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

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. 

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

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).

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
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.

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

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

scholarly journals Circulating Interleukin-7 in Human Pulmonary Arterial Hypertension

2021 ◽  
Vol 8 ◽  
Author(s):  
Franziska Diekmann ◽  
Ekaterina Legchenko ◽  
Philippe Chouvarine ◽  
Ralf Lichtinghagen ◽  
Harald Bertram ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Vascular Resistance ◽  
Nk Cell ◽  
Superior Vena ◽  
Plasma Concentrations ◽  
Vena Cava ◽  
Interleukin 7 ◽  
B Cell Maturation ◽  
Pulmonary Arterial

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.

Unusual presentation of total anomalous systemic venous connection

2016 ◽  
Vol 25 (6) ◽  
pp. 453-456 ◽  
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.

scholarly journals Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

2020 ◽  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Case Series ◽  
Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

Cor Triatriatum in Association With a Unique Form of Partial Anomalous Pulmonary Venous Connection

2021 ◽  
pp. 215013512110469
Author(s):  
Lou Capecci ◽  
Richard D. Mainwaring ◽  
Inger Olson ◽  
Frank L. Hanley
Keyword(s):  
Superior Vena Cava ◽  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Venous Drainage ◽  
Cor Triatriatum ◽  
Unique Form ◽  
Elective Repair ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Cor triatriatum may be associated with abnormalities of pulmonary venous anatomy. This case report describes a unique form of partial anomalous pulmonary venous connection. The patient presented at 5 weeks of age with symptoms of tachypnea and poor feeding. Echocardiography demonstrated cor triatriatum and partial anomalous pulmonary venous drainage of the right upper lung. The patient underwent urgent repair of cor triatriatum. It was elected to not address the partial anomalous pulmonary venous connection at that time. The patient returned at age 19 months for elective repair of the anomalous pulmonary venous connection. There was also a large vein connecting the right lower pulmonary veins to the superior vena cava. This was repaired by dividing the superior vena cava along a vertical axis to redirect the flow of the anomalous pulmonary veins through the connecting vein to the left atrium. This report describes the anatomy and surgical approach to a unique form of anomalous pulmonary venous connection.

scholarly journals Trans-right ventricle and transpulmonary metabolite gradients in human pulmonary arterial hypertension

Heart ◽  
2020 ◽  
Vol 106 (17) ◽  
pp. 1332-1341 ◽  
Author(s):  
Philippe Chouvarine ◽  
Martin Giera ◽  
Gabi Kastenmüller ◽  
Anna Artati ◽  
Jerzy Adamski ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Vena Cava ◽  
Dicarboxylic Acids ◽  
P Value ◽  
Blood Collection ◽  
Lipid Species ◽  
Metabolite Concentrations ◽  
Pulmonary Arterial

ObjectiveWhile metabolic dysfunction occurs in several pulmonary arterial hypertension (PAH) animal models, its role in the human hypertensive right ventricle (RV) and lung is not well characterised. We investigated whether circulating metabolite concentrations differ across the hypertensive RV and/or the pulmonary circulation, and correlate with invasive haemodynamic/echocardiographic variables in patients with PAH.MethodsProspective EDTA blood collection during cardiac catheterisation from the superior vena cava (SVC), pulmonary artery (PA) and ascending aorta (AAO) in children with PAH (no shunt) and non-PAH controls (Con), followed by unbiased screens of 427 metabolites and 836 lipid species and fatty acids (FAs) in blood plasma (Metabolon and Lipidyzer platforms). Metabolite concentrations were correlated with echocardiographic and invasive haemodynamic variables.ResultsMetabolomics/lipidomics analysis of differential concentrations (false discovery rate<0.15) revealed several metabolite gradients in the trans-RV (PA vs SVC) setting. Notably, dicarboxylic acids (eg, octadecanedioate: fold change (FC)_Control=0.77, FC_PAH=1.09, p value=0.044) and acylcarnitines (eg, stearoylcarnitine: FC_Control=0.74, FC_PAH=1.21, p value=0.058). Differentially regulated metabolites were also found in the transpulmonary (AAO vs PA) setting and between-group comparisons, that is, in the SVC (PAH-SVC vs Con-SVC), PA and AAO. Importantly, the differential PAH-metabolite concentrations correlated with numerous outcome-relevant variables (e.g., tricuspid annular plane systolic excursion, pulmonary vascular resistance).ConclusionsIn PAH, trans-RV and transpulmonary metabolite gradients exist and correlate with haemodynamic determinants of clinical outcome. The most pronounced differential trans-RV gradients are known to be involved in lipid metabolism/lipotoxicity, that is, accumulation of long chain FAs. The identified accumulation of dicarboxylic acids and acylcarnitines likely indicates impaired β-oxidation in the hypertensive RV and represents emerging biomarkers and therapeutic targets in PAH.

scholarly journals Right atrial function is associated with RV diastolic stiffness: RA-RV interaction in pulmonary arterial hypertension

2021 ◽  
pp. 2101454
Author(s):  
Jeroen N. Wessels ◽  
Sophia A. Mouratoglou ◽  
Jessie van Wezenbeek ◽  
M. Louis Handoko ◽  
J. Tim Marcus ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Diastolic Pressure ◽  
Vena Cava ◽  
Active Phase ◽  
Right Atrial ◽  
Stroke Work ◽  
Diastolic Stiffness ◽  
Pulmonary Arterial ◽  
The Impact

BackgroundPulmonary arterial hypertension (PAH) patients have altered right atrial (RA) function and right ventricular (RV) diastolic stiffness. This study assessed the impact of RV diastolic stiffness on RA-RV interaction.MethodsLow or high end-diastolic elastance (Eed) PAH patients (n=94) were compared to controls (n=31). Treatment response was evaluated in n=62 patients. RV and RA longitudinal strain, RA emptying and RV filling were determined and diastole was divided in a passive and active phase. Vena cava backflow was calculated as RV active filling-RA active emptying; RA stroke work as RA active emptying*RV end-diastolic pressure.ResultsWith increased Eed, RA and RV passive strain were reduced while active strain was preserved. In comparison to controls, patients had lower RV passive filling, but higher RA active emptying and RA stroke work. RV active filling was lower in high Eed patients, resulting in higher vena cava backflow. Upon treatment, Eed reduced in half of high Eedpatients, which coincided with larger reductions in afterload, RV mass and vena cava backflow and greater improvements in RV active filling and stroke volume in comparison to patients in whom Eed remained high.ConclusionsIn PAH, RA function is associated with changes in RV function. Despite increased RA stroke work, severe RV diastolic stiffness is associated with reduced RV active filling and increased vena cava backflow. In 50% of high baseline Eed patients, diastolic stiffness remains high, despite treatment. Eed reduction coincided with a large reduction in afterload, increased RV active filling and decreased vena cava backflow.

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