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Hepatic factor pulmonary arteriovenous malformation

Joseph J Vettukattil
BACKGROUND: The "hepatic factor," a molecule or group of molecules present in the hepatic venous blood, essential for the prevention of the development of pulmonary arteriovenous malformations (PAVMs) and right-to-left shunting has been a conceptual enigma in the understanding of many related conditions. METHODS: Patients with various forms of liver diseases including acute hepatic failure, and others with normal hepatic function like hereditary hemorrhagic telangiectasia (HHT), inflammatory and parasitic disorders, cardiogenic hepatopulmonary syndrome (cHPS) and skin disorders like Dyskeratosis congenita are all known to cause PAVMs...
2017: Current Cardiology Reviews
Mary Hunt Martin, Jeffrey A Feinstein, Frandics P Chan, Alison L Marsden, Weiguang Yang, V Mohan Reddy
OBJECTIVES: To demonstrate the technical feasibility and describe intermediate outcomes for the initial patients undergoing handcrafted, area-preserving, Y-graft modification of the Fontan procedure. METHODS: A retrospective review of a pilot study was undertaken to describe preoperative, intraoperative, and postoperative results. RESULTS: Six patients underwent successful procedures and remain alive 3 to 4 years later. The median age at operation was 3...
January 2015: Journal of Thoracic and Cardiovascular Surgery
Muhammed Riyas K Rahmath, Faraz Masud, Ahmad Sallehuddin, Reema Yousef Kamal, Akhlaque Nabi Bhat, Muhammad Dilawar
INTRODUCTION: Systemic venous collaterals have been found at different stages of single-ventricle palliation, specifically after Kawashima operation. In this study, we present the incidence, clinical features, associated risk factors, and management of such venovenous collaterals (VVCs) after Kawashima procedure. MATERIALS AND METHODS: A retrospective review of all the patients who underwent Kawashima procedure prior to December 2011 at Hamad General Hospital, Qatar, was performed...
January 1, 2014: World Journal for Pediatric & Congenital Heart Surgery
María González-Núñez, José M Muñoz-Félix, José M López-Novoa
Activin receptor-like kinase-1 or ALK-1 is a type I cell surface receptor for the transforming growth factor-β (TGF-β) family of proteins. The role of ALK-1 in endothelial cells biology and in angiogenesis has been thoroughly studied by many authors. However, it has been recently suggested a possible role of ALK-1 in cardiovascular homeostasis. ALK-1 is not only expressed in endothelial cells but also in smooth muscle cells, myofibroblast, hepatic stellate cells, chondrocytes, monocytes, myoblasts, macrophages or fibroblasts, but its role in these cells have not been deeply analyzed...
October 2013: Biochimica et Biophysica Acta
Bernardo Sopeña, Ma Teresa Pérez-Rodríguez, Daniel Portela, Alberto Rivera, Mayka Freire, César Martínez-Vázquez
OBJECTIVE: Hereditary hemorrhagic telangiectasia (HHT) is a vascular disorder causing mucocutaneous telangiectases and visceral arteriovenous malformations (AVMs). Pulmonary hypertension (PH) is considered an uncommon complication of HHT whose impact on the survival of these patients is currently unknown. METHODS: From January 1995 to December 2008, 29 hospitalized patients with definite HHT were included and followed until January 2011. Data on demographics, clinical symptoms and survival were recorded...
April 2013: European Journal of Internal Medicine
Polakit Teekakirikul, Dianna M Milewicz, David T Miller, Ronald V Lacro, Ellen S Regalado, Ana Maria Rosales, Daniel P Ryan, Tomi L Toler, Angela E Lin
Dilation or aneurysm of the ascending aorta can progress to acute aortic dissection (Thoracic Aortic Aneurysms and Aortic Dissections, TAAD). Mutations in genes encoding TGF-β-related proteins (TGFBR1, TGFBR2, FBN1, and SMAD3) cause syndromic and inherited TAAD. SMAD4 mutations are associated with juvenile polyposis syndrome (JPS) and a combined JPS-hereditary hemorrhagic telangiectasia (HHT) known as JPS-HHT. A family with JPS-HHT was reported to have aortic root dilation and mitral valve abnormalities. We report on two patients with JPS-HHT with SMAD4 mutations associated with thoracic aortic disease...
January 2013: American Journal of Medical Genetics. Part A
Aida Field-Ridley, Ritva Heljasvaara, Taina Pihlajaniemi, Ian Adatia, Christine Sun, Roberta L Keller, Wen Hui Gong, Sanjeev Datar, Peter Oishi, Jeffrey R Fineman
Pulmonary arteriovenous malformations (PAVMs) are a common source of morbidity after bidirectional superior cavopulmonary anastomosis (Glenn). The diversion of hepatic venous effluent away from the pulmonary circulation after Glenn appears to play a significant role in the pathogenesis of PAVMs. Although the liver is known to produce factors that regulate vascular development, specific hepatic inhibitors of angiogenesis have not been described in the post-Glenn population. Endostatin, produced from its precursor collagen XVIII, is a potent inhibitor of angiogenesis produced by the liver...
February 2013: Pediatric Cardiology
Fumihiro Ogawa, Kagami Miyaji, Masahiro Ishii, Akira Iyoda, Yukitoshi Satoh
Because the cavopulmonary shunt procedure is widely used for palliation of complex congenital heart diseases, pulmonary arteriovenous malformations (PAVMs) are relatively well-known complications. The reported patient was a 23-year-old woman who experienced PAVMs in the right lower lobe after a classical Glenn anastomosis and Björk procedure for tricuspid atresia. Her arterial oxygen saturation (SaO2) 14 years after the Björk procedure was ~80 %. She then underwent a total cavopulmonary connection (TCPC) conversion to reduce her PAVMs in the right lower lobe using the "hepatic factor...
March 2013: Pediatric Cardiology
Jessica M Fleagle, Ravi K Bobba, Carl G Kardinal, Carl E Freter
Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant condition associated with arteriovenous malformations (AVMs) or telangiectasias of the pulmonary, gastrointestinal or hepatic circulations. The authors present a case of a 52-year-old woman with a known diagnosis of HHT who presented for evaluation of anemia. She had an extensive history of iron sucrose infusions, frequent blood transfusions and hospitalizations for anemia related to gastrointestinal bleeding and epistaxis. The patient was treated with bevacizumab at a dose of 5 mg/kg infusion every 2 weeks for 4 cycles...
March 2012: American Journal of the Medical Sciences
Anne Vollebregt, Kuberan Pushparajah, Maleeha Rizvi, Andreas Hoschtitzky, David Anderson, Conal Austin, Shane M Tibby, John Simpson
OBJECTIVES: Patients with left atrial isomerism and interrupted inferior vena cava palliated with a superior cavopulmonary connection or Kawashima procedure (KP) have a high incidence of developing pulmonary arteriovenous malformations. The necessity for hepatic vein redirection (HVR) and its timing remains a controversy. We aimed to assess the clinical outcome of patients with left atrial isomerism following a KP. The main end points were death, requirement for HVR and the impact of HVR on oxygen saturation...
March 2012: European Journal of Cardio-thoracic Surgery
Michael Ruygrok, Brandon Combs, Jessica Campbell, Mary Maher
No abstract text is available yet for this article.
July 2011: American Journal of Medicine
Sonay Incesoy Özdemir, Ceyhun Bozkurt, Utku Arman Orün, Gürses Sahin, Nazmiye Yüksek, Semra Cetinkaya, Ulya Ertem
No abstract text is available yet for this article.
March 2011: Anadolu Kardiyoloji Dergisi: AKD, the Anatolian Journal of Cardiology
Doff B McElhinney, Gerald R Marx, Audrey C Marshall, John E Mayer, Pedro J Del Nido
OBJECTIVE: Pulmonary arteriovenous malformations are an important but uncommon complication of cavopulmonary connection, particularly in patients with heterotaxy. Absence of hepatic venous effluent in pulmonary arterial blood seems to be a predisposing factor. Pulmonary arteriovenous malformations are most common after superior cavopulmonary anastomosis, but may develop, progress, or persist in 1 lung after Fontan completion if hepatic venous blood streams completely or primarily to the contralateral lung...
June 2011: Journal of Thoracic and Cardiovascular Surgery
S Dupuis-Girod, S Bailly, H Plauchu
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by severe and recurrent nosebleeds, mucocutaneous telangiectases, and, in some cases, life-threatening visceral arteriovenous malformations of various types, including pulmonary, hepatic, cerebral, and spinal. Gastrointestinal telangiectases are frequent and may cause severe bleeding. HHT type 1 results from mutations in ENG on chromosome 9 (coding for endoglin), and HHT type 2 results from mutations in ACVRL1 on chromosome 12 (coding for activin receptor-like kinase 1)...
July 2010: Journal of Thrombosis and Haemostasis: JTH
Marwa Mahmoud, Kathleen R Allinson, Zhenhua Zhai, Rachael Oakenfull, Pranita Ghandi, Ralf H Adams, Marcus Fruttiger, Helen M Arthur
RATIONALE: Arteriovenous malformations (AVMs) result in anomalous direct blood flow between arteries and veins, bypassing the normal capillary bed. Depending on size and location, AVMs may lead to severe clinical effects including systemic cyanosis (pulmonary AVMs), hemorrhagic stroke (cerebral AVMs) and high output cardiac failure (hepatic AVMs). The factors leading to AVM formation are poorly understood, but patients with the familial disease hereditary hemorrhagic telangiectasia (HHT) develop AVMs at high frequency...
April 30, 2010: Circulation Research
Henri Plauchu, Sophie Dupuis-Girod
Hereditary hemorrhagic telangiectasia (HTT, or Osler-Weber-Rendu disease) is a constitutional vascular dysplasia characterized by hemorrhagic manifestations, cutaneous or mucosal telangiectases and visceral shunting due to arteriovenous malformations. In France, its prevalence varies from 1/6,000 to 1/8,500. The diagnosis is clinical and based on the common triad: chronic epistaxis, responsible for anemias, cutaneous or mucosal telangiectases (on typical locations) and the hereditary nature of these signs inheredited in an autosomal dominant way...
September 20, 2009: La Revue du Praticien
I-Hui Wu, Khanh H Nguyen
The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery...
July 2006: Pediatric Cardiology
Martine Remy-Jardin, Philippe Dumont, Pierre-Yves Brillet, Philippe Dupuis, Alain Duhamel, Jacques Remy
PURPOSE: To retrospectively evaluate the long-term results of transcatheter embolotherapy of pulmonary arteriovenous malformations (PAVMs) with helical computed tomography (CT). MATERIALS AND METHODS: Neither institutional review board approval nor patient consent was required for this retrospective study. Thirty-eight patients underwent follow-up helical chest CT 2-21 years after successful embolotherapy of 64 PAVMs. Four outcome categories were analyzed on the basis of the PAVM morphologic changes and perfusion findings seen on CT angiograms: successful treatment (marked reduction or disappearance of the aneurysmal sac), partially successful treatment (reduced size of the aneurysmal sac and pulmonary vessels, with feeding artery[ies] less than 3 mm in diameter, deemed too small to be occluded), partially failed treatment (reduced size of the aneurysmal sac and pulmonary vessels, with feeding artery[ies] larger than 3 mm and additional embolotherapy required), and failed treatment (similar size of or interim growth in the aneurysmal sac, with unchanged or enlarged pulmonary vessels)...
May 2006: Radiology
Doff B McElhinney, Audrey C Marshall, Peter Lang, James E Lock, John E Mayer
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...
November 2005: Annals of Thoracic Surgery
Nancy A Pike, Luca A Vricella, Jeffrey A Feinstein, Michael D Black, Bruce A Reitz
Although previously described in patients undergoing staged palliation for univentricular heart disease, the mechanism by which hepatic venous flow prevents development of pulmonary arteriovenous malformations is still not completely understood. We present a case in which successful H-type Fontan revision with rerouting of hepatic venous flow through a hemiazygous vein successfully reversed the progression of severe left pulmonary arteriovenous malformations.
August 2004: Annals of Thoracic Surgery
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