scholarly journals sVEGFR1 Is Enriched in Hepatic Vein Blood—Evidence for a Provisional Hepatic Factor Candidate?

2021 ◽  
Vol 9 ◽  
Author(s):  
Andrew D. Spearman ◽  
Ankan Gupta ◽  
Amy Y. Pan ◽  
Todd M. Gudausky ◽  
Susan R. Foerster ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Hepatic Vein ◽  
Congenital Heart ◽  
Superior Vena ◽  
Vena Cava ◽  
Tube Length ◽  
Blood Samples ◽  
Independent Protein

Background: Pulmonary arteriovenous malformations (PAVMs) are common sequelae of palliated univentricular congenital heart disease, yet their pathogenesis remain poorly defined. In this preliminary study, we used paired patient blood samples to identify potential hepatic factor candidates enriched in hepatic vein blood.Methods: Paired venous blood samples were collected from the hepatic vein (HV) and superior vena cava (SVC) from children 0 to 10 years with univentricular and biventricular congenital heart disease (n = 40). We used three independent protein analyses to identify proteomic differences between HV and SVC blood. Subsequently, we investigated the relevance of our quantified protein differences with human lung microvascular endothelial assays.Results: Two independent protein arrays (semi-quantitative immunoblot and quantitative array) identified that soluble vascular endothelial growth factor receptor 1 (sVEGFR1) is significantly elevated in HV serum compared to SVC serum. Using ELISA, we confirmed the previous findings that sVEGFR1 is enriched in HV serum (n = 24, p < 0.0001). Finally, we studied the quantified HV and SVC serum levels of sVEGFR1 in vitro. HV levels of sVEGFR1 decreased tip cell selection (p = 0.0482) and tube formation (fewer tubes [p = 0.0246], shorter tube length [p = 0.0300]) in vitro compared to SVC levels of sVEGFR1.Conclusions: Based on a small heterogenous cohort, sVEGFR1 is elevated in HV serum compared to paired SVC samples, and the mean sVEGFR1 concentrations in these two systemic veins cause pulmonary endothelial phenotypic differences in vitro. Further research is needed to determine whether sVEGFR1 has a direct role in pulmonary microvascular remodeling and PAVMs in patients with palliated univentricular congenital heart disease.

scholarly journals Use of Superior Vena Cava-Right Pulmonary Artery Anastomosis in Congenital Heart Disease with Decreased Pulmonary Blood Flow

Circulation ◽  
1969 ◽  
Vol 40 (6) ◽  
pp. 777-784 ◽  
Author(s):  
IRWIN B. BORUCHOW ◽  
THOMAS D. BARTLEY ◽  
LARRY P. ELLIOTT ◽  
MYRON W. WHEAT ◽  
L. JEROME KROVETZ ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Blood Flow ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Superior Vena Cava ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Superior Vena ◽  
Vena Cava ◽  
Right Pulmonary Artery

Cerebral NIRS as a marker of superior vena cava oxygen saturation in neonates with congenital heart disease

2010 ◽  
Vol 20 (11) ◽  
pp. 1040-1045 ◽  
Author(s):  
ZACCARIA RICCI ◽  
CRISTIANA GARISTO ◽  
ISABELLA FAVIA ◽  
ULRIKE SCHLODERER ◽  
CHIARA GIORNI ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Oxygen Saturation ◽  
Superior Vena Cava ◽  
Congenital Heart ◽  
Superior Vena ◽  
Vena Cava

CHONDROECTODERMAL DYSPLASIA (ELLIS—VAN CREVELD SYNDROME) WITH A COMPLEX CARDIAC MALFORMATION

PEDIATRICS ◽  
1961 ◽  
Vol 28 (2) ◽  
pp. 285-292
Author(s):  
George S. Husson ◽  
Paul Parkman
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Venous Return ◽  
Ectodermal Dysplasia ◽  
Superior Vena ◽  
Vena Cava ◽  
Left Superior Vena Cava ◽  
Single Atrium ◽  
Ellis Van Creveld Syndrome

A 4-month-old girl with chondroectodermal dysplasia (dwarfism, bimanual polydactyly and ectodermal dysplasia) (Ellisvan Creveld syndrome) and congenital heart disease, consisting of anamalous pulmonary venous return, single atrium and a persistent left superior vena cava, is described. A review of the literature concerning chondroectodermal dysplasia reveals an incidence of congenital heart disease in more than 50% of patients.

scholarly journals Pulmonary Arterial Stenting Complication: A Case Report about the Retrieval of a Stent Lost in a Child with Bilateral Glenn Shunt

2018 ◽  
pp. 1-4
Author(s):  
M Giordano ◽  
G Gaio ◽  
MC Bigazzi ◽  
MG Russo
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Superior Vena ◽  
Vena Cava ◽  
Lower Lobe ◽  
Complex Congenital Heart Disease ◽  
Univentricular Heart ◽  
Complex Anatomy

Percutaneous stenting of the pulmonary artery is a diffuse procedure, above all in patients with congenital heart disease. The effectiveness of the procedure is associated with potential and feared complications. This case describes the embolization of a premounted stent into the left lower lobe pulmonary artery in a 3-years old patient with univentricular heart and left-side superior vena cava undergone to Norwood procedure modified according to Sano (stage I) and then bilateral cavo-pulmonary anastomosis according to Glenn (stage II). The retrieval procedures were complicated by the severe hypoplasia of the left pulmonary artery and by the complex anatomy of the patient. This case shows how to pull-back an embolized stent in a child with a complex congenital heart disease despite the limitations related to the low weight and the complex vascular anatomy.

Additional superior vena cava combined with abnormal inflow of the hepatic vein

2016 ◽  
Vol 26 (7) ◽  
pp. 566-569
Author(s):  
Leo Antonovich Bockeria ◽  
Olga Leonidovna Bockeria ◽  
Merab Konstantinovich Sanakoev ◽  
Vladimir Alexandrovich Shvartz ◽  
Tatyana Georgievna Le
Keyword(s):  
Congenital Heart Disease ◽  
Superior Vena Cava ◽  
Hepatic Vein ◽  
Congenital Heart ◽  
Septal Defect ◽  
Superior Vena ◽  
Vena Cava ◽  
Valve Regurgitation ◽  
Tricuspid Valve Regurgitation ◽  
Left Hepatic Vein

We represent a case of successful surgical treatment of a rare congenital heart disease: abnormal inflow of an additional superior vena cava into the left atrium, combined with atrial septal defect, mitral and tricuspid valve regurgitation, and abnormal inflow of the left hepatic vein into a roofless coronary sinus.

scholarly journals Ablation of Atrial Fibrillation in Patients with Congenital Heart Disease

2017 ◽  
Vol 6 (4) ◽  
pp. 191 ◽  
Author(s):  
Marwan M Refaat ◽  
Jad Ballout ◽  
Moussa Mansour ◽  
◽  
◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Catheter Ablation ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Superior Vena ◽  
Vena Cava ◽  
Surgical Techniques ◽  
Ebstein Anomaly ◽  
Increased Risk

With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF. Similar to the general population, the risk of AF increases with advancing age, and is mainly secondary to the abnormal anatomy, abnormal pressure and volume parameters in the hearts of these patients and to the increased scarring and inflammation seen in the left atrium following multiple surgical procedures. Catheter ablation for AF has been shown to be a very effective treatment modality in patients with refractory AF. However, data and guidelines regarding catheter ablation in patients with congenital heart disease are not well established. This review will shed light on the procedural techniques, success rates and complications of AF catheter ablation in patients with different types of CHD, including atrial septal defects, tetralogy of Fallot, persistent left superior vena cava, heterotaxy syndrome and atrial isomerism, and Ebstein anomaly.

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