scholarly journals Goiter in a Patient with Pulmonary Arterial Hypertension Treated with Epoprostenol

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Shaadi Abughazaleh ◽  
Zeenat Safdar

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.

2020 ◽  
Author(s):  
Nan Liu ◽  
Ying Xing ◽  
Chen Wang

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).


Author(s):  
Guillermo Cueto Robledo ◽  
Luis Eugenio Granier Palafox ◽  
Merly Yamile Jurado Hernández ◽  
Yudi Alejandra Calderón Paez ◽  
Marisol García César ◽  
...  

2017 ◽  
Vol 20 (5) ◽  
pp. 231
Author(s):  
Shuyang Lu ◽  
Lai Wei ◽  
Chunsheng Wang

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. 


2021 ◽  
Vol 8 ◽  
Author(s):  
Franziska Diekmann ◽  
Ekaterina Legchenko ◽  
Philippe Chouvarine ◽  
Ralf Lichtinghagen ◽  
Harald Bertram ◽  
...  

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.


2016 ◽  
Vol 25 (6) ◽  
pp. 453-456 ◽  
Author(s):  
Swaminathan Vaidyanathan ◽  
Sivakumar Kothandam ◽  
Rajesh Kumar ◽  
Priya M Pradhan ◽  
Ravi Agarwal

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.


2012 ◽  
Vol 10 (4) ◽  
pp. 227-232 ◽  
Author(s):  
Deborah H. McCollister ◽  
Philippe Weintraub ◽  
David B. Badesch

The recent identification of depression as an important comorbidity in pulmonary arterial hypertension (PAH)12 is leading to a broad array of efforts to further refine our understanding of this disorder, enhance patient and provider education about it, and encourage prompt recognition, appropriate diagnosis, and treatment of affected individuals. We will provide an update on the nature and extent of the problem, and describe ongoing and future efforts to address this very important determinant of quality of life and possible long-term outcome in patients with PAH.


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