The Experts below are selected from a list of 300 Experts worldwide ranked by ideXlab platform
John E Mayer - One of the best experts on this subject based on the ideXlab platform.
-
creation of a brachial arteriovenous fistula for treatment of pulmonary arteriovenous malformations after cavopulmonary anastomosis
The Annals of Thoracic Surgery, 2005Co-Authors: Doff B. Mcelhinney, Audrey C Marshall, P Lang, James E Lock, John E MayerAbstract:Background Pulmonary arteriovenous malformations (PAVMs) occur in approximately 20% of patients after unidirectional superior cavopulmonary anastomosis (CPA), and frequently after bidirectional CPA in patients with Polysplenia syndrome. It is hypothesized that exclusion of a growth-modulating factor produced in the liver may predispose to PAVM formation. Resolution of PAVMs after inclusion of hepatic venous effluent into the cavopulmonary circulation has been reported. An upper extremity systemic arteriovenous (AV) fistula may be created to augment pulmonary blood flow and improve oxygenation in hypoxemic patients with CPA, but there has been no systematic investigation of the effects of such fistulas on PAVMs after CPA. Methods We studied 11 patients with PAVMs who underwent creation of a brachial AV fistula a median of 11 years after CPA. Results Eight patients had discontinuous pulmonary arteries or unilateral flow of a bidirectional CPA and were not considered good candidates for Fontan completion; the other 3 patients had Polysplenia and unilateral hepatic venous streaming after Fontan completion. Three patients died of progressive complications of their heart disease 4 to 18 months after AV fistula creation. Pulmonary arteriovenous malformations resolved after creation of a brachial AV fistula in 4 of 5 surviving patients with unilateral flow of a superior CPA, but in none of 3 patients with Polysplenia who had unilateral hepatic venous streaming after Fontan completion and PAVMs in the contralateral lung. Conclusions These findings are consistent with the "hepatic factor" hypothesis, according to which the development of PAVMs is facilitated when an unidentified factor produced or metabolized in the liver does not reach the pulmonary circulation before traversing another capillary bed. Patients with unilateral superior CPA flow and PAVMs who are not considered candidates for Fontan completion may benefit from a brachial AV fistula.
Doff B. Mcelhinney - One of the best experts on this subject based on the ideXlab platform.
-
Congenital Portosystemic Venous Connections and Other Abdominal Venous Abnormalities in Patients with Polysplenia and Functionally Univentricular Heart Disease: A Case Series and Literature Review
Congenital Heart Disease, 2011Co-Authors: Doff B. Mcelhinney, Gerald R. Marx, Jane W NewburgerAbstract:Objective. Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially important in patients with Polysplenia syndrome. Our objective was to investigate the frequency and range of portal and other abdominal systemic venous anomalies in patients with Polysplenia and inferior vena cava (IVC) interruption who underwent a cavopulmonary anastomosis procedure at our center, and to review the published literature on this topic and the potential clinical importance of such anomalies. Design. Retrospective cohort study and literature review were used. Results. Among 77 patients with heterotaxy, univentricular heart disease, and IVC interruption who underwent a bidirectional Glenn and/or modified Fontan procedure, pulmonary arteriovenous malformations were diagnosed in 33 (43%). Bilateral superior vena cavas were present in 42 patients (55%). Despite inadequate imaging in many patients, a partial PSVC, dual IVCs, and/or renal vein anomalies were detected in 15 patients (19%). A PSVC formed by a tortuous vessel running from the systemic venous system to the extrahepatic portal vein was found in six patients (8%). Abdominal venous anomalies other than PSVC were documented in 13 patients (16%), including nine (12%) with some form of duplicated IVC system, with a large azygous vein continuing to the superior vena cava and a parallel, contralateral IVC of similar or smaller size, and seven with renal vein anomalies. In patients with a partial PSVC or a duplicate IVC that connected to the atrium, the abnormal connection allowed right-to-left shunting. Conclusions. PSVC and other abdominal venous anomalies may be clinically important but under-recognized in patients with IVC interruption and univentricular heart disease. In such patients, preoperative evaluation of the abdominal systemic venous system may be valuable. More data are necessary to determine whether there is a pathophysiologic connection between the Polysplenia variant of heterotaxy, PSVC, and cavopulmonary anastomosis-associated pulmonary arteriovenous malformations.
-
creation of a brachial arteriovenous fistula for treatment of pulmonary arteriovenous malformations after cavopulmonary anastomosis
The Annals of Thoracic Surgery, 2005Co-Authors: Doff B. Mcelhinney, Audrey C Marshall, P Lang, James E Lock, John E MayerAbstract:Background Pulmonary arteriovenous malformations (PAVMs) occur in approximately 20% of patients after unidirectional superior cavopulmonary anastomosis (CPA), and frequently after bidirectional CPA in patients with Polysplenia syndrome. It is hypothesized that exclusion of a growth-modulating factor produced in the liver may predispose to PAVM formation. Resolution of PAVMs after inclusion of hepatic venous effluent into the cavopulmonary circulation has been reported. An upper extremity systemic arteriovenous (AV) fistula may be created to augment pulmonary blood flow and improve oxygenation in hypoxemic patients with CPA, but there has been no systematic investigation of the effects of such fistulas on PAVMs after CPA. Methods We studied 11 patients with PAVMs who underwent creation of a brachial AV fistula a median of 11 years after CPA. Results Eight patients had discontinuous pulmonary arteries or unilateral flow of a bidirectional CPA and were not considered good candidates for Fontan completion; the other 3 patients had Polysplenia and unilateral hepatic venous streaming after Fontan completion. Three patients died of progressive complications of their heart disease 4 to 18 months after AV fistula creation. Pulmonary arteriovenous malformations resolved after creation of a brachial AV fistula in 4 of 5 surviving patients with unilateral flow of a superior CPA, but in none of 3 patients with Polysplenia who had unilateral hepatic venous streaming after Fontan completion and PAVMs in the contralateral lung. Conclusions These findings are consistent with the "hepatic factor" hypothesis, according to which the development of PAVMs is facilitated when an unidentified factor produced or metabolized in the liver does not reach the pulmonary circulation before traversing another capillary bed. Patients with unilateral superior CPA flow and PAVMs who are not considered candidates for Fontan completion may benefit from a brachial AV fistula.
Suk Kim - One of the best experts on this subject based on the ideXlab platform.
-
Polysplenia Syndrome with Congenital Agenesis of Dorsal Pancreas Presenting as Acute Pancreatitis and the Role of Endoscopic Ultrasonography in Its Diagnosis
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi, 2012Co-Authors: Jae Hoon Jeong, Gwang Ha Kim, Geun Am Song, Dong Gun Lee, Ji Yoon Moon, Jae Hoon Cheong, Suk KimAbstract:A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as Polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of Polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.
-
Polysplenia syndrome with preduodenal portal vein detected in adults
World Journal of Gastroenterology, 2008Co-Authors: Hyungil Seo, Tae Yong Jeon, Mun Sup Sim, Suk KimAbstract:Polysplenia syndrome, defined as the presence of multiple spleens of almost equal volume, is a rare condition involving congenital anomalies in multiple organ systems. We report this anomaly in a 41-year-old female who underwent a left lateral sectionectomy due to recurrent cholangitis and impacted left lateral duct stones. Polysplenia syndrome with preduodenal vein was diagnosed preoperatively by computed tomography (CT) and surgery was done safely. Although the Polysplenia syndrome with preduodenal portal vein (PDPV) in adult is rarely encountered, surgeons need to understand the course of the portal vein and exercise caution in approaching the biliary tract.
Alejandro Aris - One of the best experts on this subject based on the ideXlab platform.
-
Complex Cardiac Surgery in a Patient With Dextrocardia and Polysplenia
Revista espanola de cardiologia, 2005Co-Authors: Javier Cobiella, Christian Muñoz, Alejandro ArisAbstract:Dextrocardia with Polysplenia is one of a large spectrum of situs anomalies. In most cases, there are associated cardiovascular abnormalities. We report the case of a patient with dextrocardia, Polysplenia, situs ambiguous, and interruption of the inferior vena cava, with an azygos vein continuation. He underwent surgery to replace the aortic valve and the ascending aorta, and to insert a double coronary artery bypass graft.
Alan Norman Langnas - One of the best experts on this subject based on the ideXlab platform.
-
biliary atresia Polysplenia syndrome surgical and clinical relevance in liver transplantation
Annals of Surgery, 1998Co-Authors: Gustavo Varelafascinetto, P Castaldo, Ira J Fox, Debra L Sudan, Thomas G Heffron, Byers W Shaw, Alan Norman LangnasAbstract:ObjectiveTo review a single center's 10-year experience with liver transplantation (LTx) for the biliary atresia-Polysplenia syndrome (BA-PS) and to define surgical and clinical guidelines for its management.Summary Background DataBA is the most common indication for pediatric liver transplantatio
