Circulating Interleukin-7 in Human Pulmonary Arterial Hypertension

Objectives: Interleukin-7 (IL-7) secures B cell maturation, regulatory T and natural killer (NK) cell survival, and homeostasis, all of which are important for beneficial immunomodulation in pulmonary arterial hypertension (PAH). However, the role and potential impact of IL-7, VEGF-C and the vascular injury markers ICAM-1, and VCAM-1 on the pathobiology and severity of PAH is unknown.Methods: EDTA blood was collected during cardiac catheterization from the superior vena cava (SVC), pulmonary artery (PA), and ascending aorta (AAO) in children with pulmonary hypertension (PH) [n = 10; 9.1 (3.9–18.5) years] and non-PH controls [n = 10; 10.5 (2.0–17.3) years]. Compartment-specific plasma concentrations of IL-7, VEGF-C, aldosterone, ICAM-1, and VCAM-1 were determined using Meso Scale Discovery's multi array technology and the LIAISON Aldosterone Assay.Results: Children with PH had approximately 50% lower IL-7 (p < 0.01) and 59% lower VEGF-C plasma levels (p < 0.001) in the SVC, PA, and AAO versus non-PH controls. IL-7 and VEGF-C concentrations negatively correlated with the pulmonary vascular resistance (PVR)/systemic vascular resistance (SVR) ratio (rho = −0.51 and r = −0.62, respectively). Central-venous IL-7 strongly positively correlated with VEGF-C (r = 0.81). Most patients had a step down in ICAM-1 and VCAM-1 plasma concentrations across the pulmonary circulation and both ICAM-1 and VCAM-1 transpulmonary gradients negatively correlated with invasive hemodynamics.Conclusion: This manuscript is the first report on decreased circulating IL-7 and VEGF-C plasma concentrations in human PAH and their inverse correlations with invasive surrogates of PAH severity. Additional and larger studies are needed to explore the role of the immune-modulatory IL-7 and VEGF-C in pediatric and adult PAH.

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Repetitive and Brief Occlusion of Total Right Atrium Venous Blood Flow Improves Experimental Pulmonary Arterial Hypertension: a novel potential therapeutic strategy

10.1101/2020.10.04.314708 ◽

2020 ◽

Author(s):

Nan Liu ◽

Ying Xing ◽

Chen Wang

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Therapeutic Strategy ◽

Superior Vena ◽

Venous Blood ◽

Vena Cava ◽

Vegf Receptor ◽

Venous Blood Flow ◽

History Of ◽

Pulmonary Arterial

AbstractPulmonary arterial hypertension (PAH) is a vascular disorder associated with significant morbidity and mortality. The pathophysiology of PAH remains controversial, but the only currently available therapies for PAH are pharmacological pulmonary artery vasodilation, decreasing right ventricular (RV) afterload, and relieving symptoms. By now, there is no therapy being able to minimize vascular remodeling processes and thus to reverse or delay the natural history of the disease. It has been generally thought that reduction of RV preload was detrimental, which deteriorated the systemic hemodynamics. In the present study, however, we repetitively and briefly occluded (RBO) both superior vena cava and inferior vena cava by ligation (occlusion for less than 5 seconds then re-open for 30 seconds and repeated 5 cycles as one sequence, 1 sequence every 6 hours) to intermittently restrict RV preload, for continuous 24 hours, total 5 sequences, in the Sugen 5416 (VEGF receptor blocker) and hypoxia induced PAH rat models and we found this strategy was beneficial for lowering pulmonary vascular resistance (PVR).

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Goiter in a Patient with Pulmonary Arterial Hypertension Treated with Epoprostenol

Case Reports in Pulmonology ◽

10.1155/2020/1617253 ◽

2020 ◽

Vol 2020 ◽

pp. 1-4

Author(s):

Shaadi Abughazaleh ◽

Zeenat Safdar

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Thyroid Disease ◽

Superior Vena ◽

Vena Cava ◽

Large Goiter ◽

Facial Swelling ◽

Vena Cava Syndrome ◽

Pulmonary Arterial

A 35-year-old female with pulmonary arterial hypertension (PAH) who presented with complaints of progressively worsening dysphagia, facial swelling, and shortness of breath, was found to have a large goiter. In patients treated with epoprostenol for long periods of time, thyroid disease is common. Most cases of thyroid disease describe thyrotoxicosis and hyperthyroid statues, but our case was a patient on long term IV epoprostenol presenting with a superior vena cava-syndrome (SVC) like appearance and airway compromise found to have a goiter incidentally during workup.

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Tricuspid atresia with persistent left superior vena cava and pulmonary arterial hypertension. Case report

Current Problems in Cardiology ◽

10.1016/j.cpcardiol.2021.100837 ◽

2021 ◽

pp. 100837

Author(s):

Guillermo Cueto Robledo ◽

Luis Eugenio Granier Palafox ◽

Merly Yamile Jurado Hernández ◽

Yudi Alejandra Calderón Paez ◽

Marisol García César ◽

...

Keyword(s):

Case Report ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Superior Vena Cava ◽

Superior Vena ◽

Vena Cava ◽

Tricuspid Atresia ◽

Left Superior Vena Cava ◽

Persistent Left Superior Vena ◽

Pulmonary Arterial

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Atypical Case of Partial Anomalous Pulmonary Venous Connection Misdiagnosed as Primary Pulmonary Arterial Hypertension

The Heart Surgery Forum ◽

10.1532/hsf.1618 ◽

2017 ◽

Vol 20 (5) ◽

pp. 231

Author(s):

Shuyang Lu ◽

Lai Wei ◽

Chunsheng Wang

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Superior Vena ◽

Pulmonary Veins ◽

Delayed Diagnosis ◽

Vena Cava ◽

Volume Overload ◽

Heart Volume ◽

Pulmonary Arterial ◽

Anomalous Pulmonary Venous Connection

In this case report, we present the delayed diagnosis of an atypical partial anomalous pulmonary venous connection, which was initially misdiagnosed as primary pulmonary arterial hypertension. It was difficult to make a conclusive diagnosis using transthoracic echocardiography (TTE), because the two high right superior pulmonary veins drained into the superior vena cava and limited the shunt of patent foramen ovale. Preoperative TTE only showed right heart volume overload, pulmonary arterial hypertension, and severe tricuspid valve insufficiency. A chest CT-angiography (angio-CT) finally found the anomalous right superior pulmonary venous connection, which was further confirmed by surgery. The postoperative course was uneventful.

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Unusual presentation of total anomalous systemic venous connection

Asian Cardiovascular and Thoracic Annals ◽

10.1177/0218492316644355 ◽

2016 ◽

Vol 25 (6) ◽

pp. 453-456 ◽

Cited By ~ 2

Author(s):

Swaminathan Vaidyanathan ◽

Sivakumar Kothandam ◽

Rajesh Kumar ◽

Priya M Pradhan ◽

Ravi Agarwal

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Superior Vena Cava ◽

Superior Vena ◽

Vena Cava ◽

Venae Cavae ◽

Pulmonary Arterial ◽

The Right ◽

Severe Pulmonary Arterial Hypertension ◽

Atrial Rhythm

A 9-year-old girl who presented with dyspnea on exertion was diagnosed with total anomalous systemic venous connection to the left atrium (both venae cavae), no left superior vena cava, and a moderate-sized atrial septal defect with severe pulmonary arterial hypertension and ectopic atrial rhythm. She underwent septation of the common atrium using autologous pericardium, thereby rerouting the superior vena cava, inferior vena cava, and coronary sinus to the right atrium. Her postoperative course was uneventful. This case is reported for its rarity of presentation with severe pulmonary arterial hypertension and ectopic atrial rhythm.

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Sex Dimorphism in Pulmonary Hypertension: The Role of the Sex Chromosomes

Antioxidants ◽

10.3390/antiox10050779 ◽

2021 ◽

Vol 10 (5) ◽

pp. 779

Author(s):

Daria S. Kostyunina ◽

Paul McLoughlin

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Pulmonary Vascular Resistance ◽

Vascular Resistance ◽

Sex Chromosomes ◽

Bone Morphogenetic Protein 2 ◽

Sex Dimorphism ◽

Pulmonary Arterial ◽

The Impact

Pulmonary hypertension (PH) is a condition characterised by an abnormal elevation of pulmonary artery pressure caused by an increased pulmonary vascular resistance, frequently leading to right ventricular failure and reduced survival. Marked sexual dimorphism is observed in patients with pulmonary arterial hypertension, a form of pulmonary hypertension with a particularly severe clinical course. The incidence in females is 2–4 times greater than in males, although the disease is less severe in females. We review the contribution of the sex chromosomes to this sex dimorphism highlighting the impact of proteins, microRNAs and long non-coding RNAs encoded on the X and Y chromosomes. These genes are centrally involved in the cellular pathways that cause increased pulmonary vascular resistance including the production of reactive oxygen species, altered metabolism, apoptosis, inflammation, vasoconstriction and vascular remodelling. The interaction with genetic mutations on autosomal genes that cause heritable pulmonary arterial hypertension such as bone morphogenetic protein 2 (BMPR2) are examined. The mechanisms that can lead to differences in the expression of genes located on the X chromosomes between females and males are also reviewed. A better understanding of the mechanisms of sex dimorphism in this disease will contribute to the development of more effective therapies for both women and men.

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