Interventional management of aspergillus-related pulmonary artery pseudoaneurysm and hemoptysis.

Pulmonary artery pseudoaneurysms are rare but life-threatening vascular abnormalities. Only less than 10% hemoptysis cases are of pulmonary artery origin while most cases arise from bronchial arteries. When diagnosed, they are mostly found to accompany pre-existing cardiovascular disease, infection, (i.e. Tuberculosis or Aspergillosis), vasculitis, trauma and/or neoplastic conditions. There are rare reports of pulmonary artery pseudoaneurysms being caused by direct extension of invasive fungal infections. We report a case of a rapidly growing pulmonary artery pseudoaneurysm in a 20-year-old female with lymphoma involving the lung and mediastinum. The patient was hospitalized with complications, including hemoptysis in the setting of Aspergillus Pneumonia and respiratory failure requiring intubation. Interventional Radiology was consulted after multiple bronchoscopic interventions failed to stabilize the bleeding. Patient then underwent embolization of the left subsegmental pulmonary artery pseudoaneurysm, with resolution of hemoptysis the next day.

The retrotracheal left pulmonary artery: A rare vascular malformation.

The retrotracheal left pulmonary artery, also known as "left pulmonary artery sling," is a rare vascular malformation in which the left pulmonary artery (LPA) arises from the posterior aspect of the right pulmonary artery (RPA) and reaches the left pulmonary hilum by passing between the trachea and the esophagus, giving rise to the appearance of a sling, hence the name "sling." This vascular anomaly can be associated with other cardiac malformations or abnormalities of the tracheobronchial tree.We present the case of a 4-month-old female infant who presented with laryngeal stridor. She underwent a chest X-ray, which was normal, but thoracic CT angiography revealed an aberrant LPA originating from RPA with a retrotracheal course. This case underscores the critical role of imaging in confirming the diagnosis and guiding patient management.

Response to "Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome".

The article "Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome" offers valuable insights into Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome. This letter commends the study for its relevance and potential to improve patient care but highlights several limitations. Notably, the study overlooks the impact of Balloon angioplasty is a useful treatment for recoarctation in individuals with HLHS, but it frequently necessitates further operations. Following Norwood or Fontan palliation, stenting is safe, adaptable, and can be used to control uncommon neointimal growth. The risk of pulmonary artery stenosis may be decreased by the hemi-Fontan the technique. BA is better for recoarctation because it has fewer risks, whereas endovascular stenting helps high-risk patients avoid surgery.

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

OBJECTIVE: We aimed to compare the pulmonary artery (PA) growth between infants with univentricular hearts who underwent a ductus stenting (DS) and those who received a systemic to pulmonary shunt (SPS) as an initial palliation. METHODS: All infants with ductal-dependent pulmonary blood flow who underwent initial palliation with either a DS or SPS between 2009 and 2022 in our institution were reviewed. PA development was compared between the groups using the PA index and the symmetry index. RESULTS: A total of 130 patients were evaluated including 49 patients after DS and 81 after SPS. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19. At stage II palliation, PA index (p=0.926), right PA index (p=0.692), left PA index (p=0.297) and the symmetry index (p=0.650) were similar between the groups. At stage III Fontan completion, PA index (p=0.115), right PA index (p=0.868), and the symmetry index (p=0.144) were similar between the groups. However, left PA index (60 vs. 74mm2/m2, p=0.015) was lower, and the incidence of veno-venous collaterals (24.2 vs. 7.8%, p=0.036) was higher in DS group compared to SPS group. Freedom from PA interventions between stage II and III palliation was lower in DS group compared to SPS group (p=0.009). CONCLUSIONS: In patients after DS in infants with univentricular heart, freedom from PA interventions after stage II was lower and the left PA index was smaller, compared to those after SPS. Long-term outcomes after the Fontan procedure should be addressed in patients after DS.

Metastatic Small-Cell Lung Carcinoma Infiltrating the Heart: A Rare Case Diagnosed Using Imaging Data.

Small-cell lung carcinoma is a high-grade aggressive disease that occurs most commonly in bronchial lung cancer, and metastasis to the heart is extremely rare. The diagnosis of metastatic small-cell lung carcinoma infiltrating the heart remains challenging because of the variability of its clinical presentation. Hereinafter, we reported a case of a 41-year-old man who suffered from small-cell lung cancer that invaded the pulmonary artery. The patient presented with chest tightness, dry cough, and deterioration in exercise tolerance and diagnosed at his imaging data, who had an uneventful recovery after surgical resection of the masses.

Comparison of Safety and Efficacy of Aspiration Thrombectomy and Ultrasound Accelerated Thrombolysis for Management of Pulmonary Embolism: A Systematic Review and Meta-Analysis.

PURPOSE: To compare the safety and efficacy of mechanical thrombectomy (MT) and ultrasound-accelerated thrombolysis (USAT) in pulmonary embolism (PE) management by performing a systematic review of the literature. MATERIALS AND METHODS: The PubMed database was searched to identify articles on Inari's FlowTriever and Penumbra's Indigo mechanical thrombectomy devices (Group A) and the Ekos Endovascular system (Group B). Outcomes variables analyzed include pre- and post-procedure RV/LV ratio, pre- and post-procedure pulmonary artery pressure, hospital length of stay, technical success, specific complications, and mortality rate. Mean values were calculated using the weighted mean approach. RevMan Version 5.4 (Cochrane Collaboration) was used to perform the meta-analysis for this study. Cochrane Collaboration's Risk of Bias (RoB 2.0) approach was used to perform a quality assessment of the included articles in order to verify the validity and reliability of the research. RESULTS: 27 studies were in Group A and 28 studies pertained to Group B. There were 1662 patients in Group A and 1273 patients in Group B. Both groups had similar technical success (99.6% vs 99.4%). Thrombectomy showed longer mean procedure time (73.03 ± 14.57 min vs 47.35 ± 3.15 min), lower mean blood loss (325.20 ± 69.15 mL vs 423.05 ± 64.95 mL), shorter mean ICU stay (2.35 ± 1.64 days vs 3.22 ± 1.27 days), and shorter mean overall hospital stay (6.94 ± 4.38 days vs 7.23 ± 2.31 days). EKOS showed greater mean change in Miller Index (9.05 ± 3.35 vs 4.91 ± 3.70) and greater mean change in pulmonary artery pressure (14.17 ± 6.35 mmHg vs 8.11 ± 4.39 mmHg). CONCLUSION: Ultrasound accelerated thrombolysis and percutaneous mechanical thrombectomy are effective therapies for pulmonary embolism with comparable clinical outcomes.

Pulmonary artery mass with PIK3CA mutation after orthotopic heart transplantation.

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.

Inhibition of heterogeneous nuclear ribonucleoproteins A1 and oxidative stress reduces glycolysis via pyruvate kinase M2 in chronic thromboembolic pulmonary hypertension.

Background and Objective: Chronic thromboembolic pulmonary hypertension (CTEPH) is a lethal complication of pulmonary embolism involving pulmonary artery occlusion and microvascular disease. The glucose metabolism and reactive oxygen species (ROS) production may be perturbed in CTEPH, but the precise mechanisms are unclear. This study investigated glucose metabolism in CTEPH employing pulmonary endarterectomy (PEA)-derived pulmonary artery smooth muscle cells (PASMCs) and characterized the roles of pyruvate kinase M2 (PKM2) and its regulation by heterogeneous nuclear ribonucleoproteins A1 (hnRNPA1) and ROS in CTEPH. Methods: PEA tissues and blood samples of CTEPH patients were collected to study the levels of PKM2. Primary PASMCs were isolated from PEA tissues. We used small interfering RNAs to knock down PKM2 and hnRNPAI, and applied antioxidant N-acetylcysteine (NAC) and mito-TEMPO to reduce ROS production. The expression of glucometabolic genes, ROS production, glycolysis rate and proliferative and migratory activities were analyzed in PEA-derived PASMCs. Results: PKM2 levels in serum and PEA tissues of CTEPH patients were higher than that of the healthy controls. Compared to the control PASMCs, PEA-derived PASMCs showed increased PKM2 expression and ROS production. The rates of glycolysis, proliferation and migration were increased in PEA-PASMCs and could be mitigated by PKM2 downregulation through hnRNPA1 or ROS inhibition. Conclusions: Increased glycolysis and PKM2 expression were found in PEA-PASMCs. Inhibition of hnRNPA1 or ROS corrected the aberrant glycolysis, cell proliferation and migration by downregulating PKM2. Regulation of the hnRNPA1/PKM2 axis represents a potential therapeutic target for the treatment of CTEPH.

Coronary artery compression by dilated pulmonary artery in an early infantile case of tetralogy of Fallot with absent pulmonary valve: ventricular fibrillation as the initial symptom of myocardial ischaemia.

Coronary artery compression by a dilated pulmonary artery is a rare complication in patients with tetralogy of Fallot with absent pulmonary valve. We present a case in which this condition manifested at two months of age, with ventricular fibrillation as the initial symptom of myocardial ischaemia. It is important to recognise that this potentially fatal complication can occur in early infancy.

Anaesthetic management of a patient with idiopathic pulmonary arterial hypertension, suprasystemic pulmonary artery pressures and carcinoma of the ascending colon.

A 35-year-old woman with severe pulmonary arterial hypertension underwent open hemicolectomy with cholecystectomy under combined general and epidural anaesthesia. Intra-operative pulmonary artery pressure, as measured by Swan-Ganz catheter, was suprasystemic and managed with inodilators. She developed postoperative right ventricular dysfunction requiring inotropes, incremental pulmonary vasodilators and prolonged oxygen supplementation. One year after surgery, she is recurrence-free with oxygen saturations of 88-90% on air. This case highlights that with meticulous care and multidisciplinary team input, patients with severe pulmonary arterial hypertension can have favourable outcomes after major cancer surgery.

Vieussens' arterial ring forming a coronary-pulmonary artery fistula with large aneurysmal dilatation: a case report.

Background: Vieussens' arterial ring (VAR) is an embryonic conotruncal ring remnant that represents a rare vascular anomaly involving a connection between the right coronary artery (RCA) and the left anterior descending artery (LAD). We describe a unique case of VAR associated with large aneurysmal dilatation, which presents as the formation of a fistula between the coronary artery and pulmonary artery. Case summary: An 80-year-old Japanese woman presented with an asymptomatic mediastinal mass that was incidentally detected on computed tomography. Subsequent imaging over 2 years revealed the progression of the two masses connecting the RCA to the LAD measuring 8 × 7 mm and 28 × 21 mm in diameter. Transthoracic echocardiography identified a cystic lesion anterior to the right ventricular outflow tract, and colour Doppler imaging confirmed flow into the pulmonary artery. Furthermore, coronary angiography revealed a large aneurysm arising from the LAD, with an efferent vessel communicating with the pulmonary artery. Surgical intervention involved resection of the aneurysms and closure of the coronary-pulmonary artery fistula, which yielded favourable postoperative outcomes. Discussion: Vieussens' arterial ring is a rare but clinically significant anomaly with varied presentations; the incidence of VAR remains uncertain. In this case, surgical resection and closure of the fistula were performed to mitigate the risk of rupture and to address the potential haemodynamic consequences. Understanding and documenting such cases will contribute to refining treatment approaches and improving patient care in cardiovascular medicine.

A Belgian consensus on sotatercept for the treatment of pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a rare disease affecting the small pulmonary vessels, ultimately leading to right ventricular failure and death. Current treatment options target three different pathways (endothelin, nitric oxide/cGMP and prostacyclin pathways). Despite their demonstrated efficacy, these therapies (commonly used in combination) do not cure the disease which is why novel pathways beyond the traditional 'big three' are being developed. Sotatercept is a ligand trap for multiple proteins within the TGF-ß superfamily that was recently approved in the US for the treatment of PAH. Unlike currently available therapies, sotatercept has the potential to act as an anti-remodelling agent rather than a vasodilator. The safety and efficacy of subcutaneous (SC) sotatercept have been established in two multicentre, placebo-controlled randomised-controlled trials. The compound has been shown to consistently improve a variety of measurable endpoints, including exercise capacity, haemodynamics, quality of life and delay of clinical worsening. The drug appears to have an acceptable safety profile, although it is associated with an increased risk in developing telangiectasia and biological changes affecting platelet counts and haemoglobin. This study reviews the current evidence on SC sotatercept and provides a Belgian perspective on its place in the future treatment strategy for PAH.

Right ventricular-pulmonary artery uncoupling in patients with atrial fibrillation on peritoneal dialysis.

BACKGROUND: Tricuspid annular plane systolic excursion (TAPSE)/pulmonary artery systolic pressure (PASP) as a noninvasively measured index of right ventricular-pulmonary artery uncoupling is associated with poor outcomes in heart failure patients. However, the relationship by which the TAPSE/PASP is linked to atrial fibrillation (AF) in peritoneal dialysis (PD) patients is not clear. We aimed to investigate the relationship between the TAPSE/PASP and AF in PD patients. METHODS: This study was divided into two parts. First, we included 329 PD patients. All the subjects provided detailed a medical history, laboratory analysis and transthoracic echocardiography on admission. We evaluated the differences in the TASPE/PASP ratios between the AF and non-AF groups. Second, a total of 121 patients were followed up to compare mortality between the AF and non-AF groups. RESULTS: Age, BNP, RDW, LA, and septal E/e' were significantly higher, and TAPSE/PASP was significantly lower in patients with AF than in those without AF (p < 0.05). Moreover, the TAPSE/PASP was more pronounced in persistent AF patients. PD patients with AF had a greater risk of mortality (7.2%) than did those without AF (3.8%) after an average follow-up of 12 months. Kaplan-Meier analysis revealed that patients with TAPSE/PASP ratios ≤ 0.715 had a greater risk of mortality than did those with TAPSE/PASP ratios > 0.715. CONCLUSIONS: The results suggested that the TAPSE/PASP was lower in AF patients than in non-AF patients. The TAPSE/PASP may be a useful factor for predicting mortality in AF patients with PD, but large-scale prospective studies are needed for verification.

Correlation Between Tissue Doppler Imaging Method (E/e') and Invasive Measurements of Left Ventricular Filling Pressures: A Systematic Review, Meta-Analysis, and Meta-Regression.

OBJECTIVES: Evaluation of pulmonary capillary wedge pressure (PCWP) through right heart catheterization can indirectly provide an estimation of the filling pressure of the left ventricle. Echocardiography can estimate left ventricular compliance using mitral annular tissue Doppler imaging (TDI). The E/e' ratio refers to the correlation between the peak mitral inflow (E-wave) velocity and early diastolic tissue Doppler mitral annular velocity (e'). The main purpose of this systematic review was to establish the correlation between echocardiographic E/e' ratio and PCWP. The correlation between E/e' and left ventricular end-diastolic pressure (LVEDP) was evaluated as a secondary objective. DESIGN: A systematic review and meta-analysis of observational studies was conducted. The search was based on Medline (PubMed), Scopus, and Web of Science. SETTING: Intensive care unit or cardiac intensive care unit. PARTICIPANTS: Adult patients. INTERVENTIONS: Any study comparing the left ventricular filling pressure obtained by cardiac catheterization (reference) and echocardiographic evaluation, in particular TDI analysis (intervention), were included. MEASUREMENTS AND MAIN RESULTS: The pooled analysis included 94 studies from the initially identified 7,304 records. The correlation was 0.48 (95% CI 0.42-0.54, Q = 420.52, I2 = 84.8%) for PCWP and 0.50 (95% CI 0.38-0.60, Q = 210.91, I2 = 89.1%) for LVEDP. CONCLUSIONS: The E/e' ratio moderately correlated with PCWP/LVEDP. The correlation was stable irrespective of the sites where e' was measured, but each site has its own limitations for specific patient subpopulations. The correlation was weak in patients with heart failure with a preserved ejection fraction.

Unusual Developmental Vascular Anomalies: Insights From a Chest Physician's Perspective.

This case report discusses three developmental vascular anomalies (DVAs) observed in adults and highlights the challenges related to the diagnosis and management. Even though detected at early ages, diagnostic difficulties are observed in the adult age due to the scarcity and diverse clinical features. These cases illustrate the necessity of a multidisciplinary approach involving clinicians and radiologists for precise and prompt diagnosis in adults, where misdiagnosis and delays in intervention are frequent. The cases comprised a 17-year-old female with an absent right pulmonary artery and mitral stenosis, a 46-year-old female with chronic obstructive pulmonary disease (COPD), with an absent left pulmonary artery, and a 60-year-old female with bronchial asthma and tuberculosis exhibiting a rare DVA. This discussion highlights the importance of intensified clinical suspicion and thorough evaluation for the cases of unexplained respiratory symptoms and abnormal image findings in patients, which can further provide the medical community with valuable insights.

Unmasking of Agenesis of Right Pulmonary Artery with Unilateral Absence of Perfusion on Lung Scintigraphy.

We present a rare finding on lung ventilation-perfusion (V/Q) scintigraphy for a woman with longstanding dyspnea. CT of the chest showed volume loss on the right side, which raised concern about possible bronchiolitis obliterans or Swyer-James-MacLeod syndrome; however, the right pulmonary artery could not be visualized. A subsequent V/Q scan showed absence of perfusion and decreased ventilation to the entire right lung, consistent with agenesis of the right pulmonary artery. The patient's clinical course and imaging features mimicked Swyer-James-MacLeod syndrome, which usually presents with a matched perfusion defect in a single lung or lobe on V/Q scanning. This case highlights the importance of a multimodality imaging approach to achieve a diagnosis.

The Hemodynamic Effects of Bolus Dose Calcium in Patients Undergoing Pulmonary Artery Reconstruction and Unifocalization Surgery: A Pilot Study.

OBJECTIVES: To determine if bolus administration of calcium increases pulmonary artery pressures after unifocalization procedures or pulmonary artery reconstruction surgery. DESIGN: Retrospective cohort study using Stanford University's data warehouse. SETTING: A large pediatric heart center within an academic quaternary care facility. PARTICIPANTS: All patients undergoing pulmonary artery reconstruction or unifocalization procedures identifiable in the data warehouse. INTERVENTIONS: Data were collected from Stanford University's data repository and formatted and analyzed using RStudio (v 2023.06.1+524). MEASUREMENTS AND MAIN RESULTS: The primary outcome was the change in pulmonary artery systolic pressure (PASP) after a bolus administration of calcium. Secondary endpoints include changes in pulmonary arterial-to-systemic arterial pressure ratio, mean arterial pressure, right-sided filling pressure, and left atrial pressure. The Friedman test was used to assess differences and the Durbin-Conover rank-sum for pairwise comparisons. A difference in PASP after a bolus dose of calcium was found (Friedman X2 = 13.67, p = 0.003), with a higher PASP 5 minutes after calcium administration compared with 2 minutes before administration (35 mmHg v 33 mmHg, p = 0.01), and a higher PASP 10 minutes after calcium administration compared with2 minutes before administration (35 mmHg v 33 mmHg, p = 0.008). CONCLUSIONS: Calcium bolus administration led to an increase in pulmonary arterial pressure in patients after pulmonary artery reconstruction or unifocalization surgeries. It may be prudent to avoid bolus administration in this patient population immediately after repair or in patients with right ventricular dysfunction.

The clinical characteristics associated with the ratio between the main pulmonary artery and ascending aorta diameter in patients with acute exacerbation of chronic obstructive pulmonary disease.

Background: A pulmonary artery-to-aorta (PA/A) ratio of ≥1 is a reliable indicator of pulmonary hypertension and is associated with an increased risk of acute exacerbation of chronic obstructive pulmonary disease (COPD) and long-term mortality in patients with stable COPD. However, it is unclear whether a PA/A ratio of ≥1 is associated with mortality in patients hospitalized with acute exacerbation of COPD. The purpose of this study was to evaluate the clinical course and mortality of patients with PA/A ratios of ≥1 who were hospitalized with acute exacerbation of COPD. Methods: We retrospectively reviewed the medical charts of patients admitted to a tertiary referral hospital and a secondary hospital with acute exacerbation of COPD between 2016 and 2021. Chest computed tomography was used to measure the pulmonary artery (PA), aorta (A) diameter, and the PA/A ratio. The study involved 324 and 111 patients with PA/A ratios <1 and ≥1, respectively. Results: The average age in the two groups was 74.1 and 74.5 years, which was not significantly different. When compared with the group with PA/A ratios of <1, the group with PA/A ratios of ≥1 had a lower proportion of males (71.2% vs. 89.5%, P<0.001), more patients with type 2 respiratory failure (35.1% vs. 18.8%), higher high-flow nasal cannula use (10.8% vs. 4.6%), higher use of non-invasive ventilation (NIV) (21.6% vs. 7.7%), and longer hospital stay (10.9 vs. 9.5 days). In-hospital mortality was not significantly different between the two groups. A PA/A ratio of ≥1 was identified as an independent predictor of the need for high-flow nasal cannula, NIV, and intubation in COPD patients. Conclusions: Patients with PA/A ratios of ≥1 had a high incidence of type 2 acute respiratory failure and required advanced treatment, including high-flow nasal cannula, NIV, and intubation. Therefore, hospitalized patients with acute exacerbation of COPD and PA/A ratios of ≥1 require more aggressive treatment.