Pulmonary artery angiomatoid fibrous histiocytoma mimicking pulmonary embolism.

A young man presented to the emergency department with pleuritic chest pain and shortness of breath. Of note, he recently went on a long-distance flight of about 9 hours. Given his recent long-distance travel and clinical symptoms, a pulmonary embolism was suspected. However, pathological examination of the excised pulmonary artery intraluminal mass demonstrated an angiomatoid fibrous histiocytoma. This case describes the clinicopathological and immunohistochemical features and molecular profile of a rare type of pulmonary artery tumour, a pulmonary artery angiomatoid fibrous histiocytoma.

Successful Medical-Surgical Management of Intracardiac Thrombosis and Pulmonary Pseudoaneurysms in an Adolescent With Hughes-Stovin Syndrome.

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate.

Isolated pulmonary embolism - a specific clinical entity?

Pulmonary embolism in classical meaning is a complication of deep vein thrombosis (usually in the leg veins), developing after a part of the thrombus dislodged and got wedged in pulmonary arteries. However, in half of the patients with pulmonary embolism, deep vein thrombosis is not found. One potential explanation is a different, less common location of the thrombus or previous complete embolization of the whole thrombotic mass. Another possibility is pulmonary artery thrombosis in situ, which is a specific clinical entity associated with some typical risk factors. It develops in the place of vascular injury, as a consequence of hypoxia, inflammatory changes, endothelial dysfunction and injury. Pulmonary artery thrombosis in situ can be a complication after lung resection, radiation therapy, chest trauma, in the patients with Behçet´s disease, sickle cell anemia, chronic obstructive pulmonary disease, tuberculosis or covid pneumonia. Pulmonary artery thrombosis in situ may differ from classical pulmonary embolism in prognosis as well as in therapeutic approach.

Recent Advances in Remote Pulmonary Artery Pressure Monitoring for Patients with Chronic Heart Failure: Current Evidence and Future Perspectives.

Chronic heart failure (HF) is associated with high hospital admission rates and has an enormous burden on hospital resources worldwide. Ideally, detection of worsening HF in an early phase would allow physicians to intervene timely and proactively in order to prevent HF-related hospitalizations, a concept better known as remote hemodynamic monitoring. After years of research, remote monitoring of pulmonary artery pressures (PAP) has emerged as the most successful technique for ambulatory hemodynamic monitoring in HF patients to date. Currently, the CardioMEMS and Cordella HF systems have been tested for pulmonary artery pressure monitoring and the body of evidence has been growing rapidly over the past years. However, several ongoing studies are aiming to fill the gap in evidence that is still very clinically relevant, especially for the European setting. In this comprehensive review, we provide an overview of all available evidence for PAP monitoring as well as a detailed discussion of currently ongoing studies and future perspectives for this promising technique that is likely to impact HF care worldwide.

Frail Silk: Is the Hughes-Stovin Syndrome a Behçet Syndrome Subtype with Aneurysm-Involved Gene Variants?

Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The etiology and pathogenesis of HSS are incompletely known. The current consensus is that vasculitis underlies the pathogenic process, and pulmonary thrombosis follows arterial wall inflammation. As such, Hughes-Stovin syndrome may belong to the vascular cluster with lung involvement of Behçet syndrome, although oral aphtae, arthritis, and uveitis are rarely found. Behçet syndrome is a multifactorial polygenic disease with genetic, epigenetic, environmental, and mostly immunological contributors. The different Behçet syndrome phenotypes are presumably based upon different genetic determinants involving more than one pathogenic pathway. Hughes-Stovin syndrome may have common pathways with fibromuscular dysplasias and other diseases evolving with vascular aneurysms. We describe a Hughes-Stovin syndrome case fulfilling the Behçet syndrome criteria. A MYLK variant of unknown significance was detected, along with other heterozygous mutations in genes that may impact angiogenesis pathways. We discuss the possible involvement of these genetic findings, as well as other potential common determinants of Behçet/Hughes-Stovin syndrome and aneurysms in vascular Behçet syndrome. Recent advances in diagnostic techniques, including genetic testing, could help diagnose a specific Behçet syndrome subtype and other associated conditions to personalize the disease management.

The poorly understood yet potent risk of pulmonary artery thrombosis in-situ in Post-Acute COVID-19 syndrome.

Pulmonary artery thrombosis in-situ is a term used to describe a pulmonary embolism occurs in the absence of deep vein thrombosis in the lower extremities. Most cases occur in a patient who had a recent traumatic injury to the chest. Other risk factors include the presence of hypercoagulable conditions, including inflammatory state, hypoxia and vascular endothelial injury. Although it has been discussed extensively in the acute COVID-19 disease, pulmonary artery thrombosis in-situ that occur in the setting of Post-Acute COVID-19 syndrome is not commonly reported and poorly understood.

Stage-1 Hybrid Palliation for High-Risk 2-Ventricle Patients with Ductal-Dependent Systemic Circulation in the Era of High Prenatal Detection.

OBJECTIVE: We reviewed our center's prenatal detection and surgical experience with high-risk, 2-ventricle patients, with complex congenital heart disease that underwent stage-1 hybrid palliation. METHODS: We retrospectively identified those born between March 2008 and March 2021 with 2-ventricle hearts, complex congenital cardiovascular malformations, and ductal-dependent systemic circulation that underwent stage-1 hybrid palliation consisting of surgical bilateral pulmonary artery banding and interventional catheterization placed ductus arteriosus stents. RESULTS: We identified 30 patients. Of the 30, 19 (63%) were male. For the 30, median gestational age was 35 weeks (29-39 weeks), and median birth weight was 2.2 kg (0.6-4.5 kg). Of the 30, 1 was transferred from an adjacent state, and 29 were born in Nevada. Of the 29 born in Nevada, overall statewide prenatal detection was 18 of 29 (62%); however, for 2008 to 2011 the prenatal detection rate was 3 of 10 (30%) and 15 of 19 (79%) for 2012 to 2021, P = .03. For the last 5 years, prenatal detection for Nevada-born patients was 8 of 8 (100%). Two full-term newborns, without a prenatal diagnosis, presented postnatally in extremis. For the 30 patients, there were 0 stage-1 hybrid palliation mortalities, 1 subsequent repair mortality, and 3 late nonsurgical deaths. CONCLUSIONS: Stage-1 hybrid palliation may result in excellent surgical outcomes for high-risk, 2-ventricle patients. Additionally, high rates of population-wide prenatal detection are possible for high-risk congenital heart disease, allowing prenatal planning and possibly reducing postnatal extremis presentations.

Acute phase characteristics and long-term complications of pulmonary embolism in COVID-19 compared to non-COVID-19 cohort: a large single-centre study.

BACKGROUND: To compare the severity of pulmonary embolism (PE) and the long-term complications between patients with and without COVID-19, and to investigate whether the tools for risk stratification of death are valid in this population. METHODS: We retrospectively included hospitalized patients with PE from 1 January 2016 to 31 December 2022. Comparisons for acute episode characteristics, risk stratification of the PE, outcomes, and long-term complications were made between COVID and non-COVID patients. RESULTS: We analyzed 116 (27.5%) COVID patients and 305 (72.4%) non-COVID patients. In patients with COVID-19, the traditional risk factors for PE were absent, and the incidence of deep vein thrombosis was lower. COVID patients showed significantly higher lymphocyte count, lactate dehydrogenase, lactic acid, and D-dimer levels. COVID patients had PE of smaller size (12.3% vs. 25.5% main pulmonary artery, 29.8% vs. 37.1% lobar, 44.7% vs. 29.5% segmental and 13.2% vs. 7.9% subsegmental, respectively; p < 0.001), less right ventricular dysfunction (7.7% vs. 17.7%; p = 0.007) and higher sPESI score (1.66 vs. 1.11; p < 0.001). The need for mechanical ventilation was significantly higher in COVID patients (8.6% vs. 1.3%; p < 0.001); However, the in-hospital death was less (5.2% vs. 10.8%; p = 0.074). The incidence of long-term complications was lower in COVID cohort (p < 0.001). PE severity assessed by high sPESI and intermediate and high-risk categories were independently associated with in-hospital mortality in COVID patients. CONCLUSION: The risk of in-hospital mortality and the incidence of long-term complications were lower in COVID-19. The usual tools for risk stratification of PE are valid in COVID patients.

Hypoxic pulmonary vascular response can screen subclinical lifestyle disease in healthy population.

OBJECTIVE: Subclinical life style disease can cause endothelial dysfunction associated with perfusion abnormalities and reduced vascular compliance. Subclinical elevated beta type natriuretic peptide (BNP) has been associated with altered fluid shift from extra to intracellular space during acute hypoxia. Therefore we measured vascular response and BNP levels during acute hypoxia to study endothelial functions among healthy individuals. METHODS: Individuals were exposed to acute normobaric hypoxia of FiO2 = 0.15 for one hour in supine position and their pulmonary and systemic vascular response to hypoxia was compared. Individuals were divided into two groups based on either no response (Group 1) or rise in systolic pulmonary artery pressure to hypoxia (Group 2) and their BNP levels were compared. RESULTS: BNP was raised after hypoxia exposure in group 2 only from 18.52 ± 7 to 21.56 ± 10.82 picogram/ml, p < 0.05. Group 2 also showed an increase in mean arterial pressure and no fall in total body water in response to acute hypoxia indicating decreased endothelial function compared to Group 1. CONCLUSION: Rise in pulmonary artery pressure and BNP level in response to acute normobaric hypoxia indicates reduced endothelial function and can be used to screen subclinical lifestyle disease among healthy population.

Molecular Pathways in Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension is a multifactorial, chronic disease process that leads to pulmonary arterial endothelial dysfunction and smooth muscular hypertrophy, resulting in impaired pliability and hemodynamics of the pulmonary vascular system, and consequent right ventricular dysfunction. Existing treatments target limited pathways with only modest improvement in disease morbidity, and little or no improvement in mortality. Ongoing research has focused on the molecular basis of pulmonary arterial hypertension and is going to be important in the discovery of new treatments and genetic pathways involved. This review focuses on the molecular pathogenesis of pulmonary arterial hypertension.

Pulmonary artery pressures and right ventricular dimensions of post-COVID-19 patients without previous significant cardiovascular pathology.

BACKGROUND: Pulmonary hypertension is a significant complication of COVID-19, but follow-up data on pulmonary artery pressure after recovery from COVID-19 are limited. OBJECTIVES: To investigate pulmonary artery pressure and heart dimensions in post-COVID-19 patients without a history of significant cardiac pathology. METHODS: Data for 91 eligible adult patients were subjected to 2 analyses. First, patients were grouped according to where they received COVID-19 treatment: the ICU, COVID-19 ward, or outpatient clinic. Second, the severity of COVID-19 was grouped as no pulmonary involvement, non-severe pulmonary involvement, or severe pulmonary involvement based on thoracic computed tomography scores. Heart dimensions were measured and pulmonary artery pressure was estimated using transthoracic echocardiography. The correlation between transthoracic echocardiography findings and COVID-19 severity was assessed. RESULTS: Pulmonary artery pressure and right-heart dimensions were significantly elevated in the post-COVID-19 patients without a history of risk factors for pulmonary hypertension that presented to the cardiology outpatient clinic with cardiac complaints. Both of these findings were correlated with the severity of COVID-19 and the extent of lung involvement based on thoracic computed tomography. CONCLUSION: The present findings confirm that increases in systolic pulmonary artery pressure and right ventricular dimensions persist 2-3 months after recovery from COVID-19 in patients without a history of risk factor for pulmonary hypertension. Furthermore, the increase in pulmonary artery pressure and right ventricular dimensions correlate with the severity of COVID-19 and the extent of lung involvement based on thoracic computed tomography.

First-in-man successful use of the SPECTRUM percutaneous dual-stage right ventricle and right atrium to pulmonary artery ventricular assist device.

BACKGROUND: Over the past decade, several minimally invasive mechanical support devices have been introduced into clinical practice to support the right ventricle (RV). Percutaneous cannulas are easy to insert, minimally invasive, and treat acute RV failure rapidly. In December 2021, the Food and Drug Administration approved a new 31 French dual lumen single cannula for use as a right ventricular assist device. AIMS: Descirbe the use of the new dual lumen percutaneous right ventricular assist device (RVAD) cannula. MATERIAL AND METHODS: Deployment of the RVAD can be done surgically or percutaneously. This cannula, manufactured by Spectrum, is dual staged. It has inflow ports positioned both in the right atrium (RA) as well as the RV for maximal drainage of the right heart. The distal end of the cannula which includes the outflow port is positioned in the pulmonary artery (PA). RESULTS: Deployment of the Spectrum RVAD can be done percutaneously with transesophageal and flouroscopy guidence. Cannulation requires requisite wire skills in order to navigate into the main pulmonary artery. Utilization of this cannula can be done in acute RV failure secondary to ischemia, post cardiotomy shock, acute respiratory failure or other causes of isolated RV failure. DISCUSSION: The dual stage drainage design optimizes venous drainage as well as limits suck-down events. Theoretically, direct RV decompression also decreases RV dilation and wall tension, and facilitates improved transmural pressure gradient to reduce RV strain. CONCLUSION: Here we describe the first-in-man successful use of the dual-stage RA and RV to PA Spectrum cannula in a patient with severe COVID acute respiratory distress syndrome and acute right ventricular failure, bridged to recovery.

Novel method for left ventricular unloading utilizing percutaneous pulmonary artery drainage in cardiorespiratory failure due to COVID-19 infection.

Left ventricular (LV) unloading is an important concept in patients undergoing peripheral venoarterial extracorporeal membrane oxygenation (VA-ECMO). We present a case of a 32-year-old male in acute cardiorespiratory collapse due to coronavirus disease (COVID-19) who underwent VA-ECMO cannulation in the setting of cardiogenic shock and acute respiratory distress syndrome. Due to inability to utilize percutaneous LV assist device (pLVAD) for LV unloading due to small end diastolic dimension, alternative strategies were explored. A traditionally utilized right ventricular support device, the ProTek Duo (TandemLife, Pittsburgh, PA), was utilized to drain the pulmonary artery, leading to improvement in parameters for cardiogenic shock. To our knowledge, this is the first case in which a ProTek Duo has been utilized in conjunction with VA-ECMO to provide LV unloading in support of a patient in cardiogenic shock. This method can be employed in future challenging situations where pLVAD is not feasible.

The GUIDE-HF trial of pulmonary artery pressure monitoring in heart failure: impact of the COVID-19 pandemic.

AIMS: During the coronavirus disease 2019 (COVID-19) pandemic, important changes in heart failure (HF) event rates have been widely reported, but few data address potential causes for these changes; several possibilities were examined in the GUIDE-HF study. METHODS AND RESULTS: From 15 March 2018 to 20 December 2019, patients were randomized to haemodynamic-guided management (treatment) vs. control for 12 months, with a primary endpoint of all-cause mortality plus HF events. Pre-COVID-19, the primary endpoint rate was 0.553 vs. 0.682 events/patient-year in the treatment vs. control group [hazard ratio (HR) 0.81, P = 0.049]. Treatment difference was no longer evident during COVID-19 (HR 1.11, P = 0.526), with a 21% decrease in the control group (0.536 events/patient-year) and no change in the treatment group (0.597 events/patient-year). Data reflecting provider-, disease-, and patient-dependent factors that might change the primary endpoint rate during COVID-19 were examined. Subject contact frequency was similar in the treatment vs. control group before and during COVID-19. During COVID-19, the monthly rate of medication changes fell 19.2% in the treatment vs. 10.7% in the control group to levels not different between groups (P = 0.362). COVID-19 was infrequent and not different between groups. Pulmonary artery pressure area under the curve decreased -98 mmHg-days in the treatment group vs. -100 mmHg-days in the controls (P = 0.867). Patient compliance with the study protocol was maintained during COVID-19 in both groups. CONCLUSION: During COVID-19, the primary event rate decreased in the controls and remained low in the treatment group, resulting in an effacement of group differences that were present pre-COVID-19. These outcomes did not result from changes in provider- or disease-dependent factors; pulmonary artery pressure decreased despite fewer medication changes, suggesting that patient-dependent factors played an important role in these outcomes. Clinical Trials.gov: NCT03387813.

Pulmonary Artery Pseudoaneurysm in COVID-19-Associated Pulmonary Mucormycosis: Case Series and Systematic Review of the Literature.

Literature on COVID-19-associated pulmonary mucormycosis (CAPM) is sparse. Pulmonary artery pseudoaneurysm (PAP) is an uncommon complication of pulmonary mucormycosis (PM), and rarely reported in CAPM. Herein, we report five cases of CAPM with PAP managed at our center and perform a systematic review of the literature. We diagnosed PM in those with clinico-radiological suspicion and confirmed it by microbiology or histopathology. We encountered five cases of CAPM with PAP (size ranged from 1 × 0.8 cm to ~ 4.9 × 4.8 cm). All subjects had diabetes and were aged 55-62 years (75% men). In two cases, COVID-19 and mucormycosis were diagnosed simultaneously, while in three others, COVID-19 preceded PM. One subject who underwent surgery survived, while all others died (80% mortality). From our systematic review, we identified one additional case of CAPM with PAP in a transplant recipient. CAPM with PAP is rare with high mortality. Early diagnosis and multimodality management are imperative to improve outcomes.

Pulmonary artery targeted therapy in treatment of COVID-19 related ARDS. Literature review.

INTRODUCTION: The most grievous complication of the COVID-19 is the acute respiratory distress syndrome. A specific, rescue treatment for rapidly deteriorating patients should emerge to improve respiratory function and help patients to survive the most challenging period. Drugs used in targeted therapy of pulmonary arterial hypertension (PAH) appears to be suitable for this task and this article describes their potential for treatment of severe cases of COVID-19. METHODS: The authors reviewed the following databases for randomized controlled trials, reviews and meta-analyses published up to July 2020: Pubmed, Scopus, Google Scholar, Cochrane Database and ClinicalKey. The authors included every study contributory to the assessment of the potential of drugs used in targeted PAH therapy in treatment of COVID-19. RESULTS: Endothelin receptor antagonists, phosphodiesterase 5 inhibitors, riociguat and prostacyclin have proven ani-inflammatory effect and reduce pulmonary artery blood pressure, lung oedema and remodelling. Bosentan shows antiviral properties and sildenafil, as well as epoprostenol, inhibits apoptosis of lung epithelial cells. Among patients with lung lesions the decrease of pulmonary blood pressure can lead to increase of ventilation/perfusion mismatch and decrease of blood oxygenation. CONCLUSIONS: Among all assessed drugs bosentan, sildenafil and epoprostenol appear to be most promising and a combination of these drugs should be considered due to synergism. The targeted PAH therapy in treatment of COVID-19 associated ARDS could be a useful tool saving lives of patients with severe SARS-CoV-2 infection, however, its introduction should be investigated and monitored very carefully as it can lead to transient deterioration of patient condition.