaced posterior segmental bronchus (B 2 ) accompanied by anomalous pulmonary vessels is a very rare condition. There is a risk of unexpected injuries to bronchi and blood vessels when patients with such anomalies undergo surgery for lung cancer, especially thoracoscopic surgery., Methods: We reported a case of thoracoscopic right upper lobectomy in a patient with a displaced B 2 and pulmonary vascular variation., Results: A 74-year-old woman was admitted to our hospital with a 2.2 cm × 2.1 cm nodule in the right lung. Three-dimensional computed tomography (3D-CT) revealed the combined apical/anterior segmental branch (B 1 + 3 ) taken off the beginning of the right main bronchus (RMB), at the level of the carina. The displaced B 2 taken off the end of the RMB. The anomalous central vein (CV), which passed between B 2 and B 1 + 3 , ran dorsal to the main pulmonary artery (MPA) and directly into the left atrium. The patient consequently underwent uniportal thoracoscopic right upper lobectomy and mediastinal lymph node dissection. The intraoperative findings were completely consistent with 3D-CT., Conclusions: This paper reports a case of a displaced B 2 combined with right upper pulmonary vessels malformation. Under the guidance of 3D-CT, the right upper lobectomy was successfully completed by single hole thoracoscopic surgery.

Pulmonary artery banding (PAB) is used to restrict pulmonary blood flow in select patients with large left-to-right intracardiac shunts or unrestrictive pulmonary blood flow prior to eventual surgical repair or palliation. More recently, surgical placement of an internal or intraluminal PAB (IPAB) has been used to restrict pulmonary circulation. Here we present two patients who underwent balloon angioplasty of the IPAB to treat cyanosis and improve pulmonary blood flow.