Pulmonary Arteriovenous Malformations

[Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review]

Tian Fengyan, Dong Xiao, Hou Xiaohan, Yuan Ruyue, Pan Yuanwei, Zhang DaFirst Affiliated Hospital of Zhengzhou University. China Zhonghua Er Ke Za Zhi 2024; 62: 71-75DOI: 10.3760/cma.j.cn112140-20230828-00139 AbstractObjective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the […]

[Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review] Read More »

In vivo hepatic flow distribution by computational fluid dynamics can predict pulmonary flow distribution in patients with Fontan circulation

Petter Frieberg, Pia Sjöberg, Erik Hedström, Marcus Carlsson, Petru LiubaLund University and Skåne University Hospital.Sweden Scientific ReportsSci Rep 2023; 13:DOI: 10.1038/s41598-023-45396-6 AbstractIn Fontan patients, a lung deprived of hepatic blood may develop pulmonary arterio-venous malformations (PAVMs) resulting in shunting, reduced pulmonary vascular resistance (PVR) and decreased oxygenation. To provide guidance for corrective invasive interventions, we aimed

In vivo hepatic flow distribution by computational fluid dynamics can predict pulmonary flow distribution in patients with Fontan circulation Read More »

Pregnancy and delivery after percutaneous embolization with a combination of microvascular plugs and hydrogel-coated coils for unilateral diffuse pulmonary arteriovenous malformations: a case report

Kayo Ogino, Tomohiro Hayashi, Kenji Waki, Masanobu OhyaKurashiki Central Hospital.Japan European Heart Journal Case ReportsEur Heart J Case Rep 2023; 7:DOI: 10.1093/ehjcr/ytad468 AbstractBackground: Pulmonary arteriovenous malformations (PAVMs) are abnormal communications between the pulmonary arteries and veins; right-to-left shunts can cause hypoxaemia, emboli to systemic circulation, and brain abscesses. Cyanosis during pregnancy may increase the probability of premature

Pregnancy and delivery after percutaneous embolization with a combination of microvascular plugs and hydrogel-coated coils for unilateral diffuse pulmonary arteriovenous malformations: a case report Read More »

Prevalence and Predictors of Hereditary Hemorrhagic Telangiectasia and Capillary-Malformation Arteriovenous Malformation Syndrome Among Children with Neurovascular Malformations

Elissa R. Engel, Katie Wusik, Philip Bright, Sudhakar Vadivelu, J. Michael Taylor, Adrienne HammelUniversity of Cincinnati College of Medicine and Cincinnati Children’s Hospital Medical Center. University of Kentucky College of Medicine.United States Journal of PediatricsJ Pediatr 2023; DOI: 10.1016/j.jpeds.2023.113761 AbstractObjective: To investigate the prevalence and predictors of hereditary hemorrhagic telangiectasia (HHT) and capillary-malformation arteriovenous malformation (CM-AVM) syndrome

Prevalence and Predictors of Hereditary Hemorrhagic Telangiectasia and Capillary-Malformation Arteriovenous Malformation Syndrome Among Children with Neurovascular Malformations Read More »

Longitudinal Assessment of Curaçao Criteria in Children with Hereditary Hemorrhagic Telangiectasia

Mordechai Pollak, Dvir Gatt, Michelle Shaw, Sheryl Hewko, Anthony Lamanna, Sara Santos, Felix RatjenHospital for Sick Children and Ruth Rappaport Children’s Hospital.Canada Journal of PediatricsJ Pediatr 2023; DOI: 10.1016/j.jpeds.2023.113665 AbstractObjective: To assess the utility of the Curaçao criteria by age over time in children with hereditary hemorrhagic telangiectasia (HHT).Study design: This was a single-center, retrospective analysis of patients

Longitudinal Assessment of Curaçao Criteria in Children with Hereditary Hemorrhagic Telangiectasia Read More »

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies

Jinrong Liu, Xiaomin Duan, Jie Yin, Haiming Yang, Ruxuan He, Shunying ZhaoBeijing Children’s Hospital, National Center for Children’s Health and Capital Medical University.China Orphanet Journal of Rare DiseasesOrphanet J Rare Dis 2023; 18:DOI: 10.1186/s13023-023-02732-3 AbstractPulmonary arteriovenous malformations (PAVMs), particularly where feeding artery/arteries to PAVMs ≥ 3 mm can be treated with embolization. The treatment for hypoxemia

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies Read More »

Case report of juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome: first report in Korea with a novel mutation in the SMAD4 gene

Ben Kang, Su-Kyeong Hwang, Sujin Choi, Eun Soo Kim, Sang Yub Lee, Chang-Seok Ki, Eun-Hae Cho, Ji-Hyuk Lee, Byung-Ho ChoeKyungpook National University. GC Genome. Chungbuk National University College of Medicine.Republic of Korea Translational PediatricsTransl Pediatr 2021; 10: 1369-1376DOI: 10.21037/tp-21-12 AbstractJuvenile polyposis/hereditary hemorrhagic telangiectasia (JPS/HHT) syndrome is a rare, autosomal dominant disorder caused by mutations in the SMAD4 gene,

Case report of juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome: first report in Korea with a novel mutation in the SMAD4 gene Read More »

Homozygous GDF2 nonsense mutations result in a loss of circulating BMP9 and BMP10 and are associated with either PAH or an “HHT-like” syndrome in children

Joshua Hodgson, Lidia Ruiz- Llorente, Jamie McDonald, Oliver Quarrell, Kelechi Ugonna, James Bentham, Rebecca Mason, Jennifer Martin, David Moore, Katie Bergstrom, Pinar Bayrak- Toydemir, Whitney Wooderchak-Donahue, Nicholas W. Morrell, Robin Condliffe, Carmelo Bernabeu, Paul D. UptonUniversity of Cambridge. Centro de Investigaciones Biológicas Margarita Salas, Consejo Superior de Investigaciones Científicas (CSIC), and Centro de Investigación Biomédica en Red de Enfermedades Raras. University of Alcalá, Madrid. University of Utah. Sheffield Children’s Hospital. Leeds

Homozygous GDF2 nonsense mutations result in a loss of circulating BMP9 and BMP10 and are associated with either PAH or an “HHT-like” syndrome in children Read More »

Pulmonary arteriovenous malformation in a child with multiple brain abscesses: A case report

S. T. Hlophe, R. MasekelaNelson R Mandela School of Clinical Medicine and University of KwaZulu-Nata.South Africa African Journal of Thoracic and Critical Care Medicine Afr J Thorac Crit Care Med 2021; 27: DOI: 10.7196/AJTCCM.2021.v27i1.109 AbstractPulmonary arteriovenous malformations (PAVMs) are caused by abnormal connections between arteries and veins, which lead to right-to-left shunting of deoxygenated blood. Here, we

Pulmonary arteriovenous malformation in a child with multiple brain abscesses: A case report Read More »

Abnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation

Carlo R. Bartoli, Samson Hennessy-Strahs, Robert D. Dowling, J. William Gaynor, Andrew C. GlatzHospital of the University of Pennsylvania, Children’s Hospital of Philadelphia and University of Pennsylvania. Penn State University College of Medicine.United States Journal of the American College of Cardiology Basic Translational ScienceJACC Basic Transl Sci 2021; 6: 222-235DOI: 10.1016/j.jacbts.2020.12.014 AbstractChildren with a bidirectional superior

Abnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation Read More »

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