1,113 results match your criteria Pulmonary Venoocclusive Disease


Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease.

Pediatr Radiol 2019 Jan 16. Epub 2019 Jan 16.

M3C-Necker, Congenital and Pediatric Cardiology, Hôpital Universitaire Necker-Enfants malades, Paris, France.

Background: In children, idiopathic and heritable pulmonary arterial hypertension present echocardiographic and heart catheterization findings similar to findings in pulmonary veno-occlusive disease.

Objective: To provide a systematic analysis of CT angiography anomalies in children with idiopathic or heritable pulmonary arterial hypertension, or pulmonary veno-occlusive disease. We also sought to identify correlations between CT findings and patients' baseline characteristics. Read More

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http://dx.doi.org/10.1007/s00247-018-04331-yDOI Listing
January 2019

Widening the landscape of heritable pulmonary hypertension mutations in pediatric and adult cases.

Eur Respir J 2018 Dec 21. Epub 2018 Dec 21.

Département de Génétique, hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris.

Background: Heritable forms of pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH) diverge by lung histopathological lesions, clinical and para-clinical presentation, their responsible genes and mode of transmission. Since identification of the Bone Morphogenetic Protein Receptor Type 2 (BMPR-2) gene in families affected by PAH, mutations in several other genes have been discovered for both forms. The mutation landscape in these new genes is not yet well known. Read More

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http://erj.ersjournals.com/lookup/doi/10.1183/13993003.01371
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http://dx.doi.org/10.1183/13993003.01371-2018DOI Listing
December 2018
8 Reads

Pulmonary Veno-occlusive Disease: A Surgical Lung Biopsy-proven and Autopsied Case Radiologically Mimicking Hypersensitivity Pneumonitis at the Time of a Transbronchial Lung Biopsy.

Intern Med 2018 Dec 18. Epub 2018 Dec 18.

Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan.

Pulmonary veno-occlusive disease (PVOD) is a rare disease in the subgroup of conditions known as pulmonary arterial hypertension. Although a histological examination is needed for a definitive diagnosis, a non-invasive diagnosis is required for patients with pulmonary hypertension because a lung biopsy is deemed risky. We herein report a 32-year-old woman diagnosed with PVOD via a surgical lung biopsy and autopsy whose disease showed radiological findings mimicking those of hypersensitivity pneumonitis (pneumonia) at the time of the transbronchial lung biopsy, without obvious pulmonary hypertension on admission. Read More

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http://dx.doi.org/10.2169/internalmedicine.0681-17DOI Listing
December 2018

Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives.

Eur Respir J 2018 Dec 13. Epub 2018 Dec 13.

Cardiovascular Institute, Dept of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA.

Clinical and translational research has played a major role in advancing our understanding of pulmonary hypertension (PH), including pulmonary arterial hypertension and other forms of PH with severe vascular remodelling ( chronic thromboembolic PH and pulmonary veno-occlusive disease). However, PH remains an incurable condition with a high mortality rate, underscoring the need for a better transfer of novel scientific knowledge into healthcare interventions. Herein, we review recent findings in pathology (with the questioning of the strict morphological categorisation of various forms of PH into pre- or post-capillary involvement of pulmonary vessels) and cellular mechanisms contributing to the onset and progression of pulmonary vascular remodelling associated with various forms of PH. Read More

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http://erj.ersjournals.com/lookup/doi/10.1183/13993003.01887
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http://dx.doi.org/10.1183/13993003.01887-2018DOI Listing
December 2018
15 Reads

Haemodynamic definitions and updated clinical classification of pulmonary hypertension.

Eur Respir J 2018 Dec 13. Epub 2018 Dec 13.

Pulmonary Circulation Unit, Pulmonary Division, Heart Institute (InCor), Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.

Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3. Read More

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http://erj.ersjournals.com/lookup/doi/10.1183/13993003.01913
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http://dx.doi.org/10.1183/13993003.01913-2018DOI Listing
December 2018
6 Reads

Pulmonary veno-occlusive disease as a cause of severe pulmonary hypertension in a dog.

Acta Vet Scand 2018 Dec 5;60(1):78. Epub 2018 Dec 5.

Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Yalelaan 108, 3508 TD, Utrecht, The Netherlands.

Background: Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH) in humans and can be classified in idiopathic, heritable, drug and radiation-induced, and associated with connective tissue disease or human immunodeficiency virus infection. Recently, biallelic mutations of the EIF2AK4 gene have been discovered as a cause for an autosomal recessive form of PVOD in humans. In dogs, PAH is poorly characterized and is generally considered to be idiopathic or secondary to (for example) congenital left-to right cardiovascular shunts or heartworm disease. Read More

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https://actavetscand.biomedcentral.com/articles/10.1186/s130
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http://dx.doi.org/10.1186/s13028-018-0433-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6282261PMC
December 2018
6 Reads

Clinical features of canine pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis.

J Vet Intern Med 2019 Jan 29;33(1):114-123. Epub 2018 Nov 29.

Department of Pathobiology and Diagnostic Investigation, College of Veterinary Medicine, Michigan State University, East Lansing, Michigan.

Background: Histologic features of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) have been described in dogs but without a thorough clinical description.

Objectives: To report the clinical features, diagnostics, treatment, and outcome of dogs with histologic evidence of PVOD and PCH.

Animals: Fifteen pet dogs meeting histopathologic criteria of PVOD (occlusive remodeling of small-sized to medium-sized pulmonary veins) or PCH (alveolar capillary proliferation and congestion), or both. Read More

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http://doi.wiley.com/10.1111/jvim.15351
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http://dx.doi.org/10.1111/jvim.15351DOI Listing
January 2019
1 Read

Use of vasodilators for the treatment of pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: A systematic review.

Respir Investig 2018 Nov 22. Epub 2018 Nov 22.

Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan. Electronic address:

Background: There are several medications available to treat pulmonary arterial hypertension (PAH): PAH-targeted drugs. However, in patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis (PVOD/PCH), rare diseases that cause pulmonary hypertension, the effectiveness and safety of vasodilators, including PAH-targeted drugs, are unclear.

Methods: We searched English-language publications listed in three electronic databases (PubMed, Cochrane Library, and the Japan Medical Abstracts Society). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22125345183017
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http://dx.doi.org/10.1016/j.resinv.2018.10.004DOI Listing
November 2018
2 Reads

Lung transplantation as a viable option of treatment for pulmonary veno-occlusive disease.

Adv Respir Med 2018 ;86(5):249-254

Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, SDMZ in Zabrze, Medical University of Silesia in Katowice, Silesian Centre for Heart Disease in Zabrze, Poland.

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension caused by alteration of pulmonary veins. Many clinical and hemodynamic similarities to idiopathic pulmonary arterial hypertension (IPAH) may cause diagnostic and therapeutic difficulties. This case report is about a patient with PVOD, whose first symptoms of the disease occurred after infectious mononucleosis. Read More

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https://journals.viamedica.pl/advances_in_respiratory_medici
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http://dx.doi.org/10.5603/ARM.2018.0040DOI Listing
January 2018
10 Reads

Respiratory failure in the hematopoietic stem cell transplant recipient.

World J Crit Care Med 2018 Oct 16;7(5):62-72. Epub 2018 Oct 16.

Multidisciplinary Epidemiology and Translational Research in Intensive Care Group, Mayo Clinic, Rochester, MN 55905, United States.

The number of patients receiving hematopoietic stem cell transplantation (HSCT) is rapidly rising worldwide. Despite substantial improvements in peri-transplant care, pulmonary complications resulting in respiratory failure remain a major contributor to morbidity and mortality in the post-transplant period, and represent a major barrier to the overall success of HSCT. Infectious complications include pneumonia due to bacteria, viruses, and fungi, and most commonly occur during neutropenia in the early post-transplant period. Read More

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http://www.wjgnet.com/2220-3141/full/v7/i5/62.htm
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http://dx.doi.org/10.5492/wjccm.v7.i5.62DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6201323PMC
October 2018
20 Reads

Defibrotide for the treatment of hepatic veno-occlusive disease/sinusoidal obstruction syndrome with multiorgan failure.

Int J Hematol Oncol 2017 Nov 11;6(3):75-93. Epub 2017 Aug 11.

Department of Pediatric Hematology, Oncology & Stem Cell Transplantation, University of Regensburg, Regensburg, Germany.

Hepatic veno-occlusive disease, also called sinusoidal obstruction syndrome (VOD/SOS), is a potentially life-threatening and unpredictable complication of hematopoietic stem cell transplantation (HSCT). Characterized by a prothrombotic-hypofibrinolytic state, VOD/SOS typically presents with hyperbilirubinemia, ascites, weight gain and painful hepatomegaly; VOD/SOS with multiorgan failure may be associated with >80% mortality. Treatment has been mainly supportive. Read More

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https://www.futuremedicine.com/doi/10.2217/ijh-2017-0015
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http://dx.doi.org/10.2217/ijh-2017-0015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171967PMC
November 2017
10 Reads

Good response to PAH-targeted drugs in a PVOD patient carrying Biallelic EIF2AK4 mutation.

Respir Res 2018 Oct 1;19(1):192. Epub 2018 Oct 1.

Department of Respiratory medicine, Sir Run Run Shaw Hospital, Medical School of Zhejiang University, Hangzhou, China.

Pulmonary veno-occlusive disease (PVOD) is a rare and fatal cause of pulmonary arterial hypertension (PAH). Different from other types of PAH, PVOD patients have a dismal 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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https://respiratory-research.biomedcentral.com/articles/10.1
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http://dx.doi.org/10.1186/s12931-018-0900-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6167821PMC
October 2018
2 Reads

Pharmacovigilance in a rare disease: example of the VIGIAPATH program in pulmonary arterial hypertension.

Int J Clin Pharm 2018 Aug 13;40(4):790-794. Epub 2018 Aug 13.

INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.

Spontaneous reporting is the primary method used in pharmacovigilance (PV) to detect drug safety signal. Specific criteria used in pharmacovigilance to prove accountability of a drug are rarely present in rare disease. The low number of alerts also makes it challenging. Read More

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http://dx.doi.org/10.1007/s11096-018-0712-yDOI Listing
August 2018
62 Reads

Co-existence of vocal cord dysfunction with pulmonary conditions other than asthma: A case series.

Respir Med Case Rep 2018 3;25:104-108. Epub 2018 Aug 3.

Division of Pulmonary, Allergy, and Critical Care Medicine; Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.

Background: Vocal cord dysfunction (VCD) is defined as inappropriate movement of the vocal cords resulting in functional airway obstruction and symptoms including cough, wheezing, and dyspnea. VCD is often misdiagnosed with asthma but can also co-exist with asthma. The association of VCD with other serious pulmonary conditions has not been described to date. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.08.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6083429PMC
August 2018
5 Reads

Pulmonary tumor thrombotic microangiopathy and pulmonary veno-occlusive disease in a woman with cervical cancer treated with cediranib and durvalumab.

BMC Pulm Med 2018 Jul 11;18(1):112. Epub 2018 Jul 11.

Critical Care Medicine Department, National Institutes of Health Clinical Center, Bethesda, MD, USA.

Background: Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare cause of pulmonary hypertension that is associated with malignancies and is marked by the presence of non-occlusive tumor emboli and fibrocellular intimal proliferation of small pulmonary arteries leading to increased pulmonary vascular resistance and right heart failure. The diagnosis of PTTM is challenging to make pre-mortem and guidelines on treatment are lacking.

Case Presentation: A 45-year-old woman with advanced squamous cell carcinoma of the cervix developed symptoms of dyspnea and evidence of right heart failure during a phase I clinical trial with cediranib and durvalumab. Read More

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http://dx.doi.org/10.1186/s12890-018-0681-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6042377PMC
July 2018
11 Reads

Pulmonary veno-occlusive disease: pathogenesis, risk factors, clinical features and diagnostic algorithm - state of the art.

Adv Respir Med 2018 ;86(3)

1st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland.

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) are rare disorders, with the estimated prevalence of less than 1 case per million inhabitants. The vascular pathology in PVOD/PCH involves pre-septal and septal veins, alveolar capillaries and small pulmonary arteries. According to the ERS/ESC classification of pulmonary hypertension (PH) from 2015, PVOD/PCH have been included in the subgroup 1' of pulmonary arterial hypertension (PAH). Read More

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http://dx.doi.org/10.5603/ARM.2018.0021DOI Listing
November 2018
2 Reads

The use of defibrotide in blood and marrow transplantation.

Blood Adv 2018 Jun;2(12):1495-1509

Galena Biopharma, San Ramon, CA.

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication of conditioning during hematopoietic stem cell transplantation (HSCT) or chemotherapy without HSCT, with a historically reported mean incidence of 13.7% post-HSCT. Typical symptoms of VOD/SOS may include hyperbilirubinemia, painful hepatomegaly, weight gain, and ascites. Read More

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http://dx.doi.org/10.1182/bloodadvances.2017008375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6020812PMC
June 2018
11 Reads

[Pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis: A case report and literature review].

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2018 May;43(5):571-576

Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha 410011, China.

Pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary vascular disease that causes pulmonary arterial hypertension. The diagnosis of PVOD/PCH can be established by the combination of clinical features, physical examination, radiological findings, lung function, bronchoscopy and other resources. There is no established medical therapy for PVOD/PCH, and the only curative therapy for PVOD/PCH is lung transplantation. Read More

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http://www.csumed.org/xbwk/CN/10.11817/j.issn.1672-7347.2018
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http://dx.doi.org/10.11817/j.issn.1672-7347.2018.05.017DOI Listing
May 2018
7 Reads

Lung Transplantation in Pulmonary Hypertension: A Multidisciplinary Unit's Management Experience.

Transplant Proc 2018 Jun 9;50(5):1496-1503. Epub 2018 Mar 9.

Lung Transplant Unit, Division of Thoracic Surgery, Hospital Universitario 12 de Octubre, Madrid, Spain; CIBER Respiratorio, Instituto de Investigación Carlos III, Madrid, Spain.

Background: Prognosis assessment of pulmonary hypertension (PH) is multifactorial and placement of patients on the lung transplantation (LT) waiting list requires the weighing of a complex set of criteria. The aim of this retrospective cohort study was to analyze a series of patients treated in our unit at the moment of their inclusion on the LT waiting list and long-term survival after LT.

Material And Methods: Baseline characteristics, LT outcomes, and survival were evaluated in all patients diagnosed with pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD) who were included on the LT waiting list in 2011-2016. Read More

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http://dx.doi.org/10.1016/j.transproceed.2018.02.073DOI Listing
June 2018
2 Reads

Defibrotide for the treatment of hepatic veno-occlusive disease/sinusoidal obstruction syndrome following nontransplant-associated chemotherapy: Final results from a post hoc analysis of data from an expanded-access program.

Pediatr Blood Cancer 2018 Oct 6;65(10):e27269. Epub 2018 Jun 6.

Pediatric Oncology, The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Background: Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially fatal complication of conditioning for hematopoietic stem cell transplantation (HSCT) but can occur after nontransplant-associated chemotherapy. Following HSCT, VOD/SOS with multi-organ dysfunction (MOD) may be associated with >80% mortality. Defibrotide is approved to treat severe hepatic VOD/SOS post-HSCT in patients aged >1 month in the European Union and hepatic VOD/SOS with renal or pulmonary dysfunction post-HSCT in the United States. Read More

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http://dx.doi.org/10.1002/pbc.27269DOI Listing
October 2018
20 Reads
2.562 Impact Factor

Key technical steps in dual-pathway repair of congenital pulmonary vein stenosis.

Asian Cardiovasc Thorac Ann 2018 Jul 3;26(6):498-501. Epub 2018 Jun 3.

Department of Pediatric Cardiac Sciences, Sir HN Reliance Foundation Hospital, Mumbai, India.

We present the case of a 2-year-old girl with congenital stenosis of the left inferior pulmonary vein associated with a large perimembranous ventricular septal defect. The child underwent repair of the left inferior pulmonary vein with autologous left atrial appendage as a pedicled tube, followed by closure of the ventricular septal defect. Important technical steps to minimize the restenosis rate are highlighted. Read More

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http://dx.doi.org/10.1177/0218492318780804DOI Listing
July 2018
1 Read

Pulmonary capillary hemangiomatosis diagnosed by pathology of explanted lungs: a unique etiology serves as a key of clinical diagnosis.

Gen Thorac Cardiovasc Surg 2018 May 26. Epub 2018 May 26.

Department of General Thoracic Surgery, Chiba University Graduate School of Medicine, Chiba, Japan.

A 27-year-old female patient had presented progressing exertional dyspnea due to pulmonary hypertension. Chest CT revealed diffusely spread patchy ground-glass opacities sparing subpleural parenchymal areas suggesting the diagnosis of pulmonary veno-occlusive disease (PVOD). Despite the diagnosis of PVOD, she was somehow managed by a repetitive escalation of the epoprostenol dose and oxygen supply during the 12-month waiting period until successful bilateral lung transplantation was performed. Read More

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http://dx.doi.org/10.1007/s11748-018-0950-xDOI Listing
May 2018
10 Reads

Neonatal Outcomes in Total Anomalous Pulmonary Venous Return: The Role of Prenatal Diagnosis and Pulmonary Venous Obstruction.

Pediatr Cardiol 2018 Oct 23;39(7):1346-1354. Epub 2018 May 23.

Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.

The objective of this study is to evaluate neonatal outcomes of total anomalous pulmonary venous return (TAPVR) and identify fetal echocardiography findings associated with preoperative pulmonary venous obstruction (PPVO). This retrospective study evaluated TAPVR cases from 2005 to 2014 for preoperative and postoperative outcomes based on prenatal diagnosis, PPVO, and heterotaxy syndrome. Fetal pulmonary and vertical vein Dopplers were analyzed as predictors of PPVO. Read More

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http://link.springer.com/10.1007/s00246-018-1901-0
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http://dx.doi.org/10.1007/s00246-018-1901-0DOI Listing
October 2018
8 Reads

Final results from a defibrotide treatment-IND study for patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome.

Br J Haematol 2018 Jun 16;181(6):816-827. Epub 2018 May 16.

Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication of haematopoietic stem cell transplant (HSCT) conditioning and chemotherapy. Defibrotide is approved for treatment of hepatic VOD/SOS with pulmonary or renal dysfunction [i.e. Read More

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http://dx.doi.org/10.1111/bjh.15267DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6032999PMC
June 2018
3 Reads
4.710 Impact Factor

Genetic analyses in a cohort of 191 pulmonary arterial hypertension patients.

Respir Res 2018 05 9;19(1):87. Epub 2018 May 9.

State Key Laboratory of Cardiovascular Disease, Beijing Key Laboratory for Molecular Diagnostics of Cardiovascular Diseases, Diagnostic Laboratory Service, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Background: Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder associated with high pulmonary artery pressure. Genetic testing enables early diagnosis and offers an opportunity for family screening. To identify genetic mutations and help make a precise diagnosis, we performed genetic testing in 191 probands with PAH and tried to analyze the genotype-phenotype correlation. Read More

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http://dx.doi.org/10.1186/s12931-018-0789-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5944100PMC

Conventional repair of total anomalous venous drainage without primary sutureless technique: surgical tips to prevent pulmonary vein obstruction.

Gen Thorac Cardiovasc Surg 2018 Jul 26;66(7):405-410. Epub 2018 Apr 26.

Department of Cardiovascular Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ward, Sagamihara, Japan.

Objectives: Although primary sutureless technique for total anomalous pulmonary venous drainage has been introduced to reduce postoperative pulmonary vein obstruction (PVO), controversy still exists about superiority of the procedure between the conventional repair and primary sutureless technique at the initial repair. In our unit, the conventional repair has been consistently used based on four important surgical policies: (1) mark incision lines between 2 chambers to gain anatomically natural alignment, (2) place precise stitches by "intima-to-intima" using monofilament suture, (3) adequate orifice size should be guaranteed in greater than expected mitral valve size, (4) do not hesitate to undertake a redo additional anastomosis by a different approach when an echocardiography shows the velocity more than 1.5 m/s. Read More

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http://dx.doi.org/10.1007/s11748-018-0921-2DOI Listing
July 2018
4 Reads

Pressure monitoring predicts pulmonary vein occlusion in cryoballoon ablation.

J Interv Card Electrophysiol 2018 Oct 10;53(1):115-121. Epub 2018 Apr 10.

Kansai Rosai Hospital Cardiovascular Center, 3-1-69, Inabaso, Amagasakishi, Hyogo, 660-8511, Japan.

Purpose: Pulmonary venography is routinely used to confirm pulmonary vein (PV) occlusion during cryoballoon ablation. However, this technique is significantly limited by the risks associated with contrast media, such as renal injury and contrast allergy. We hypothesized that PV occlusion can be predicted by elevation of the balloon catheter tip pressure, avoiding the need for contrast media. Read More

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http://dx.doi.org/10.1007/s10840-018-0366-7DOI Listing
October 2018
1 Read

Lung transplantation for scleroderma lung disease: An international, multicenter, observational cohort study.

J Heart Lung Transplant 2018 Jul 15;37(7):903-911. Epub 2018 Mar 15.

Service de Chirurgie Thoracique, Vasculaire et Transplantation Cardio-pulmonaire, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France; Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France; UMR_S 999, Université Paris-Sud, INSERM, Hôpital Marie Lannelongue, Le Plessis Robinson, France. Electronic address:

Background: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD). Furthermore, it remains unknown whether scleroderma-associated pulmonary arterial hypertension (PAH) influences post-transplant outcomes. Read More

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http://dx.doi.org/10.1016/j.healun.2018.03.003DOI Listing
July 2018
15 Reads

Clinical prediction score for identifying patients with pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis.

J Cardiol 2018 Sep 13;72(3):255-260. Epub 2018 Mar 13.

Department of Clinical Science, National Hospital Organization Okayama Medical Center, Okayama, Japan; Department of Cardiology, National Hospital Organization Okayama Medical Center, Okayama, Japan. Electronic address:

Background: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension. Although diagnosis is based on pathological findings, an early diagnosis is crucial because of poor prognosis compared to other types of pulmonary hypertension. Furthermore, vasodilators may cause fatal pulmonary edema in patients with PVOD/PCH. Read More

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http://dx.doi.org/10.1016/j.jjcc.2018.02.009DOI Listing
September 2018
12 Reads

Drug-associated pulmonary arterial hypertension.

Clin Toxicol (Phila) 2018 Sep 6;56(9):801-809. Epub 2018 Mar 6.

a Cardiovascular Department , John Hunter Hospital , Newcastle , Australia.

Introduction: While pulmonary arterial hypertension remains an uncommon diagnosis, various therapeutic agents are recognized as important associations. These agents are typically categorized into "definite", "likely", "possible", or "unlikely" to cause pulmonary arterial hypertension, based on the strength of evidence.

Objective: This review will focus on those therapeutic agents where there is sufficient literature to adequately comment on the role of the agent in the pathogenesis of pulmonary arterial hypertension. Read More

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https://www.tandfonline.com/doi/full/10.1080/15563650.2018.1
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http://dx.doi.org/10.1080/15563650.2018.1447119DOI Listing
September 2018
8 Reads

[Pulmonary veno-occlusive disease].

Rev Mal Respir 2018 Feb 1;35(2):160-170. Epub 2018 Mar 1.

Faculté de médecine, university Paris-Sud, 94270 Le Kremlin-Bicêtre, France; Département hospitalo-universitaire (DHU) thorax innovation (TORINO), service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, hôpital de Bicêtre, université Paris-Sud, AP-HP, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France; UMR_S999, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), hôpital Marie-Lannelongue, university Paris-Sud, 92350 Le Plessis-Robinson, France. Electronic address:

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Read More

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http://dx.doi.org/10.1016/j.rmr.2017.11.005DOI Listing
February 2018

Extracorporeal Membrane Oxygenation Support During Pregnancy in Pulmonary Veno-occlusive Disease.

Rev Esp Cardiol (Engl Ed) 2018 Feb 7. Epub 2018 Feb 7.

Unidad de Hipertensión Pulmonar, Servicio de Cardiología, Hospital Universitario 12 de Octubre, Madrid, Spain; Facultad de Medicina, Universidad Complutense, Madrid, Spain.

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http://dx.doi.org/10.1016/j.rec.2018.01.001DOI Listing
February 2018
29 Reads

Respiratory effects of trichloroethylene.

Respir Med 2018 01 1;134:47-53. Epub 2017 Dec 1.

Inserm, U1168, VIMA: Aging and Chronic Diseases, Epidemiological and Public Health Approaches, F-94807, Villejuif, France; Univ Versailles St-Quentin-en-Yvelines, UMR-S 1168, F-78180, Montigny le Bretonneux, France; AP-HP UVSQ, Occupational Health Unit/Population-Based Epidemiological Cohorts Unit, UMS 011, University Hospital of Poincaré, Garches, France. Electronic address:

Trichloroethylene (TCE) is a chlorinated solvent that has been used widely around the world in the twentieth century for metal degreasing and dry cleaning. Although TCE displays general toxicity and is classified as a human carcinogen, the association between TCE exposure and respiratory disorders are conflicting. In this review we aimed to systematically evaluate the current evidence for the respiratory effects of TCE exposure and the implications for the practicing clinician. Read More

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http://dx.doi.org/10.1016/j.rmed.2017.11.021DOI Listing
January 2018
75 Reads

[Clinical and imaging features of pulmonary veno-occlusive disease and pulmonary capillary hemangioma].

Zhonghua Jie He He Hu Xi Za Zhi 2018 Jan;41(1):41-46

Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medice, Shanghai 200433, China.

To improve the diagnosis and treatment of the pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangioma (PCH). The clinical features, radiological findings, laboratory testing and treatment in 8 cases of PVOD/PCH which was diagnosed from 2013 to 2017 were described. PVOD/PCH was rare. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1001-0939.2018.01.011DOI Listing
January 2018
9 Reads

Sinusoidal Obstruction Syndrome during Treatment for Wilms' Tumor: A Life-threatening Complication.

Indian J Med Paediatr Oncol 2017 Oct-Dec;38(4):447-451

Department of Pediatrics, Advanced Pediatrics Center, Pediatric Hematology-Oncology Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication.

Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Read More

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http://dx.doi.org/10.4103/ijmpo.ijmpo_188_16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5759062PMC
January 2018
5 Reads

Defibrotide sodium for the treatment of hepatic veno-occlusive disease/sinusoidal obstruction syndrome.

Expert Rev Clin Pharmacol 2018 Feb 5;11(2):113-124. Epub 2018 Jan 5.

f Hematology Department , Hôpital Saint Antoine, AP-HP, Université Pierre & Marie Curie , Paris , France.

Introduction: Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is an unpredictable condition associated with endothelial-cell damage due to conditioning for hematopoietic stem-cell transplantation (HSCT) or chemotherapy without HSCT. Mortality in patients with VOD/SOS and multi-organ dysfunction (MOD) may be >80%. Areas covered: Defibrotide is the only approved drug for the treatment of severe hepatic VOD/SOS after HSCT in the European Union and hepatic VOD/SOS with renal or pulmonary dysfunction in the United States. Read More

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http://dx.doi.org/10.1080/17512433.2018.1421943DOI Listing
February 2018
17 Reads

Global Pulmonary Vascular Remodeling in Pulmonary Hypertension Associated With Heart Failure and Preserved or Reduced Ejection Fraction.

Circulation 2018 Apr 15;137(17):1796-1810. Epub 2017 Dec 15.

Department of Cardiovascular Medicine (A.U.F., J.J.M., E.A.K., B.A.B., R.P.F., M.M.F.)

Background: We hypothesized that pulmonary venous hypertension in heart failure (HF) leads to predominate remodeling of pulmonary veins and that the severity of venous remodeling is associated with the severity of pulmonary hypertension (PH) in HF.

Methods: Patients with HF (n=108; 53 preserved and 55 reduced ejection fraction) with PH (HF-PH; pulmonary artery systolic pressure [PASP] ≥40 mm Hg) were compared to normal controls (n=12) and patients with primary pulmonary veno-occlusive disease (PVOD; n=17). In lung specimens from autopsy (control, HF-PH, and 7 PVOD) or surgery (10 PVOD), quantitative histomorphometry was performed in all analyzable arteries (n=4949), veins (n=7630), and small indeterminate vessels (IV; n=2168) to define percent medial thickness (arteries) and percent intimal thickness (%IT) (arteries, veins, and IV) relative to external diameter. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.117.031608DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5915920PMC
April 2018
17 Reads
4 Citations
14.430 Impact Factor

Pulmonary veno-occlusive disease: An important consideration in patients with pulmonary hypertension.

Respir Med 2017 Nov 20;132:203-209. Epub 2017 Oct 20.

Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University School of Medicine, Durham, NC, USA.

Pulmonary veno-occlusive disease is a rare subcategory of pulmonary arterial hypertension (WHO Group 1). The disease is poorly understood and difficult to diagnose; it has no definitive cure to date. These patients present with nonspecific symptoms, including dyspnea, exercise intolerance, and weakness. Read More

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http://dx.doi.org/10.1016/j.rmed.2017.10.015DOI Listing
November 2017
10 Reads

Outcomes of Surgery for Mixed Total Anomalous Pulmonary Venous Drainage in Children.

Semin Thorac Cardiovasc Surg 2017 Autumn;29(3):338-344. Epub 2017 May 29.

Department of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. Electronic address:

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. Read More

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http://dx.doi.org/10.1053/j.semtcvs.2017.05.010DOI Listing
December 2017
7 Reads

Pulmonary arterial hypertension and acute respiratory distress syndrome in a patient with adult-onset stills disease.

Pulm Circ 2017 Oct-Dec;7(4):797-802. Epub 2017 Jun 6.

2 Pulmonary Hypertension Program, Division of Pulmonary, Critical Care, and Sleep Medicine, University of Florida, Gainesville, FL, USA.

Adult-onset Still's disease (AOSD) is an inflammatory disorder characterized by recurrent fevers, arthralgia, leukocytosis, and a salmon-colored rash. Diagnosis is made based on the Yamaguchi criteria. Various cardiac and pulmonary manifestations have been described in association with AOSD, including acute respiratory distress syndrome (ARDS) and pulmonary arterial hypertension (PAH). Read More

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http://dx.doi.org/10.1177/2045893217712710DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5703120PMC
June 2017
1 Read

Pulmonary Veno-Occlusive Disease: Welcome to the PAHty (Bostonian for Party).

Circulation 2017 Nov;136(21):2034-2036

Pulmonary Center, Boston University School of Medicine, MA (H.W.F.).

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http://dx.doi.org/10.1161/CIRCULATIONAHA.117.031158DOI Listing
November 2017
1 Read

Optimisation of empirical antimicrobial therapy in patients with haematological malignancies and febrile neutropenia (How Long study): an open-label, randomised, controlled phase 4 trial.

Lancet Haematol 2017 Dec 15;4(12):e573-e583. Epub 2017 Nov 15.

Department of Infectious Diseases, Microbiology, and Preventive Medicine, University Hospital Virgen del Rocío-Institute of Biomedicine of Seville, Seville, Spain. Electronic address:

Background: Continuation of empirical antimicrobial therapy (EAT) for febrile neutropenia in patients with haematological malignancies until neutrophil recovery could prolong the therapy unnecessarily. We aimed to establish whether EAT discontinuation driven by a clinical approach regardless of neutrophil recovery would optimise the duration of therapy.

Methods: We did an investigator-driven, superiority, open-label, randomised, controlled phase 4 clinical trial in six academic hospitals in Spain. Read More

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http://dx.doi.org/10.1016/S2352-3026(17)30211-9DOI Listing
December 2017
14 Reads

Lung transplantation for mitomycin-induced pulmonary veno-occlusive disease.

Presse Med 2017 Dec 10;46(12 Pt 1):1223-1225. Epub 2017 Nov 10.

University Paris-Sud, faculté de médecine, Le-Kremlin-Bicêtre 94270, France; AP-HP, centre de référence de l'hypertension pulmonaire, département hospitalo-universitaire (DHU) Thorax Innovation (TORINO), service de pneumologie, hôpital de Bicêtre, Le-Kremlin-Bicêtre 94270, France; UMR_S 999, university Paris-Sud, Inserm, centre chirurgical Marie-Lannelongue, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), Le-Plessis-Robinson 92350, France. Electronic address:

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http://dx.doi.org/10.1016/j.lpm.2017.09.026DOI Listing
December 2017
90 Reads

Pulmonary vascular remodeling patterns and expression of general control nonderepressible 2 (GCN2) in pulmonary veno-occlusive disease.

J Heart Lung Transplant 2018 May 4;37(5):647-655. Epub 2017 Oct 4.

Institut National de la Santé et de la Recherche Unités Mixtes de Recherche_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Le Plessis-Robinson, Paris, France; Faculty of Medicine, Paris-South University, Kremlin-Bicêtre, Paris, France; Department of Pathology, Hôpital Marie Lannelongue, Le Plessis-Robinson, Paris, France. Electronic address:

Background: Heritable pulmonary veno-occlusive disease (PVOD) is linked to mutations in the eukaryotic initiation factor 2 alpha kinase 4 (EIF2AK4) gene, leading to a loss of general control nonderepressible 2 (GCN2). The role of GCN2 expression in pulmonary vascular remodeling remains obscure. We sought to identify specific histologic and biologic features in heritable PVOD. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10532498173203
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http://dx.doi.org/10.1016/j.healun.2017.09.022DOI Listing
May 2018
165 Reads

[Pulmonary arterial hypertension as leading manifestation of methylmalonic aciduria: clinical characteristics and gene testing in 15 cases].

Beijing Da Xue Xue Bao Yi Xue Ban 2017 10;49(5):768-777

Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.

Objective: To deepen our understanding of Methylmalonic aciduria (MMA) associated pulmonary hypertension (PH) by analyzing the characteristics of clinical presentation, pulmonary high resolusion CT(HRCT), treatment response and gene mutation.

Methods: This study includes 15 cases of pediatric patients with MMA associated PH diagnosed and treated in Peking University First Hospital pediatric department between May 2012 and May 2016 with symptoms of PH as their leading presentation. Clinical symptoms and signs were recorded, Routine blood laboratory examinations was done including arterial blood gas analysis. Read More

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October 2017
5 Reads

Prognostic significance of computed tomography criteria for pulmonary veno-occlusive disease in systemic sclerosis-pulmonary arterial hypertension.

Rheumatology (Oxford) 2017 12;56(12):2197-2203

National Pulmonary Hypertension Service, Royal Free London NHS Foundation Trust.

Objectives: SSc-pulmonary arterial hypertension (SSc-PAH) is associated with worse response to therapy and survival when compared with idiopathic PAH. It is suggested that the vasculopathy in SSc may involve postcapillary pulmonary venules resulting in pulmonary veno-occlusive disease (PVOD). This may underlie the lower gas transfer and worse outcome on therapy. Read More

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http://dx.doi.org/10.1093/rheumatology/kex351DOI Listing
December 2017
6 Reads

Phenotypic Characterization of Mutation Carriers in a Large Cohort of Patients Diagnosed Clinically With Pulmonary Arterial Hypertension.

Circulation 2017 11 28;136(21):2022-2033. Epub 2017 Sep 28.

Department of Medicine, University of Cambridge, UK (C.H., M.B., M.H., J.M., C.T., K.Y., M.N., M.T., S. Gräf, N.W.M.)

Background: Pulmonary arterial hypertension (PAH) is a rare disease with an emerging genetic basis. Heterozygous mutations in the gene encoding the bone morphogenetic protein receptor type 2 () are the commonest genetic cause of PAH, whereas biallelic mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 gene () are described in pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis. Here, we determine the frequency of these mutations and define the genotype-phenotype characteristics in a large cohort of patients diagnosed clinically with PAH. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.117.028351DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5700414PMC
November 2017
106 Reads

Pulmonary veno-occlusive disease in a pediatric hematopoietic stem cell transplant patient: a cautionary tale.

Leuk Lymphoma 2018 06 28;59(6):1494-1497. Epub 2017 Sep 28.

a Benioff Children's Hospital , University of California , San Francisco , CA , USA.

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http://dx.doi.org/10.1080/10428194.2017.1382697DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5897181PMC
June 2018
2 Reads

Pulmonary hypertension in systemic sclerosis: different phenotypes.

Eur Respir Rev 2017 Sep 27;26(145). Epub 2017 Sep 27.

Faculté de Médecine, Université Paris-Sud, Université Paris-Saclay, Le Kremlin-Bicêtre, Paris, France.

Pulmonary hypertension (PH) is a frequent and severe complication of systemic sclerosis (SSc). PH in SSc is highly heterogeneous because of the various clinical phenotypes of SSc itself and because the mechanisms of PH can vary from one patient to another. PH in SSc may be due to vasculopathy of the small pulmonary arteries (group 1; pulmonary arterial hypertension), interstitial lung disease (group 3; PH due to lung disease or chronic hypoxia) or myocardial fibrosis leading to left ventricular systolic or diastolic dysfunction (group 2; PH due to chronic left-heart disease). Read More

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http://dx.doi.org/10.1183/16000617.0056-2017DOI Listing
September 2017
61 Reads

Efficacy and safety of long-term imatinib therapy for patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis.

Respir Med 2017 10 12;131:215-219. Epub 2017 Sep 12.

Department of Clinical Science, National Hospital Organization Okayama Medical Center, Okayama, Japan; Department of Cardiology, National Hospital Organization Okayama Medical Center, Okayama, Japan. Electronic address:

Background: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are categorized as Group 1' in the clinical classification of pulmonary hypertension. No medical therapy has been proven to be effective in patients with PVOD/PCH. Imatinib is a molecular targeted drug and was expected to be effective in patients with pulmonary arterial hypertension. Read More

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http://dx.doi.org/10.1016/j.rmed.2017.08.032DOI Listing
October 2017
12 Reads