1,286 results match your criteria Pulmonary Venoocclusive Disease


Feasibility of Assessing Cryoballoon Pulmonary Vein Occlusion With Saline Injection and a Novel Mapping System.

JACC Clin Electrophysiol 2022 Jun;8(6):795-799

Wexner Medical Center, Ohio State University, Columbus, Ohio, USA.

Thirty-eight patients had assessment of pulmonary vein occlusion with the dielectric mapping system and injection of saline as an alternative to contrast. Contrast injection was required to ascertain pulmonary vein occlusion in 31.6% (12 of 38) of subjects and 17. Read More

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Screening for pulmonary veno-occlusive disease in heterozygous variant carriers.

Eur Respir J 2022 Jun 16. Epub 2022 Jun 16.

Assistance Publique - Hôpitaux de Paris (AP-HP), Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, DMU 5 Thorinno, Hôpital Bicêtre, Le Kremlin-Bicêtre, France

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Pulmonary Veno-occlusive Disease that Developed Following Hematopoietic Stem Cell Transplantation for Acute Myeloid Leukemia: A Case Report.

Intern Med 2022 Jun 14. Epub 2022 Jun 14.

Department of Respirology, Graduate School of Medicine, Chiba University, Japan.

We herein report a case of pulmonary veno-occlusive disease (PVOD) induced by allo-hematopoietic stem cell transplantation (HSCT) in a 48-year-old man who was diagnosed with acute myeloid leukemia. Five months after transplantation, he developed dyspnea and was diagnosed with pulmonary hypertension based on right heart catheterization. Although he received treatment with pulmonary vasodilators, diuretics, and corticosteroids, his pulmonary artery pressure did not decrease, and his pulmonary edema worsened. Read More

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A Systematic Review on Pulmonary Complications Secondary to Hematopoietic Stem Cell Transplantation.

Cureus 2022 May 7;14(5):e24807. Epub 2022 May 7.

Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.

The main purpose of this systematic review was to identify and synthesize evidence about pulmonary complications following stem cell transplantation to raise awareness among physicians since it is a lesser-known topic. Studies that included targeted pulmonary complications that occurred after stem cell transplantation; in humans; and were randomized controlled trials, cohort studies, and case studies between January 2011 and 2021. Fifteen intervention features were identified and analyzed in terms of their association with successful or unsuccessful interventions. Read More

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Clinical radiological and pathological staging of children with hypersensitivity pneumonitis.

Pediatr Pulmonol 2022 Jun 8. Epub 2022 Jun 8.

Department of Pediatric Pulmonology, Ihsan Dogramaci Children's Hospital, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Objective: Hypersensitivity pneumonitis (HP) is rare in the pediatric population. To date, there are no studies defining a correlation between clinical, radiological, and pathological findings in children with HP. The objective of this study is to define the clinical, and radiological characteristics and prognosis of childhood HP and to examine the clinical, radiological, and pathological correlation between HP stages. Read More

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A case of severe cancer-related pulmonary hypertension; An unexpected resolution.

Respir Med Case Rep 2022 23;38:101671. Epub 2022 May 23.

Division of Pulmonary and Critical Care Medicine, Albany Medical College, Albany, NY, USA.

Pulmonary hypertension (PH) in cancer patients can be caused by several mechanisms. It can be a direct cancer effect through pulmonary tumor thrombotic microangiopathy, pulmonary tumor emboli, extrinsic compression, intravascular tumors, or a secondary consequence of therapy, including chemotherapy agents, radiation, and stem cell transplantation. We present the first case of complete resolution of cancer-related PH. Read More

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A Systematic Review and Meta-Analysis of Studies of Defibrotide Prophylaxis for Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome.

Clin Drug Investig 2022 Jun 20;42(6):465-476. Epub 2022 May 20.

NOVEL Health Strategies, Bethesda, MD, USA.

Background And Objectives: Defibrotide is approved to treat severe veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) after haematopoietic cell transplantation in patients aged > 1 month in the European Union and for VOD/SOS with renal/pulmonary dysfunction post-haematopoietic cell transplantation in the United States. This meta-analysis estimated the incidence and risk of VOD/SOS after intravenous defibrotide prophylaxis using the published literature.

Methods: PubMed, Embase and Web of Science were searched through 30 November 2021 for defibrotide studies in VOD/SOS "prevention" or "prophylaxis," excluding phase I studies, case reports, studies with fewer than ten patients and reviews. Read More

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The role of cardiopulmonary exercise testing in identifying and monitoring pulmonary veno-oclusive disease: a case report with ING.

Eur Heart J Case Rep 2022 May 28;6(5):ytac138. Epub 2022 Mar 28.

Department of Cardiology, Hospital Universitario 12 de Octubre, Madrid, Spain.

Background: Pulmonary veno-occlusive disease (PVOD) is a subgroup of pulmonary arterial hypertension (PAH) where vascular remodelling affects mainly the post-capillary vessels. It is characterized by a particularly worse prognosis and by the risk of developing life-threatening pulmonary oedema, especially after PAH-targeted therapy. Therefore, suspicion of PVOD is crucial to guide the patient's management. Read More

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Prospective clinical assessment of patients with pulmonary arterial hypertension switched from bosentan to macitentan (POTENT).

Pulm Circ 2022 Apr 3;12(2):e12083. Epub 2022 May 3.

Department of Medicine, Pulmonary Hypertension Program King Faisal Specialist Hospital & Research Center Riyadh Saudi Arabia.

Even though pulmonary arterial hypertension (PAH) remains an incurable disease, the combination of PAH-specific therapies allowed evolving from symptom-based strategies to others aiming to move patients to low-risk conditions. Endothelin-1 (ET-1) receptor antagonists emerged as specific-PAH drugs that can be used in combination with other specific therapies. This work aimed to perform a prospective clinical assessment of patients with PAH that switched from bosentan to macitentan (POTENT), due to inadequate response. Read More

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Progression of pulmonary veno-occlusive disease without pulmonary hypertension.

Pulm Circ 2022 Jan 21;12(1):e12046. Epub 2022 Feb 21.

Department of Internal Medicine, Division of Pulmonary, Critical Care & Sleep Medicine Houston Methodist Lung Center Houston Texas USA.

Pulmonary veno-occlusive disease (PVOD) is a progressively fatal disease with no definitive treatment options. PVOD can be a result of genetic mutation but can also be due secondary to exposure to solvents or chemotherapeutic agents. Generally, at the time of diagnosis PVOD is associated with hemodynamically confirmed pulmonary hypertension (PH). Read More

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January 2022

Predicting Group II pulmonary hypertension: diagnostic accuracy of the H2FPEF and OPTICS scores in Scotland.

Open Heart 2022 04;9(1)

Scottish Pulmonary Vascular Unit, NHS Greater Glasgow and Clyde, Glasgow, UK.

Objective: Group II pulmonary hypertension (PH) can be challenging to distinguish from Group I PH without proceeding to right heart catheterisation (RHC). The diagnostic accuracy of the H2FPEF and OPTICS scores was investigated in Scotland.

Methods: Patients were included in the study if they were referred to the Scottish Pulmonary Vascular Unit between 2016 and 2020 and subsequently diagnosed with Group II PH or Group I PH which was either idiopathic, heritable or pulmonary veno-occlusive disease. Read More

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Sutureless Closure Versus Conventional Technique in the Primary Surgery of Total Anomalous Pulmonary Venous Connection: A Systematic Review and Meta-analysis.

Pediatr Cardiol 2022 Jun 15;43(5):943-951. Epub 2022 Apr 15.

Faculty of Public Health, University of Medicine and Pharmacy at Ho Chi Minh City, 217 Hong Bang, Ward 11, District 5, Ho Chi Minh City, Vietnam.

Sutureless closure has been used for primary repair of total anomalous pulmonary venous connection (TAPVC) for over 20 years but its superiority over conventional technique is still uncertain. This systematic review was conducted to compare the effectiveness of sutureless closure and conventional surgery as the primary repair for TAPVC. Systematic search was performed in June 2021 on 12 databases. Read More

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Diagnosis and Treatment of Pulmonary Arterial Hypertension: A Review.

JAMA 2022 04;327(14):1379-1391

Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

Importance: Pulmonary arterial hypertension (PAH) is a subtype of pulmonary hypertension (PH), characterized by pulmonary arterial remodeling. The prevalence of PAH is approximately 10.6 cases per 1 million adults in the US. Read More

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Favorable outcomes of patients with sickle cell disease hospitalized due to COVID-19: A report of three cases.

Exp Ther Med 2022 May 21;23(5):338. Epub 2022 Mar 21.

Department of Infectious Diseases-COVID-19 Unit, Laiko General Hospital, 11527 Athens, Greece.

Sickle cell disease (SCD) is one of the most frequent and severe monogenic disorders, affecting millions of individuals worldwide. SCD represents a fatal hematological illness, characterized by veno-occlusive events and hemolytic anemia. Hemolytic anemia is caused by abnormal sickle-shaped erythrocytes, which induce parenchymal destruction and persistent organ damage, resulting in considerable morbidity and mortality. Read More

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An Overview of Different Techniques for Improving the Treatment of Pulmonary Hypertension Secondary in Systemic Sclerosis Patients.

Diagnostics (Basel) 2022 Mar 1;12(3). Epub 2022 Mar 1.

Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-Under-Lyne OL6 9RW, UK.

In systemic sclerosis (SSc) mortality is mainly linked to lung involvement which is characterized by interstitial lung disease (ILD) and pulmonary hypertension (PH). In SSc, PH may be due to different etiologies, including ILD, chronic thromboembolic disease, pulmonary veno-occlusive disease, and pulmonary arterial hypertension (PAH). The main tool to screen PAH is transthoracic echocardiography (TTE), which has a sensitivity of 90%, even if definitive diagnosis should be confirmed by right heart catheterization (RHC). Read More

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Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.

J Card Surg 2022 Jun 21;37(6):1544-1549. Epub 2022 Mar 21.

Division of Pediatric and Congenital Heart Surgery, Memorial Hermann Hospital, Children's Heart Institute, University of Texas Health McGovern Medical School, Houston, Texas, USA.

Background: Total anomalous pulmonary venous connection (TAPVC) is a major risk factor in infants with single ventricle (SV). Exact definition of TAPVC anatomy is crucial for surgical planning.

Aim: To evaluate the role of cardiac computed tomography (CT) in this setting. Read More

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Dual-energy CT lung perfusion characteristics in pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH): preliminary experience in 63 patients.

Eur Radiol 2022 Jul 14;32(7):4574-4586. Epub 2022 Mar 14.

Univ Lille, CHU Lille, Department of Thoracic Imaging, Cardio-Pulmonary Institute, Boulevard Jules Leclercq, F-59000, Lille, France.

Background: In the stratification of potential causes of PH, current guidelines recommend performing V/Q lung scintigraphy to screen for CTEPH. The recognition of CTEPH is based on the identification of lung segments or sub-segments without perfusion but preserved ventilation. The presence of mismatched perfusion defects has also been described in a small proportion of idiopathic pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH). Read More

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Genetic cause of pulmonary veno-occlusive disease.

Lung India 2022 Mar-Apr;39(2):191-194

Department of Pediatrics, Narayana Health City, Bengaluru, Karnataka, India.

Pulmonary veno-occlusive disease (PVOD) is an important cause of pulmonary arterial hypertension (PAH) and is classified under idiopathic cause of PAH. Over a period of time, PVOD has been studied in detail in the western countries and various diagnostic criteria are formulated. Being a rapidly progressive disease, early diagnosis is of utmost importance which helps to initiate appropriate treatment. Read More

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Identification of a Novel EIF2AK Variant and Genetics-Assisted Approach to Diagnosis of Pulmonary Capillary Hemangiomatosis.

Lung 2022 04 6;200(2):217-219. Epub 2022 Mar 6.

Department of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA.

Pulmonary capillary hemangiomatosis (PCH) is an uncommon type of pulmonary vascular disease characterized by capillary proliferation and very poor prognosis owing to misdiagnosis and lack of effective therapeutic options. Mutations in the eukaryotic translation initiation factor 2α kinase 4 (EIF2AK4) gene have been reported in pulmonary veno-occlusive disease and PCH. In this report, we present a patient whose diagnosis of PCH was delayed by 2 ½ years despite prior surgical lung biopsy and clinical and laboratory findings suggestive of pulmonary hypertension. Read More

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Differential Expression Profile of microRNAs and Tight Junction in the Lung Tissues of Rat With Mitomycin-C-Induced Pulmonary Veno-Occlusive Disease.

Front Cardiovasc Med 2022 16;9:746888. Epub 2022 Feb 16.

Department of Respiratory and Critical Care Medicine, The Second Xiangya Hospital, Central South University, Changsha, China.

Background: Pulmonary veno-occlusive disease (PVOD) is characterized by increased pulmonary vascular resistance. Currently, there is a lack of effective treatment. It is of great significance to explore molecular targets for treatment. Read More

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February 2022

1,2-Dehydropyrrolizidine Alkaloids: Their Potential as a Dietary Cause of Sporadic Motor Neuron Diseases.

Chem Res Toxicol 2022 03 3;35(3):340-354. Epub 2022 Mar 3.

Poisonous Plant Research Laboratory, ARS/USDA, 1150 East 1400 North, Logan, Utah 84341, United States.

Sporadic motor neuron diseases (MNDs), such as amyotrophic lateral sclerosis (ALS), can be caused by spontaneous genetic mutations. However, many sporadic cases of ALS and other debilitating neurodegenerative diseases (NDDs) are believed to be caused by environmental factors, subject to considerable debate and requiring intensive research. A common pathology associated with MND development involves progressive mitochondrial dysfunction and oxidative stress in motor neurons and glial cells of the central nervous system (CNS), leading to apoptosis. Read More

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Pediatric pulmonary veno-occlusive disease associated with a novel BMPR2 variant.

Pediatr Pulmonol 2022 05 17;57(5):1366-1369. Epub 2022 Mar 17.

Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Pulmonary veno-occlusive disease (PVOD) and idiopathic/heritable pulmonary arterial hypertension (I/HPAH) cause progressive PH on the distinct genetic impact. A 29-month-old boy presented with a loss of consciousness. He had severe PH refractory to pulmonary vasodilators. Read More

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Veno-occlusive disease of the lung after allogeneic haematopoietic stem-cell transplantation: An autopsy study.

Pathol Res Pract 2022 Mar 11;231:153799. Epub 2022 Feb 11.

Institute of Pathology, Germany; Tissue Biobank, University Medical Center Mainz, Langenbeckstr. 1, 55101 Mainz, Germany.

Pulmonary veno-occlusive disease (pVOD) is a potentially life-threatening sequela of allogeneic haematopoietic stem-cell transplantation (alloHSCT). We conducted a morphometric evaluation of autopsy lung tissue to determine the incidence of pVOD and its association with donor type, conditioning regime, hepatic sinusoidal obstruction syndrome (hSOS), survival time, and graft versus host disease (GvHD). The degree of occlusion of pulmonary veins in 78 autopsy cases after alloHSCT and 12 control cases was assigned to one of the following categories: none, minor thickening of the intima (up to 33% narrowing), moderate pVOD wherein about half of the lumen (34-66%) is occluded, or advanced pVOD with near total or total (67-100%) obliteration of the lumen. Read More

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Review of surgical experience in 61 patients with mixed total anomalous pulmonary venous connection.

Eur J Cardiothorac Surg 2022 May;61(6):1299-1305

Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.

Objectives: Prior studies have reported a high mortality and incidence of post-repair pulmonary venous obstruction (PVO) in mixed total anomalous pulmonary venous connection (TAPVC). This study sought to review the surgical outcomes in this entity.

Methods: A review of 61 patients undergoing surgical repair of mixed TAPVC was conducted. Read More

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The Prophylaxis of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome With Defibrotide After Hematopoietic Stem Cell Transplantation in Children: Single Center Experience.

J Pediatr Hematol Oncol 2022 01;44(1):e35-e39

Department of Pediatric Hematology/Oncology and Bone Marrow Transplantation Unit.

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is one of the most severe and life-threatening complications after hematopoietic stem cell transplantation (HSCT). Defibrotide (DF) is approved for adult and pediatric patients with VOD/SOS with renal or pulmonary dysfunction after HSCT in the United States, and for severe VOD/SOS post-HSCT in patients above 1 month of age in the European Union. Several studies have examined whether DF prophylaxis can reduce the incidence of VOD/SOS in high-risk patients. Read More

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January 2022

Pulmonary arterial hypertension associated with pulmonary arteriovenous malformations and pulmonary veno-occlusive disease: A devastating combination.

Respir Med Case Rep 2021 1;34:101564. Epub 2021 Dec 1.

University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Cardiology, the Netherlands.

We describe a case of an adolescent male with the rare combination of pulmonary arterial hypertension (PAH) and pulmonary arteriovenous malformations (PAVM's) without confirmed hereditary hemorrhagic telangiectasia (HHT). The patient showed clinical deterioration on standard vasodilator therapy, leading us to question our initial diagnosis. Post-mortem evaluation confirmed the presence of pulmonary veno-occlusive disease of which no conclusive signs were recognized at diagnostic work-up. Read More

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December 2021

Preemptive Bundle Therapy for Subclinical Pulmonary Hypertension After Liver Transplant.

Exp Clin Transplant 2021 11;19(11):1173-1181

From the Department of General and Digestive Surgery, Kanazawa Medical University, Kahoku, Ishikawa, Japan.

Objectives: After liver transplant, veno-occlusive disease and infectious complications may result from subclinical pulmonary hypertension. In this retrospective study, we investigated whether our preemptive bundle therapy was effective for subclinical pulmonary hypertension and extrasinusoidal platelet aggregation after liver transplant.

Materials And Methods: After January 2014, nutrition therapy with glutamine, synbiotics, phosphodiesterase 3 inhibitors, prostaglandin E1, prostaglandin I2, closedloop artificial pancreas, and sivelestat has been used to reduce bacterialtranslocation, vascular endothelial cell damage, and extrasinusoidal platelet aggregation, which is administered as preemptive bundle therapy for all livertransplantrecipients. Read More

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November 2021

A 24-Year-Old Woman With Dyspnea, Chest Pain, and Dry Cough.

Chest 2021 11;160(5):e503-e506

Department of Pulmonary Medicine, Gaziantep University, Sahinbey Research Hospital, Gaziantep, Turkey.

Case Presentation: A 24-year-old woman, a baby-sitter with no known comorbidities, presented to the outpatient department with complaints of modified Medical Research Council grade IV breathlessness for 3 months, chest pain, and dry cough for 2 weeks. There was no known disease history, including respiratory, flu-like illness, or connective tissue disorder. There was no use of chemotherapeutic, oral contraceptive drugs, exposure to toxic substances, or smoking. Read More

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November 2021

Good response to pulmonary arterial hypertension-targeted therapy in 2 pulmonary veno-occlusive disease patients: A case report.

Medicine (Baltimore) 2021 Oct;100(41):e27334

Department of Respiratory medicine, Zhongda Hospital of Southeast University, Nanjing, China.

Rationale: Pulmonary veno-occlusive disease (PVOD) is a kind of rare and fatal pulmonary arterial hypertension (PAH). Different from other subtypes of PAH, PVOD patients have a very poor prognosis because of the progressive nature of pulmonary vascular involvement and fatal pulmonary edema induced by PAH-targeted drugs. Lung transplantation is the only choice for these patients. Read More

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October 2021

Evolution of Ventricular Assist Device Support Strategy in Children With Univentricular Physiology.

Ann Thorac Surg 2021 Oct 26. Epub 2021 Oct 26.

Division of Pediatric Cardiothoracic Surgery, Washington University School of Medicine in St Louis, St Louis, Missouri. Electronic address:

Background: Since 2012, we have supported 18 children with single ventricle (SV) physiology on ventricular assist devices (VADs) as a bridge to decision, transplantation, or recovery. We provide a detailed report of our cumulative surgical experience and lessons learned from these patients.

Methods: We reviewed all SV-VADs between March 2012 and April 2020. Read More

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October 2021