Pulmonary Branches Research Articles

Evaluation of an engineered vascular graft exhibiting somatic growth in lambs to model repair of absent pulmonary artery branch

BackgroundGrowth is the holy grail of tissue implants in pediatrics. No vascular graft currently in use for surgical repairs of congenital heart defects has somatic growth capacity.MethodsBiologically-engineered grafts (6 mm) grown from donor ovine fibroblasts in a sacrificial fibrin gel were implanted into the left pulmonary branch of 3-month old lambs for 3, 6, and 18 months. A control group of Propaten® PTFE grafts was implanted for 6 months.ResultsThe engineered grafts exhibit extensive site-appropriate recellularization after only 3 months and near-normal increase of diameter from the preimplant value of 6 mm to 12.9 mm and also a doubling of length from 6.0 mm to 13.0 mm at 6 months (n = 3) associated with apparent somatic graft growth (collagen content increase of 265% over 18-month, n = 2), along with excellent hemodynamics and no calcification, in contrast to the Propaten® grafts. The left-right flow distribution is nearly 50–50 for the engineered grafts at 6 months (n = 3) compared to about 20–80 for the Propaten® grafts (n = 3), which have less than one-half the diameter, a 6-fold higher pressure gradient, and stunted vascular development downstream of the graft. The engineered grafts exhibit a stable diameter over months 12–18 when the lambs become adult sheep (n = 2).ConclusionsThis study supports the use of these regenerative grafts with somatic growth capacity for clinical trial in patients born with a unilateral absent pulmonary artery branch, and it shows their potential for improving development of the downstream pulmonary vasculature.

Electrical vagus nerve stimulation is a promising approach to reducing pulmonary complications after an esophagectomy: an experimental rodent model.

After esophagectomy, an imbalanced inflammatory response increases the risk of postoperative morbidity. The vagus nerve modulates local and systemic inflammatory responses, but its pulmonary branches are transected during esophagectomy as part of the oncological resection, which may account for the high incidence of postoperative (pulmonary) complications. This study investigated the effect of electrical vagus nerve stimulation (VNS) on lipopolysaccharide (LPS)-induced lung injury in rats. Rats (n = 60) were randomly assigned to a non-vagotomy or cervical vagotomy group, with VNS or without (NOSTIM). There were four non-vagotomy groups: NOSTIM and bilateral VNS with 100, 50, or 10 µA. The four vagotomy groups were NOSTIM and VNS with fixed amplitude (50 µA) bilaterally before (VNS-50-before) or after bilateral vagotomy (VNS-50-after), or unilaterally (left) before ipsilateral vagotomy (VNS-50-unilaterally). LPS was administered intratracheally after surgery. Pulmonary function, pro-inflammatory cytokines in serum, broncho-alveolar lavage fluid (BALF), and histopathological lung injury (LIS) were assessed 180min post-procedure. In non-vagotomized rats, neutrophil influx in BALF following intra-tracheal LPS (mean 30 [± 23]; P = 0.075) and LIS (mean 0.342 [± 0.067]; P = 0.142) were similar after VNS-100, compared with NOSTIM. VNS-50 reduced neutrophil influx (23 [± 19]; P = 0.024) and LIS (0.316 [± 0.093]; P = 0.043). VNS-10 reduced neutrophil influx (15 [± 6]; P = 0.009), while LIS (0.331 [± 0.053]; P = 0.088) was similar. In vagotomized rats, neutrophil influx (52 [± 37]; P = 0.818) and LIS (0.407 [SD ± 0.037]; P = 0.895) in VNS-50-before were similar compared with NOSTIM, as well as in VNS-50-after (neutrophils 30 [± 26]; P = 0.090 and LIS 0.344 [± 0.053]; P = 0.073). In contrast, VNS-50-unilaterally reduced neutrophil influx (26 [± 10]; P = 0.050) and LIS (0.296 [± 0.065]; P = 0.005). Systemic levels of cytokines TNF-α and IL-6 were undetectable in all groups. Pulmonary function was not statistically significantly affected. In conclusion,VNS limited influx of neutrophils in lungs in non-vagotomized rats and may attenuate LIS. Unilateral VNS attenuated lung injury even after ipsilateral vagotomy. This effect was absent for bilateral VNS before and after bilateral vagotomy. It is suggested that the effect of VNS is dependent on (partially) intact vagus nerves and that the level of the vagotomy during esophagectomy may influence postoperative pulmonary outcomes.

Complex Pulmonary Artery Rehabilitation in Children with Alagille Syndrome: An Early Single-Center Experience of a Successful Collaborative Work.

Objective: In this paper, we share our single-center experience of successful multidisciplinary management of patients with Alagille syndrome. In addition, we aim to highlight the need for an Alagille program for effectively managing these patients, in general, and particularly peripheral pulmonary artery stenosis associated with this syndrome. Study Design: This is a retrospective review of six children with Alagille syndrome and advanced liver involvement who underwent pulmonary artery reconstruction between 2021 and 2022. Cardiac diagnosis, co-existing liver disease burdens, management approach, and short-term outcomes were analyzed. Results: All the patients underwent one-stage extensive bilateral branch pulmonary rehabilitation. Concomitant procedures included repair of tetralogy of Fallot in one patient and repair of supravalvar pulmonary artery stenosis in two. One patient had balloon pulmonary branch angioplasty before surgery. In all patients, there was a decrease in right ventricular systolic pressure post-operatively. Three patients underwent liver transplantation for pre-existing liver dysfunction. At a median 3-year follow-up, all the patients were alive with their right ventricular systolic pressure less than half of their systemic systolic pressure. One patient underwent balloon angioplasty due to new and recurrent left pulmonary artery stenosis 13 months after surgery. Conclusion: Pulmonary arteries can be successfully rehabilitated surgically in the presence of complex branch disease. Patients with advanced liver disease can undergo successful complex pulmonary artery reconstruction, which can facilitate their future liver transplantation course. A multidisciplinary team approach is a key for successful management of Alagille patients.

Catheter-assisted local lysis therapy for submassive pulmonary embolism

Pulmonary embolism is the third most common cardiovascular disease. Interventional treatment options as an alternative to systemic lysis therapy of hemodynamically stable, submassive pulmonary embolisms have received an unprecedented boost in innovation in recent years. The treatment options are heterogeneous and can be roughly divided into local thrombolysis and local thrombectomy. For years in our center we have been carrying out catheter-assisted, locoregional lysis therapy with side-hole lysis catheters and acumulative dose per pulmonary branch of 10 mg alteplase over 15 h for hemodynamically stable, submassive pulmonary emboli. The aim of this retrospective study was to review this therapeutic concept and to collect data on clinical endpoints and possible complications. The study included data from 01/2018-03/2023. For this purpose, the patients were selected based on the OPS codes (8.838.60 and 1‑276.0), and the data was collected using the medical records. Biometric data, data on previous illnesses and vital parameters, laboratory chemistry data, CT diagnostic data, echocardiographic data, data on drug treatment and data on complications were collected anonymously. There was asignificant reduction in the strain on the right heart. Peripheral oxygen saturation also improved significantly and heart rate decreased significantly. The complication rate remained low and was almost exclusively limited to access-related problems. Catheter-assisted, locoregional lysis therapy is asafe and effective treatment method for submassive pulmonary embolism.

Presentation of 5 patients with abnormal origin of the pulmonary branch artery in the ascending aorta (hemitroncus arteriosus). Rare cardiac patholoy.

Presentation of 5 patients with abnormal origin of the pulmonary branch artery in the ascending aorta (hemitroncus arteriosus). Rare cardiac patholoy.

Effect of Preserving the Pulmonary Branch of Vagus Nerve on Postoperative Cough in Patients with Stage I Peripheral Lung Adenocarcinoma

Cough is one of the main complications after pulmonary surgery, which seriously affects the postoperative quality of life. Preserving the pulmonary branch of vagus nerve may reduce the incidence of postoperative cough. Therefore, the aim of this study was to investigate whether preserving the pulmonary branch of the vagus nerve could reduce the incidence of postoperative chronic cough in patients with stage I peripheral lung adenocarcinoma. A total of 125 patients who underwent single-port thoracoscopic radical resection for lung cancer in the Department of Thoracic Surgery, The First Affiliated Hospital of University of Science and Technology of China from June 2022 to June 2023 were retrospectively selected, and divided into two groups according to whether the vagopulmonary branch was preserved during the operation, namely, the vagopulmonary branch group (n=61) and the traditional group (n=64). The general clinical data, perioperative conditions, lymph node dissection, Mandarin Chinese version of The Leicester Cough Questionnaire (LCQ-MC) scores before and 8 weeks after operation were recorded in the two groups. Both the two groups were divided into tamponade group and non-tamponade group according to whether autologous fat or gelatin sponge was tamponade after lymph node dissection. LCQ-MC scores and postoperative chronic cough of both groups were calculated. The LCQ-MC score of the traditional group was significantly lower than that of the vagopulmonary branch group in physiological, psychological, social and total scores at 8 weeks after surgery, and the difference was statistically significant (P<0.05). There were more cough patients in the traditional group than the vagopulmonary branch group at 8 weeks after surgery, with significant difference (P=0.006). Subgroup analysis was conducted separately for the vagopulmonary branch group and the traditional group. Among the patients in the vagopulmonary branch group and the traditional group, the LCQ-MC scores of the non-tamponade group 8 weeks after surgery were lower than those of the tamponade group (P<0.05). There were more patients with cough in the group 8 weeks after surgery than in the tamponade group (P=0.001, P=0.024). For patients with stage I peripheral lung adenocarcinoma, the preservation of the pulmonary branch of vagus nerve is safe and effective, which can reduce the incidence of postoperative chronic cough and improve the postoperative quality of life of the patients.

Preservation of the pulmonary branches of the vagus nerve during three-dimensional thoracoscopic radical resection of lung cancer: a retrospective study

BackgroundIn this study, we investigated the effect of preservation of the pulmonary branches of the vagus nerve during systematic dissection of mediastinal lymph nodes, when performing radical resection of lung cancer, on the postoperative complication rate.MethodsThe clinical data for 80 patients who underwent three-dimensional thoracoscopic radical resection of lung cancer in the Department of Thoracic Surgery at Huizhou Municipal Central Hospital between 2020 and 2022 were analyzed. The patients were divided into two groups according to whether the pulmonary branches of the vagus nerve were retained during intraoperative carinal lymph node dissection. The operation time, time until first postoperative defecation, duration for which a chest tube was needed, total chest drainage volume, average pain intensity during the first 5 postoperative days, incidence of postoperative pneumonia, and postoperative length of stay were compared between the two groups.ResultsThere was no statistically significant difference in histological staging or in time until first postoperative defecation between the two groups (p > 0.05). However, there were significant differences in operation time, the duration for which a chest tube was needed, total chest drainage volume, average pain intensity during the first 5 postoperative days, white blood cell count and procalcitonin level on postoperative days 1 and 5, and postoperative length of stay between the two groups (p < 0.05).ConclusionPreserving the pulmonary branches of the vagus nerve during carinal lymph node dissection when performing three-dimensional thoracoscopic radical resection of lung cancer can reduce the risk of postoperative complications.

Impact of preoperative left pulmonary artery stenting on the Fontan procedure: a retrospective multicentre study.

Left pulmonary artery (LPA) or bifurcation stenoses at Fontan palliation can be very challenging to treat and may also require cardioplegia and aortic transection. Moreover, the low pressure of Fontan circulation and the bulkiness of the aorta increase the risk of a patch angioplasty collapse. Pre-Fontan LPA stenting of stenotic LPAs overcomes those drawbacks therefore the present study aimed to evaluate its advantageous impact on Fontan surgery. A multicentre retrospective analysis was performed on 304 consecutive Fontan patients. The study population was divided into 2 groups (LPA stented, n = 62 vs not stented, n = 242); pre-and postoperative data were compared. LPA-stented patients had a higher prevalence of systemic right ventricle (P = 0.01), hypoplastic left heart syndrome (P = 0.042), complex neonatal palliations (Norwood/Damus-Kaye-Stansel) and surgical LPA patch repair at Glenn (P < 0.001). No differences were found in cross-clamp rates, early (P = 0.29) and late survival (94.6% vs 98.4, P = 0.2) or complications (P = 0.14). Complex palliations on ascending aorta/aortic arch (P = 0.013) and surgical LPA repair at Glenn (P < 0.001) proved to be risk factors for LPA stenting before Fontan at multivariable analysis. The LPA-stented group showed similar outcomes in terms of survival and complications rate compared to patients without LPA stenosis; however, they significantly differ in their higher preoperative risk profile and in their more complex anatomy. Complex neonatal palliations involving ascending aorta or aortic arch may increase the risk of pulmonary branches stenosis requiring stenting; therefore, preoperative stenting of LPA stenoses could help to reduce the surgical risk of complex Fontan procedure by avoiding the need for cross-clamp or complex mediastinal dissections to perform a high-risk surgical repair.

Right ventricle to pulmonary artery conduit: a comparison of long-term graft-related events between bovine jugular vein conduit, aortic homograft, and porcine-valved conduits.

The optimal conduit for right ventricle to pulmonary artery (RV-PA) reconstruction does not exist. Reinterventions are common due to conduit stenosis and endocarditis. Tailoring conduit choice according to patients' characteristics could improve the outcomes. The study aimed to compare graft-related events (infective endocarditis, transcatheter pulmonary valve replacement (PVR), transcatheter conduit dilatation, surgical conduit replacement, and transcatheter pulmonary branch intervention for RV-PA reconstruction using bovine jugular vein, aortic homograft, and porcine-valved conduits. In a retrospective cohort study, 155 patients with 193 procedures that were done in King Faisal Specialist Hospital and Research Center-Jeddah (KFSHRC JED) for implanting RV-PA conduits from 1999 to 2021 were included. The patients were grouped according to the type of conduit into 3 groups; Group 1 (n=153) received bovine jugular vein (BJVs) grafts, Group 2 (n=29) received aortic homografts, Group 3 (n=11) received porcine-valved conduits. Factors associated with graft-related events were evaluated using stepwise Cox regression analysis. Patients with BJVs were significantly younger than those with porcine-valved conduits 3 (P=0.009). The weight of BJVs patients was significantly lower than homografts (P=0.002) and porcine-valved conduits patients 3 (P<0.001). The conduit size was as expected significantly lower in BJVs patients than patients who received porcine-valved conduits (P<0.001) and patients who received aortic homografts (P<0.001). There was no difference between Group 2 and 3 (P=0.084). Operative mortality occurred in 13 patients: 12 (7.84%) with BJV conduit and 1 (9.09%) with porcine valved conduit (P=0.351). Male gender [odds ratio (OR): 10.04; 95% confidence interval (CI): 1.28-78.86; P=0.028] and smaller conduit size (OR: 0.78; 95% CI: 0.61-0.99; P=0.048) were associated with increased operative mortality. Freedom from graft-related events at 5 and 10 years was 67% and 52% in BJVs patients, 74% and 36% in patients who received aortic homografts, and 53% in patients who received porcine-valved conduits. Factors associated with increased graft-related events were male gender (HR: 1.58; 95% CI: 1.004-2.50, P=0.048) and younger age (HR: 0.995; 95% CI: 0.991-0.999, P=0.041). RV-PA reconstruction was associated with low mortality, unrelated to the conduit type. Reinterventions for graft-related events were common. The durability and graft-related events might be comparable among BJV grafts, aortic homografts, and porcine-valved conduits. Factors associated with increased graft-related events in this study were male gender and younger age.

Pulmonary artery thrombosis. Clinical cases

Accumulating evidence supports the need to consider pulmonary artery thrombosis the separate thrombotic complication, which requires its own treatment algorithms.&#x0D; The aim of the work is to demonstrate the pulmonary artery thrombosis clinical aspects and the effect of the standard thrombotic prophylactic algorithms with clinical examples.&#x0D; Clinical cases of patients with pulmonary artery thrombosis are presented. Pulmonary artery thrombosis is considered to be the thrombotic obstruction of the pulmonary artery branches in the absence of venous and cardiac chambers thrombosis.&#x0D; Patient S. with COVID-19 and 75% of lung involvement was treated in the accordance with the actual COVID-19 treatment recommendations. Enoxaparin sodium 6000 IE once a day followed by the increasing dosage of 8000 IE twice a day was used. To prevent exacerbation tocilizumab was infused, and systemic thrombolytic therapy was performed. On the 6th day after systemic thrombolytic therapy patient died. Examination revealed thrombi in the pulmonary artery branches.&#x0D; Patient P. with continued growth of cerebral left frontoparietal region glioblastoma was hospitalized. Surgical tumor removal was performed a year ago. Conservative therapy, dexamethasone injections and anticoagulant prophylaxis (enoxaparin sodium 4000 IE once a day) were prescribed. After the patient death thrombi were found in the pulmonary artery segmental branches.&#x0D; Outpatient D. had a history of four episodes of dyspnea during the acute respiratory diseases. Computed tomography performed at the time of the third and fourth episodes revealed thrombi in the pulmonary artery segmental branches. The fourth episode occured despite anticoagulant prophylaxis with rivaroxaban (10 mg once a day). Patient was recommended to switch to low molecular weight heparins in case of any disease. There have been no recurrent episodes over the past 8 months.&#x0D; Distinctive features of pulmonary artery thrombosis pathogenesis determine the development of the complication despite anticoagulant prophylaxis. Preventive effect of anti-inflammatory drugs on the pulmonary artery thrombosis development has not been demonstrated.

Hemorragia digestiva como primeira manifestação na síndrome de Alagille - Relato de caso

Introduction: The aim of this report is to describe a case of gastrointestinal bleeding that started at 1 month of age in a patient with Alagille Syndrome. Case report: Patient started recurrent gastrointestinal bleeding, leading to hypovolemic shock in the second month of life. During the investigation, cholestasis and incoagulable INR were evidenced. The facial stigmata, associated with the posterior embryotoxon in the ophthalmologic examination and transthoracic echocardiogram with stenosis of the pulmonary branches and patent foramen ovale, suggested the hypothesis of Alagille Syndrome, later confirmed by compatible liver biopsy and genetic testing with a mutation in the JAGGED1 gene. After administration of vitamin K, the patient evolved with normalization of the coagulogram, without new episodes of gastrointestinal bleeding. At follow-up, replacement of fat-soluble vitamins and treatment with ursodeoxycholic acid were initiated. Discussion: Reports in the literature describe bleeding episodes in patients with Alagille Syndrome, however, the main site of bleeding is the central nervous system. There is no description of bleeding in the gastrointestinal tract as the initial and neonatal manifestation of the syndrome. The case reported is an uncommon form of presentation and shows the importance of diagnostic suspicion in cases of neonatal cholestasis with gastrointestinal bleeding, allowing for adequate treatment and improved prognosis.

Clinical Course of TGA After Arterial Switch Operation in the Current Era

BackgroundThe number of patients with an arterial switch operation (ASO) for transposition of the great arteries (TGA) is steadily growing; limited information is available regarding the clinical course in the current era. ObjectivesThe purpose was to describe clinical outcome late after ASO in a national cohort, including survival, rates of (re-)interventions, and clinical events. MethodsA total of 1,061 TGA-ASO patients (median age 10.7 years [IQR: 2.0-18.2 years]) from a nationwide prospective registry with a median follow-up of 8.0 years (IQR: 5.4-8.8 years) were included. Using an analysis with age as the primary time scale, cumulative incidence of survival, (re)interventions, and clinical events were determined. ResultsAt the age of 35 years, late survival was 93% (95% CI: 88%-98%). The cumulative re-intervention rate at the right ventricular outflow tract and pulmonary branches was 36% (95% CI: 31%-41%). Other cumulative re-intervention rates at 35 years were on the left ventricular outflow tract (neo-aortic root and valve) 16% (95% CI: 10%-22%), aortic arch 9% (95% CI: 5%-13%), and coronary arteries 3% (95% CI: 1%-6%). Furthermore, 11% (95% CI: 6%-16%) of the patients required electrophysiological interventions. Clinical events, including heart failure, endocarditis, and myocardial infarction occurred in 8% (95% CI: 5%-11%). Independent risk factors for any (re-)intervention were TGA morphological subtype (Taussig-Bing double outlet right ventricle [HR: 4.9, 95% CI: 2.9-8.1]) and previous pulmonary artery banding (HR: 1.6, 95% CI: 1.0-2.2). ConclusionsTGA-ASO patients have an excellent survival. However, their clinical course is characterized by an ongoing need for (re-)interventions, especially on the right ventricular outflow tract and the left ventricular outflow tract indicating a strict lifelong surveillance, also in adulthood.

Decreased clinical performance in TGA-ASO patients after RVOT re-interventions; multicenter European collaboration

Abstract Background In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT reinterventions. However, data on the impact of reinterventions on clinical performance is scarce. Therefore, we aimed to assess in TGA-ASO patients the cardiopulmonary exercise capacity (CPET) and right ventricular function stratified for the number of RVOT reinterventions. Method This multicenter observational study was performed in adult (≥16 years) TGA-ASO patients from the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). All patients underwent a comprehensive clinical workup including CPET and echocardiography, including right ventriculo-arterial (VA) coupling (defined as tricuspid annular plane systolic excursion (TAPSE) / right ventricular systolic pressure (RVSP) ratio). Patients were stratified for the number of reinterventions at the RVOT, including re-interventions at the pulmonary valve, main pulmonary artery and pulmonary branches. Association between RVOT re-intervention(s) and functional parameters, including predicted (%) peak oxygen uptake (peak VO2), TAPSE, right ventricle (RV) S’ and VA coupling of the RV were analyzed with linear regression. Results A total of 449 TGA patients were included in this study, with a mean age of 25.4 ± 5.6 years and median time until ASO was 8 days [IQR 6-15]. Eleven (2.5%) patients with previous re-interventions at the left ventricular outflow tract (LVOT) or aortic arch were excluded from the analysis. Twenty-six percent of the included patients had a ventricular septal defect (VSD) and 1.6% were diagnosed with double outlet right ventricle (DORV). In the total cohort, 13% of the patients had 1 previous RVOT re-intervention, 8% had 2 RVOT re-interventions and 5% had 3 RVOT re-interventions. Patients had echocardiographic examination and 55% underwent CPET. Predicted VO2peak was significantly reduced in patients with 1 RVOT re-intervention (72%), 2 RVOT re-interventions (69%) and 3 RVOT re-interventions (71%) compared to patients without any-reintervention (77%) (p=0.0268). Right VA coupling was a significant predictor (p=0.017) for reduced hemodynamic performance in patients with 1 RVOT re-intervention (0,50), 2 RVOT re-interventions (0,51) and 3 RVOT re-interventions (0,26). Conclusion In TGA-ASO patients, RVOT reinterventions were associated with a reduced clinical performance. VA coupling of the right ventricle was the most sensitive marker for hemodynamic performance in TGA-ASO patients.

Do Patients with Antiphospholipid Syndrome Have More Significant Venous Thromboembolic Clot Burden?: A Single-Center Retrospective Analysis

Background: Antiphospholipid syndrome (APS) is a prothrombotic condition defined by arterial or venous thrombotic events and/or obstetrical morbidity in the presence of persistently positive antiphospholipid antibodies (aPL). Venous thromboembolic disease (VTE), comprised mostly of deep vein thrombosis (DVT) and pulmonary embolism (PE), is the initial presenting clinical manifestation in approximately 35% of APS diagnoses. VTE is a heterogenous disease and can present with varying degrees of clot burden. Lower extremity DVT can present with thrombus in the iliofemoral, femoropopliteal, and/or distal calf veins; pulmonary embolism can present with thrombus in the main pulmonary arteries, lobar branches, segmental, and/or subsegmental branches. In both DVT and PE, more proximal clot burden portends higher morbidity and mortality. While VTE is a commonly encountered clinical presentation, the decision regarding whom to test for APS is often challenging. Current guidelines recommend that clinicians consider testing for APS in patients with unexplained venous or arterial thrombotic events, especially young patients, or when a patient has one or more specific adverse outcomes related to pregnancy. To date, no data exist to suggest that patients with APS are more likely to present with more extensive venous thromboembolic clot burden as compared to patients without APS. Purpose: To assess clot burden in patients with a co-diagnosis of VTE and APS in comparison to patients with VTE without APS to determine if clot burden should be considered when deciding whom to test for APS. Methods: This retrospective cohort study included adult patients with a history of VTE who had also been tested for APS within a multihospital academic healthcare system from December 1, 2019 through January 31, 2022. All patients in the study had a history of VTE and were considered to be positive for APS if they had persistently positive aPL and/or lupus anticoagulant in accordance with the revised Sapporo APS Classification Criteria. Patients were excluded if they had confounding provoking risk factors for thrombosis. Patients were stratified as APS and non-APS. Significant clot burden was defined as PE involving the main and/or lobar pulmonary arteries or DVT involving the iliofemoral veins. Assessment of clot burden was performed by review of radiology reports of the sentinel clotting event.Patients were further stratified based on age, BMI, insurance status, severity of APS (triple-positive APS versus non-triple-positive APS), and type of APS (primary versus secondary). Exploratory analysis of the patient groups was performed. Summary statistics are presented as percentages for categorical data and median with interquartile range for quantitative data. Chi square test was used to compare percentages and t-test was used to compare means. Univariate and multivariate regression models were used to analyze the predictors of significant clot burden. Results: We identified 748 patients with a history of VTE who had also been tested for APS. The study population was comprised of 53.1% females with a median age of 59 (IQR 46-69). A majority of the patients were Caucasian (85%). APS was detected in 75 (10%) patients. Significant clot burden was present in 29 (38.7%) APS patients and 269 (40.0%) non-APS patients (OR 0.95, 95% CI 0.58-1.56; P = 0.85). On univariate analysis, age (OR 1.01, 95% CI 1.00-1.02; P = 0.02) and being uninsured (OR 9.2, 95% CI 1.10-76.10; P = 0.04) were associated with more significant clot burden. No predictors for significant clot burden were found on multivariable analysis. Triple positive APS (OR 0.83, 95% CI 0.16-4.21; P = 0.82) and primary APS (OR 0.72, 95% CI 0.15-3.45; P = 0.68) were not associated with more significant clot burden. Conclusions: In this retrospective cohort of 748 patients with a history of VTE, the presence of antiphospholipid syndrome was not associated with more significant VTE clot burden as compared to non-APS patients. Based on these data, VTE clot burden may not be a reliable predictor for the presence of APS. Study limitations include selection bias, retrospective design, and lack of precise volumetric measurement of blood clots.

Evaluating AI-powered text-to-image generators for anatomical illustration: A comparative study.

Medical illustration, which involves the creation of visual representations of anatomy, has long been an essential tool for medical professionals and educators. The integration of AI and medical illustration has the potential to revolutionize the field of anatomy education, providing highly accurate, customizable images. The authors evaluated three AI-powered text-to-image generators in producing anatomical illustrations of the human skulls, heart, and brain. The generators were assessed for their accurate depiction of foramina, suture lines, coronary arteries, aortic and pulmonary trunk branching, gyri, sulci, and the relationship between the cerebellum and temporal lobes. None of the generators produced illustrations with comprehensive anatomical details. Foramina, such as the mental and supraorbital foramina, were frequently omitted, and suture lines were inaccurately represented. The illustrations of the heart failed to indicate proper coronary artery origins, and the branching of the aorta and pulmonary trunk was often incorrect. Brain illustrations lacked accurate gyri and sulci depiction, and the relationship between the cerebellum and temporal lobes remained unclear. Although AI generators tended toward esoteric imagery, they exhibited significant speed and cost advantages over human illustrators. However, improving their accuracy necessitates augmenting the training databases with anatomically correct images. The study emphasizes the ongoing role of human medical illustrators, especially in ensuring the provision of accurate and accessible illustrations.

Prenatal assessment of pulmonary vasculature development in fetuses with congenital diaphragmatic hernia: A literature review.

Pathophysiological studies have shown that pulmonary vascular development is impaired in fetuses with a congenital diaphragmatic hernia (CDH), leading to a simplified vascular tree and increased vascular resistance. Multiple studies have described prenatal ultrasound parameters for the assessment of the pulmonary vasculature, but none of these parameters are used in daily clinical practice. We provide a comprehensive review of the literature published between January 1990 and February 2022 describing these parameters, and aim to explain the clinical relevance of these parameters from what is known from pathophysiological studies. Prenatal detection of a smaller diameter of the contralateral (i.e. contralateral to the diaphragmatic defect) first branch of the pulmonary artery (PA), higher pulsatility indices (PI), higher peak early diastolic reverse flow values, and a lower vascularization index seem of added value for the prediction of survival and, to a lesser extent, morbidity. Integration within the routine evaluation is complicated by the lack of uniformity of the methods used. To address the main components of the pathophysiological changes, we recommend future prenatal studies in CDH with a focus on PI values, PA diameters and pulmonary vascular branching.

Outcomes of manually modified microvascular plugs to pulmonary flow restrictors in various congenital heart lesions.

The development of microvascular plugs (MVPs) has enabled novel transcatheter deliverable endoluminal pulmonary flow restrictors (PFRs) with the potential to treat newborns and infants with life-threatening congenital heart diseases (CHDs) in a minimally invasive manner. We present our experience to evaluate the efficacy of this concept in controlling pulmonary blood flow in various CHDs. Retrospective clinical data review of patients with CHD and pulmonary over-circulation who received bilateral PFRs percutaneously. Twenty-eight PFRs (7 MVP-5Q, 12 MVP-7Q, and 9 MVP-9Q) were finally implanted in 14 patients with a median age of 1.6 months (IQR, 0.9-2.3) and a median weight of 3.1 Kg (IQR, 2.7-3.6). Nine patients had large intra-cardiac left-to-right shunts (including 3 with fatal trisomy and palliative programs), 2 had borderline left ventricles, 2 had Taussig-Bing anomaly, and one had a hypoplastic left heart. Four patients had concomitant ductal stenting. Two MVP-5Qs were snare-removed and upsized to MVP-7Q. Patients experienced a significant drop in oxygen saturation and Qp/Qs. All patients were discharged from the ICU after a median of 3.5 days (IQR, 2-5.8) postoperative. Five patients had routine inter-stage catheterization and no device embolization or pulmonary branch distortion was seen. Fourteen (50%) PFRs were surgically explanted uneventfully on a median of 4.3 months (IQR, 1.2-6) post-implantation during biventricular repair in 6 patients and stage-2 palliation in one patient. The latter died 1 month post-operative from severe sepsis. Four patients are scheduled for surgical PFR removal and biventricular repair. Two patients with trisomy 18 died at 1 and 6.8 months post-procedure from non-cardiac causes. One patient with trisomy 13 is alive at 2.7 months post-procedure. It is feasible to bespoke MVPs and implant them as effective PFRs in various CHDs. This approach enables staged left ventricular recruitment, comprehensive stage-2 or biventricular repair with lower risk by postponing surgeries to later infancy. Device explantation is uneventful, and the outcomes afterward are promising.

4D Flow Cardiovascular Magnetic Resonance-Derived Flow Biomarkers for Early Detection of Flow Abnormalities: A Multicohort New Zealand Based Study

Four-dimensional (4D) flow cardiovascular magnetic resonance (4D flow CMR) imaging has evolved as a versatile imaging technique for the assessment of cardiovascular three-dimensional time resolved haemodynamics. This study aimed to examine the utility and metrics derived from 4D flow CMR in different patient cohorts. Controls (n=26), adult tetralogy of Fallot (rToF; n=14), and left ventricular hypertrophy (LVH; n=10) patients were scanned at the Centre of Advanced MRI, University of Auckland. Quantification of flow and examination of between-group differences were performed. Intraclass correlation coefficients (ICC) were used to correlate 4D and 2D CMR. Forward flow rates averaged in the pulmonary veins were significantly greater in LVH than controls (79.11±54.24 mL/sec vs 32.58±35.98 mL/sec, respectively, p<0.05), as was the pulmonary venous atrial reversal peak velocity (–0.44±0.23 m/sec vs –0.13±0.27 m/sec, respectively, p<0.05). Mitral indices of net flow and peak velocity expressed no significant between-group differences. In the rToF group, quantification of pulmonary artery and branches demonstrated a good correlation with 2D CMR. Greatest correlation was between pulmonary artery backward flow (difference of s–2.50±6.91 mL/cycle, ICC 0.96). In relation to pulmonary valve regurgitation categorisation, 1 of 14 cases were differently categorised using 4D flow (ICC 0.91). Quantification of flow with 4D flow CMR was feasible, with a single acquisition introducing an array of flow metrics that may be valuable for the evaluation of various conditions. Larger scale investigations of 4D flow CMR are required, with wider-scale application in routine clinical workflows.

Can CT-Based Arterial and Venous Morphological Markers of Chronic Obstructive Pulmonary Disease Explain Pulmonary Vascular Remodeling?

We analyzed changes in quantitative pulmonary artery and vein parameters to investigate pulmonary vascular remodeling characteristics in chronic obstructive pulmonary disease (COPD) patients. This retrospective study recruited healthy volunteers and COPD patients. Participants undergoing standard-of-care pulmonary function testing (PFT) and computed tomography (CT) evaluations were classified into five groups: normal and Global Initiative for Chronic Obstructive Lung Disease (GOLD) grades 1-4. Artery and vein analyses (volumes, numbers, densities, and fractions) were performed using artificial intelligence. Among 139 subjects (136 men; mean age, 64years±8 [SD]) with GOLD grade 1 (n=13), grade 2 (n=49), grade 3 (n=42), grade 4 (n=17) and control subjects (n=18) enrolled, differences in arterial volumes (BV5-10, BV10+, pulmonary arterial volume) and venous densities (BV5 density, BV10+ density, pulmonary venous density, pulmonary venous branch density) among control and GOLD grades 1-4 were statistically significant (P<.05). Higher pulmonary arterial volumes and lower number were observed with more advanced COPD. The number and volumes of pulmonary veins were lower in GOLD grades 2 and 3 than in GOLD grade 1 but higher in GOLD grade 4 than in GOLD grade 3. The numbers and volumes of pulmonary arteries and veins showed varying positive correlations (γ=0.18-0.96, P<.05). Pulmonary vascular densities were mildly to moderately correlated with PFT results (γ=0.236-0.495, P<.05) and were moderately negatively correlated with the emphysema percentage (γ=-0.591 to -0.315, P<.05). Patients with COPD exhibited pulmonary vascular remodeling, which occurred in the arteries at the early grade of COPD and in the veins at the late grade. CT-based quantitative analysis of pulmonary vasculature may become an imaging marker for early diagnosis and assessment of COPD severity.

C32 CONSTRICTIVE PERICARDITIS WITH PLATYPNEA ORTHODEOXIA

Abstract A 56–year–old woman is admitted to our centre for biventricular heart failure. In anamnesis she reports a submandibular phlegmon complicated by pleuropericarditis at the age of 13 years requiring repeated pericardiocentesis. In the last three years she has been hospitalised twice for ascites and discharged with a diagnosis of idiopathic liver cirrhosis. On admission she presented with peripheral oedema, ascites and hippocratic fingers; oxygen saturation is 80% in room air. On blood tests creatinine, transaminases, albumin, coagulation and NT–proBNP are normal, bilirubin is elevated. An echocardiography is performed showing a calcific spicola impinging the free wall of the right ventricle producing endocavitary obstruction; diffusely thickened pericardium, septal bounce, annulus reversus and expiratory reflux in the suprahepatic veins. Biventricular contractile function is normal and no significant valvulopathy is present. CT scan confirms the presence of diffuse pericardial calcifications, small liver and irregular profile with large ascitic effusion. Decongestive therapy is started with weight loss of 22 kg and regression of oedema but severe hypoxia persists with evidence of platypnea–orthodeoxia syndrome. An echocardiography is repeated showing passage of microbubbles in the left atrium from the sixth beat onwards, compatible with intrapulmonary shunt. A large arteriovenous malformation (AVM) of the right lower pulmonary branch is found at CT angiography, which is also revealed by targeted invasive angiography. Cardiac catheterisation and cardiac MRI confirmed the diagnosis of constrictive pericarditis. Percutaneous closure of the AVM with complete resolution of the hypoxia and subsequent pericardiectomy is performed. The patient is discharged on day XXVII with normal saturation on room air, in good haemodynamic compensation and with normal liver stasis parameters. In conclusion, constrictive pericarditis is a rare cause of heart failure and to our knowledge this is the second case described reporting a pulmonary AVM secondary to constrictive pericarditis.