1,231 results match your criteria Pulmonary Venoocclusive Disease


Pulmonary capillary hemangiomatosis: a case series and review of literature.

Am J Cardiovasc Dis 2021 15;11(2):239-245. Epub 2021 Apr 15.

Radiodiagnosis Department, Medical Research Institute, Alexandria University Alexandria, Egypt.

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH) of unknown etiology resulting from pulmonary capillary proliferation. Clinically, PCH is seen in young adults with equal sex predilection and rarely reported familial predisposition. PCH's main clinical presentations are progressive dyspnea, fatigue, hemoptysis, palpitations, and later irreversible pulmonary hypertension and right-sided heart failure. Read More

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Echocardiography Monitoring of Pulmonary Hypertension after Pediatric Hematopoietic Stem Cell Transplantation: Pediatric PAH and PVOD after HSCT.

Transplant Cell Ther 2021 May 28. Epub 2021 May 28.

Department of Pediatrics, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550 Japan. Electronic address:

Background: Pulmonary hypertension (PH) is associated with a high morbidity in children receiving hematopoietic stem cell transplantation (HSCT). However, due to the lack of sequential echocardiography, the nature of the condition is not fully understood.

Objectives: To study whether routine echocardiography performed after HSCT could detect patients with PH at an earlier stage and elucidate the role of intervention using tadalafil. Read More

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Epoprostenol for the treatment of pulmonary arterial hypertension.

Expert Rev Clin Pharmacol 2021 Jun 7:1-9. Epub 2021 Jun 7.

Pulmonary Hypertension Unit, Cardiology Department, Hospital Universitario 12 De Octubre, Madrid, Spain.

: Pulmonary arterial hypertension (PAH) is a rare, progressive, and severe disease. Since the first demonstration of survival benefit of intravenous epoprostenol in monotherapy in 1996, prostanoids remain the cornerstone for PAH patients at high risk. This review is essential to understand the current situation of this drug among all the therapeutic possibilities concerning this entity. Read More

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GCN2 Regulates ATF3-p38 MAPK Signaling Transduction in Pulmonary Veno-Occlusive Disease.

J Cardiovasc Pharmacol Ther 2021 May 14:10742484211015535. Epub 2021 May 14.

Department of Physiology, 26441Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.

Pulmonary veno-occlusive disease (PVOD) is a fatal disease of pulmonary vascular lesions leading to right heart failure. Heritable PVOD (hPVOD) is related to biallelic mutation of (encoding GCN2), but its molecular mechanism remains unclear. In this study, we aimed to investigate the pathogenesis of PVOD and to find potential drug targets for PVOD. Read More

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Rapidly progressive fatal hypoxia in a young woman.

Proc (Bayl Univ Med Cent) 2021 Jan 22;34(3):407-408. Epub 2021 Jan 22.

Center for Advanced Lung Diseases, Baylor University Medical Center, Dallas, Texas.

We present a rare cause of pulmonary arterial hypertension in a 29-year-old woman with rapidly progressive and fatal hypoxia. Subsequent workup revealed classic radiological findings and pathologic confirmation of pulmonary veno-occlusive disease. Read More

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January 2021

Successful Treatment of Sinusoidal Obstructive Syndrome with Deceased-Donor Liver Transplant Following Hematopoietic Stem Cell Transplant.

Exp Clin Transplant 2021 May 6. Epub 2021 May 6.

From the Department of Surgery, Division of HBP Surgery and Liver Transplantation, Korea University Medical Center, Korea University Medical College, Seoul, Korea.

Sinusoidal obstructive syndrome, also known as venoocclusive disease, is a potentially life-threatening complication of hematopoietic stem cell transplant. The conditioning regimens given before hematopoietic stem cell transplant result in the production of toxic metabolites that trigger the activation, damage, and inflammation of the endothelial cells that line the sinusoids. Although liver transplant has been performed for treatment of sinusoidal obstructive syndrome, reports by various liver transplant centers worldwide have been limited and disappointing. Read More

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No Good Deed Goes Unpunished: Mitomycin-Induced Pulmonary Venoocclusive Disease and Cancer.

Chest 2021 Mar;159(3):910-911

Division of Pulmonary, Allergy, and Critical Care Medicine, Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford, Stanford University School of Medicine, Stanford, CA. Electronic address:

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[Screening for pulmonary veno-occlusive disease: A specific approach].

Rev Mal Respir 2021 Feb 8;38(2):215-216. Epub 2021 Feb 8.

Assistance Publique-Hôpitaux de Paris (AP-HP), Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France; INSERM UMR_S 999 Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, France.

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February 2021

Mixed type total anomalous pulmonary venous connection: Early results and midterm outcomes of surgical correction.

J Card Surg 2021 Apr 10;36(4):1370-1375. Epub 2021 Feb 10.

Department of Anaesthesia and Intensive Care, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Background And Aim Of The Study: The morphological heterogeneity of anomalous pulmonary venous drainage in mixed type total anomalous pulmonary venous connection (TAPVC) has important implications in preoperative diagnosis and surgical repair resulting in high mortality in these patients.

Methods: A retrospective review of 14 patients with mixed type TAPVC undergoing biventricular repair between January 2012 and December 2019 was conducted. A descriptive analysis was done, highlighting the anatomic variation, diagnostic and surgical approach, and surgical outcomes in these patients. Read More

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COVID-19-induced endotheliitis: emerging evidence and possible therapeutic strategies.

Br J Haematol 2021 04 4;193(1):43-51. Epub 2021 Feb 4.

Department of Medical Oncology, Division of Hematologic Malignancies, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.

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Pulmonary venous thrombosis in a patient with COVID-19 infection.

J Thromb Thrombolysis 2021 May 30;51(4):985-988. Epub 2021 Jan 30.

Vascular Division, Department of Cardiology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Objectives: Infection with the SARS-COV2 virus (COVID-19) may be complicated by thrombotic diathesis. This complication often involves the pulmonary microcirculation. While macrovascular thrombotic complications of the lung may include pulmonary artery embolism, pulmonary artery thrombus in situ has also been hypothesized. Read More

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Dyspnoea and diffuse pulmonary nodules in a patient with pulmonary veno-occlusive disease: a case report and literature review.

J Int Med Res 2021 Jan;49(1):300060520986689

Department of Respiratory Diseases, Tongji Hospital, Key Lab of Pulmonary Diseases of Health Ministry, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Pulmonary veno-occlusive disease (PVOD) is a rare type of pulmonary hypertension characterized by capillary damage or arterial pulmonary hypertension. Early lung transplantation is the only effective treatment for PVOD because of the lack of specificity in its clinical manifestations and its rapid progression and poor prognosis. A 28-year-old woman presented with exertional dyspnoea. Read More

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January 2021

Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties.

Diagnostics (Basel) 2021 Jan 19;11(1). Epub 2021 Jan 19.

Centro de Referencia Nacional de Hipertensión Pulmonar Compleja, Spain and ERN-Lung-Pulmonary Hypertension) Referal Center, 28041 Madrid, Spain.

Pulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantation. However, in the absence of histological or genetic confirmation, the diagnosis is complex, requiring a clinical suspicion. Read More

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January 2021

Pulmonary Veno Occlusive Disease Presenting as Failure to Thrive.

Indian J Pediatr 2021 05 14;88(5):494. Epub 2021 Jan 14.

Department of Pediatric Critical Care, Oishei Children's Hospital, University of Buffalo, 9th Floor, 818 Ellicott Street, Buffalo, NY, 14203, USA.

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Valved reverse Potts shunt in a case of pulmonary hypertension due to pulmonary veno-occlusive disease.

Indian J Thorac Cardiovasc Surg 2021 Jan 7;37(1):89-92. Epub 2020 Aug 7.

Division of Pediatric Cardiothoracic Surgery, Fortis Hospital, Mulund, Mumbai, 400078 India.

Idiopathic pulmonary hypertension has a predictably morbid natural history with an absence of a uniformly successful treatment strategy. We describe our palliative surgical strategy in a symptomatic teenager. A 16-year-old girl, with a recent diagnosis of severe suprasystemic pulmonary hypertension, with severe right ventricular dysfunction, presented with syncope and World Health Organization functional class 4 symptoms. Read More

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January 2021

Pulmonary hypertension secondary to pulmonary veno occlusive disease: Catastrophe in the catheterization laboratory.

Ann Pediatr Cardiol 2020 Oct-Dec;13(4):377-379. Epub 2020 Sep 23.

Department of Pediatric Cardiology, Childnrens Heart Center, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India. E-mail:

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September 2020

Pulmonary Edema: A Pictorial Review of Imaging Manifestations and Current Understanding of Mechanisms of Disease.

Authors:
Maria Barile

Eur J Radiol Open 2020 30;7:100274. Epub 2020 Oct 30.

Department of Radiology at University of Massachusetts Memorial Medical Center, University of Massachusetts Medical School, Worcester, MA, United States.

Pulmonary edema is a common clinical entity caused by the extravascular movement of fluid into the pulmonary interstitium and alveoli. The four physiologic categories of edema include hydrostatic pressure edema, permeability edema with and without diffuse alveolar damage (DAD), and mixed edema where there is both an increase in hydrostatic pressure and membrane permeability. As radiographic manifestations and etiologies are varied, an appreciation for both the common and uncommon manifestations and causes of pulmonary edema is essential for accurate diagnosis. Read More

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October 2020

Trichloroethylene increases pulmonary endothelial permeability: implication for pulmonary veno-occlusive disease.

Pulm Circ 2020 Oct-Dec;10(4):2045894020907884. Epub 2020 Oct 16.

School of Medicine, Université Paris-Saclay, Le Kremlin-Bicêtre, France.

Trichloroethylene exposure is a major risk factor for pulmonary veno-occlusive disease. We demonstrated that trichloroethylene alters the endothelial barrier integrity, at least in part, through vascular endothelial (VE)-Cadherin internalisation, and suggested that this mechanism may play a role in the development of pulmonary veno-occlusive disease. Read More

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October 2020

Mitomycin C induces pulmonary vascular endothelial-to-mesenchymal transition and pulmonary veno-occlusive disease via Smad3-dependent pathway in rats.

Br J Pharmacol 2021 01 14;178(1):217-235. Epub 2020 Dec 14.

State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangdong Key Laboratory of Vascular Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, China.

Background And Purpose: Pulmonary veno-occlusive disease (PVOD) is a rare disease characterized by the obstruction of small pulmonary veins leading to pulmonary hypertension. However, the mechanisms underlying pulmonary vessel occlusion remain largely unclear.

Experimental Approach: A mitomycin C (MMC)-induced PVOD rat model was used as in vivo animal model, and primarily cultured rat pulmonary microvascular endothelial cells (PMVECs) were used as in vitro cell model. Read More

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January 2021

Two patients with mixed connective tissue disease complicated by pulmonary arterial hypertension showing contrasting responses to pulmonary vasodilators.

Mod Rheumatol Case Rep 2020 07 4;4(2):253-261. Epub 2020 May 4.

The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.

Mixed connective tissue disease (MCTD) involves various clinical manifestations, and pulmonary hypertension (PH) is an important organ dysfunction defining the prognosis of MCTD. The pathology of PH is heterogeneous. Here, we present 2 cases of MCTD complicated by PH that had contrasting clinical courses. Read More

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A case report, a case who developed limited cutaneous scleroderma and pulmonary hypertension 8 years after diagnosis of anti-centromere antibody-positive Sjögren syndrome.

Mod Rheumatol Case Rep 2020 07 30;4(2):248-252. Epub 2020 Apr 30.

Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, Kurume, Japan.

A 52-year-old woman was diagnosed as having anti-centromere antibody (ACA)-positive primary Sjögren syndrome (pSS). Eight years later, she visited our hospital because she had developed dyspnoea. She was diagnosed as having pulmonary arterial hypertension (PAH) with pulmonary veno-occlusive disease on the basis of the results of right heart catheterisation, a severe decrease in diffusing capacity of the lung for carbon monoxide (D, 17%) and desaturation (69%) after a 6-minute walk test. Read More

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[Pulmonary involvement in cancers].

Pneumologe (Berl) 2020 Oct 13:1-9. Epub 2020 Oct 13.

Abteilung für Pneumologie, Asklepios Fachkliniken München-Gauting, 82131 Gauting, Deutschland.

The lungs are often involved in tumors and are affected in a wide variety of ways. Lung cancer comprises one of the most common cancer entities and has been characterized by a vast expansion of treatment approaches in recent years. Moreover, the lungs are a common metastatic site of multiple other cancer entities. Read More

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October 2020

[Familial pulmonary veno-occlusive disease with a composite biallelic heterozygous EIF2AK4 mutation].

Rev Mal Respir 2020 Dec 15;37(10):823-828. Epub 2020 Oct 15.

Département de pneumologie, centre de compétences de l'hypertension pulmonaire, CHU de Nancy, bâtiment Philippe-Canton, rue de Morvan, 54511 Vandœuvre-lès-Nancy, France.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Heritable and sporadic forms have been distinguished. Hypoxemia, profound reduction in the diffusion of carbon monoxide and haemodynamic confirmation of pre-capillary pulmonary hypertension are the major diagnostic criteria. Read More

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December 2020

Genetic Evaluation in a Cohort of 126 Dutch Pulmonary Arterial Hypertension Patients.

Genes (Basel) 2020 10 13;11(10). Epub 2020 Oct 13.

Department of Clinical Genetics, Amsterdam UMC (location VUmc), 1081HV Amsterdam, The Netherlands.

Pulmonary arterial hypertension (PAH) is a severe, life-threatening disease, and in some cases is caused by genetic defects. This study sought to assess the diagnostic yield of genetic testing in a Dutch cohort of 126 PAH patients. Historically, genetic testing in the Netherlands consisted of the analysis of BMPR2 and SMAD9. Read More

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October 2020

Maternal Mortality: 10 Year Experience of a Tertiary Center in Turkey.

Case Rep Med 2020 30;2020:3595024. Epub 2020 Sep 30.

Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University Medical Faculty, Ankara, Turkey.

We retrospectively evaluated five maternal mortality cases that occurred in our institution within the last 10 years. Rate of maternal mortality was 24.5 per 100000 live births. Read More

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September 2020

Pulmonary capillary hemangiomatosis-predominant vasculopathy in a patient with rheumatoid arthritis-associated interstitial lung disease: An autopsy report.

Respir Med Case Rep 2020 8;31:101215. Epub 2020 Sep 8.

First Department of Medicine, Hokkaido University Hospital, Kita-14, Nishi-5, Kita-ku, Sapporo, 060-8648, Japan.

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension (PH) associated with poor prognosis. Clinically, it is characterized by severe hypoxemia, centrilobular ground-glass opacities on computed tomography, and pulmonary congestion triggered by pulmonary vasodilating therapy. In some cases, PCH has been reported to develop with other disorders including connective tissue disease; however, to date, no reports have described PCH in a patient with rheumatoid arthritis. Read More

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September 2020

Characteristics and Long-term Outcomes of Pulmonary Venoocclusive Disease Induced by Mitomycin C.

Chest 2021 Mar 24;159(3):1197-1207. Epub 2020 Sep 24.

INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hôpital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; University of Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France. Electronic address:

Background: Pulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) predominantly characterized by pulmonary vein and capillary involvement. An association between chemotherapy, in particular mitomycin C (MMC), and PVOD has been reported.

Research Question: What are the characteristics of MMC-induced PVOD, and what is the prognosis for patients with MMC-induced PVOD?

Study Design And Methods: We report the clinical, functional, radiologic, and hemodynamic characteristics at diagnosis and outcomes of patients with PVOD from the French PH Registry after exposure to MMC. Read More

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Analysis of Time to Complete Response after Defibrotide Initiation in Patients with Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Hematopoietic Cell Transplantation.

Transplant Cell Ther 2021 Jan 17;27(1):88.e1-88.e6. Epub 2020 Sep 17.

Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication that occurs after hematopoietic cell transplantation (HCT). The mortality associated with untreated VOD/SOS with multiorgan dysfunction (MOD) has been reported to be >80%. The recommended dose of defibrotide is 6. Read More

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January 2021