1,173 results match your criteria Pulmonary Venoocclusive Disease


A novel BMPR2 mutation with widely disparate heritable pulmonary arterial hypertension clinical phenotype.

Pulm Circ 2020 Jul-Sep;10(3):2045894020931315. Epub 2020 Jun 3.

Houston Methodist Hospital Research Institute, and Institute for Academic Medicine, Houston, TX, USA.

Mutations in the gene encoding bone morphogenetic protein receptor type II () have been associated with heritable pulmonary arterial hypertension (HPAH), whereas mutations in the gene encoding eukaryotic translation initiation factor 2 alpha kinase 4 () are associated with heritable pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (HPVOD/PCH). We describe two unrelated patients found to carry the same hitherto unreported pathogenic mutation; one of whom presented with typical pulmonary arterial hypertension, whereas the second patient presented with aggressive disease and characteristic clinical features of PVOD/PCH. These two clinically divergent cases representative of the same novel pathogenic mutation exemplify the variable phenotype of HPAH and the variable involvement of venules and capillaries in the pathology of the pulmonary vascular bed in pulmonary arterial hypertension. Read More

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http://dx.doi.org/10.1177/2045894020931315DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7273341PMC

Clinical characteristics and survival of Chinese patients diagnosed with pulmonary arterial hypertension who carry BMPR2 or EIF2KAK4 variants.

BMC Pulm Med 2020 May 29;20(1):150. Epub 2020 May 29.

State Key Laboratory of Cardiovascular Disease, Center of Pulmonary Vascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, North Lishi Road, Xicheng District, No.167, Beijing, China.

Background: Variants in the gene encoding bone morphogenetic protein receptor type II (BMPR2) are the most common genetic cause of pulmonary arterial hypertension (PAH), whereas biallelic variants in the eukaryotic translation initiation factor 2 alpha kinase 4 gene (EIF2AK4) are described in pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis (PVOD/PCH). Racial background may influence the clinical characteristics of patients diagnosed with PAH or PVOD/PCH. Here, we compared the clinical characteristics and survival between patients with BMPR2 variants or EIF2AK4 variants in a Chinese population. Read More

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http://dx.doi.org/10.1186/s12890-020-01179-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7257189PMC

Pulmonary capillary haemangiomatosis: a distinct entity?

Eur Respir Rev 2020 Jun 27;29(156). Epub 2020 May 27.

Inserm UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France

Pulmonary capillary haemangiomatosis (PCH) is a rare and incompletely understood histopathological finding characterised by abnormal capillary proliferation within the alveolar interstitium, which has long been noted to share many overlapping features with pulmonary veno-occlusive disease (PVOD). But are PCH and PVOD distinct entities that occur in isolation, or are they closely intertwined manifestations along a spectrum of the same disease? The classic clinical features of both PCH and PVOD include signs and symptoms related to pulmonary hypertension, hypoxaemia, markedly impaired diffusion capacity of the lung and abnormal chest imaging with ground glass opacities, septal lines and lymphadenopathy. In recent years, increasing evidence suggests that the clinical presentation, histopathological features, genetic substrate and pathobiological mechanisms of PCH and PVOD are overlapping and usually indistinguishable. Read More

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http://dx.doi.org/10.1183/16000617.0168-2019DOI Listing

Clinical heterogeneity of Pulmonary Arterial Hypertension associated with variants in TBX4.

PLoS One 2020 29;15(4):e0232216. Epub 2020 Apr 29.

Department of Cardiology, Pulmonary Hypertension Unit, Hospital Universitario Doce de Octubre, Madrid, Spain.

Background: The knowledge of hereditary predisposition has changed our understanding of Pulmonary Arterial Hypertension. Genetic testing has been widely extended and the application of Pulmonary Arterial Hypertension specific gene panels has allowed its inclusion in the diagnostic workup and increase the diagnostic ratio compared to the traditional sequencing techniques. This is particularly important in the differential diagnosis between Pulmonary Arterial Hypertension and Pulmonary Venoocclusive Disease. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0232216PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7190146PMC

Pulmonary vasodilators can lead to various complications in pulmonary "arterial" hypertension associated with congenital heart disease.

Heart Vessels 2020 Apr 13. Epub 2020 Apr 13.

Department of Pediatrics, Hokkaido University, Kita14, Nishi5, Kita-Ku, Sapporo, Hokkaido, 060-8648, Japan.

Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-ranging pathological mechanism in PAH could elucidate the clinical state of severe CHD-PAH. Read More

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http://dx.doi.org/10.1007/s00380-020-01604-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7152743PMC

Molecular Profiling of Vascular Remodeling in Chronic Pulmonary Disease.

Am J Pathol 2020 Jul 7;190(7):1382-1396. Epub 2020 Apr 7.

Institute of Pathology, Hannover Medical School, Hannover, Germany; German Center for Lung Research (DZL), Biomedical Research in End-stage and Obstructive Lung Disease Hannover (BREATH), Hannover, Germany.

Pulmonary hypertension and pulmonary vascular remodeling (PVR) are common in many lung diseases leading to right ventricular dysfunction and death. Differences in PVR result in significant prognostic divergences in both the pulmonary arterial and venous compartments, as in pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD), respectively. Our goal was to identify compartment-specific molecular hallmarks of PVR, considering the risk of life-threatening pulmonary edema in PVOD, if treated by conventional pulmonary hypertension therapy. Read More

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http://dx.doi.org/10.1016/j.ajpath.2020.03.008DOI Listing

Pulmonary vein occlusion and veno-occlusive disease in a bilateral lung transplant patient: A case report.

Respir Med Case Rep 2020 28;30:101031. Epub 2020 Feb 28.

Department of Radiology, University of Minnesota Medical School, Minneapolis, MN, USA.

A pulmonary vein occlusion and biopsy proven pulmonary veno-occlusive disease (PVOD) and hemangiomatosis is found in a bilateral lung transplant patient. A 61-year-old male presents with dyspnea and chest pain with minimal exertion at routine follow up on post-transplant day of 50. Chest CT demonstrates new occlusion of bilateral superior pulmonary veins and diffuse pulmonary edema. Read More

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http://dx.doi.org/10.1016/j.rmcr.2020.101031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7097520PMC
February 2020

Venoocclusive Disease With Both Hepatic and Pulmonary Involvement.

Chest 2020 Apr;157(4):e107-e109

Service de Pneumologie, Oncologie Thoracique et Allergologie Respiratoire, CHU de Besançon, Besançon, France. Electronic address:

Pulmonary venoocclusive disease (PVOD) is a rare form of pulmonary vascular disease with pulmonary hypertension characterized by preferential involvement of the pulmonary venous system. Hepatic venoocclusive disease (HVOD), also known as sinusoidal obstruction syndrome, is a condition that occurs in 13% to 15% of patients after hematopoietic stem cell transplantation (HSCT). Although hepatic and pulmonary venoocclusive diseases may share some pathologic features as well as some etiologies such as HSCT, these two disorders have never been described together in a single adult patient. Read More

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http://dx.doi.org/10.1016/j.chest.2019.11.021DOI Listing

Comparison of Human and Experimental Pulmonary Veno-Occlusive Disease.

Am J Respir Cell Mol Biol 2020 Jul;63(1):118-131

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

Pulmonary veno-occlusive disease (PVOD) occurs in humans either as a heritable form (hPVOD) due to biallelic inactivating mutations of encoding GCN2) or as a sporadic form in older age (sPVOD). The chemotherapeutic agent mitomycin C (MMC) is a potent inducer of PVOD in humans and in rats (MMC-PVOD). Here, we compared human hPVOD and sPVOD, and MMC-PVOD pathophysiology at the histological, cellular, and molecular levels to unravel common altered pathomechanisms. Read More

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http://dx.doi.org/10.1165/rcmb.2019-0015OCDOI Listing

Case 276: Pulmonary Veno-Occlusive Disease and Pulmonary Capillary Hemangiomatosis Disease.

Radiology 2020 Apr;295(1):240-244

From the Department of Radiology, CHU Brugmann, Place A. Van Gehuchten 4, 1020 Brussels, Belgium (C.D., M.W.); and Department of Radiology, CHR Haute Senne, Soignies, Belgium (M.H.).

HistoryA 34-year-old man presented to the emergency department of our hospital for progressive shortness of breath and worsening productive cough of 2 weeks duration. He reported a 10-kg weight loss over 4 months but denied experiencing fever, chills, night sweats, or gastrointestinal, musculoskeletal, or neurologic symptoms. His medical history was unremarkable. Read More

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http://dx.doi.org/10.1148/radiol.2020180473DOI Listing

Gas Exchange and Ventilatory Efficiency During Exercise in Pulmonary Vascular Diseases.

Arch Bronconeumol 2020 Feb 25. Epub 2020 Feb 25.

Sorbonne Université, INSERM, UMRS1158 Neurophysiologie respiratoire expérimentale et clinique, F-75005 Paris, France; APHP.Sorbonne Université, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service des Explorations Fonctionnelles de la Respiration, de l'Exercice et de la Dyspnée, Hôpitaux Universitaires Pitié-Salpêtrière, Tenon et Saint-Antoine, Département Médico-Universitaire "APPROCHES", F-75013 Paris, France. Electronic address:

Background And Objective: Ventilatory inefficiency (high V'/V'CO) and resting hypocapnia are common in pulmonary vascular disease and are associated with poor prognosis. Low resting PaCO suggests increased chemosensitivity or an altered PaCO set-point. We aimed to determine the relationships between exercise gas exchange variables reflecting the PaCO set-point, exercise capacity, hemodynamics and V'/V'CO. Read More

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http://dx.doi.org/10.1016/j.arbres.2019.12.030DOI Listing
February 2020

Pulmonary veno-occlusive disease: a probably underdiagnosed cause of pulmonary hypertension in systemic sclerosis.

Clin Rheumatol 2020 May 22;39(5):1687-1691. Epub 2020 Jan 22.

Cardiology Department, Hospital Garcia de Orta EPE, Avenida Torrado da Silva, 2805-267, Almada, Portugal.

Pulmonary hypertension is a serious complication of systemic sclerosis and remains one of the leading causes of mortality. Pulmonary veno-occlusive disease (PVOD), recently reclassified as pulmonary arterial hypertension (PAH) with overt features of venous/capillaries involvement, is a subgroup of group 1 pulmonary hypertension, which has been rarely reported in systemic sclerosis patients. It is symptomatically indistinguishable from idiopathic pulmonary arterial hypertension and should be suspected in those with manifestations of pulmonary arterial hypertension who have evidence of pulmonary venous congestion in the absence of left-sided heart disease. Read More

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http://dx.doi.org/10.1007/s10067-020-04953-4DOI Listing

Features of radiological and physiological findings in pulmonary capillary hemangiomatosis: an updated pooled analysis of confirmed diagnostic cases.

Pulm Circ 2019 Oct-Dec;9(4):2045894019896696. Epub 2019 Dec 27.

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

Pulmonary capillary hemangiomatosis (PCH) is a very rare and refractory disease characterized by capillary angioproliferation. The updated classification of pulmonary hypertension categorizes PCH into a subgroup of pulmonary arterial hypertension (PAH) alongside pulmonary veno-occlusive disease (PVOD). However, the definitive diagnosis of PCH only with noninvasive tools remains difficult. Read More

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http://dx.doi.org/10.1177/2045894019896696DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6935884PMC
December 2019

Targeted therapy in pulmonary veno-occlusive disease: time for a rethink?

BMC Pulm Med 2019 Dec 19;19(1):257. Epub 2019 Dec 19.

Center for Pulmonary Vascular Diseases, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishi Road, Xicheng District, Beijing, 100037, China.

Background: Pulmonary veno-occlusive disease (PVOD) is a rare condition with poor prognosis, and lung transplantation is recommended as the only curative therapy. The role of pulmonary arterial hypertension targeted therapy in PVOD remains controversial, and long-term effects of targeted therapy have been rarely reported. This study aims to retrospectively evaluate the role of targeted therapy in PVOD patients and the long-term outcome. Read More

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http://dx.doi.org/10.1186/s12890-019-1031-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6924010PMC
December 2019
2.489 Impact Factor

Commentary: It takes a village: Changing the trajectory of pulmonary vein stenosis outcomes.

J Thorac Cardiovasc Surg 2020 03 3;159(3):1037-1038. Epub 2019 Oct 3.

Division of Cardiovascular Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2019.09.100DOI Listing

An Autopsy Case of Pulmonary Capillary Hemangiomatosis with an Electron Microscopy Study.

Am J Case Rep 2019 Oct 22;20:1551-1557. Epub 2019 Oct 22.

Department of Pathology, Tachikawa General Hospital, Nagaoka, Niigata, Japan.

BACKGROUND Pulmonary capillary hemangiomatosis (PCH) and pulmonary veno-occlusive disease (PVOD) are rare diseases that share clinical, X-ray, and histological features. Most patients have poor prognosis due to severe respiratory impairment. Recently, EIF2AK4 mutations were found in some patients with PCH and PVOD, but the role of this mutation is still unknown. Read More

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http://dx.doi.org/10.12659/AJCR.918375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6818641PMC
October 2019

Evaluation and classification of pulmonary arterial hypertension.

Authors:
Sandeep Sahay

J Thorac Dis 2019 Sep;11(Suppl 14):S1789-S1799

Division of Pulmonary and Critical Care Medicine, Weill Cornell Medical College, Institute of Academic Medicine, Houston Methodist Hospital, Houston, TX, USA.

In early 2019, the 6th World Symposium on Pulmonary Hypertension (WSPH) released an updated document highlighting the advances in the last five years. During the quinquennial event many experts worked together to suggest new changes in the disease diagnosis and management. Since inception of the WSPH in 1973, this is the first time when the hemodynamic definition of pulmonary hypertension (PH) has been updated. Read More

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http://dx.doi.org/10.21037/jtd.2019.08.54DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6783722PMC
September 2019
2 Reads

Looking backwards: is it time to assess veno-atrial interactions in pulmonary arterial hypertension?

Eur Respir J 2019 10 17;54(4). Epub 2019 Oct 17.

Division of Translational and Regenerative Medicine, University of Arizona, Tucson, AZ, USA.

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http://dx.doi.org/10.1183/13993003.01598-2019DOI Listing
October 2019

Bilateral Lobar Transplants Using One Donor for Two Small-Sized Recipients.

Ann Thorac Surg 2020 05 3;109(5):e331-e334. Epub 2019 Oct 3.

Toronto Lung Transplant Program, University Health Network, Toronto, Ontario, Canada.

Cadaveric lobar lung transplantation is an alternative for patients whose chest cavities have small dimensions. We present here a case where 1 donor was used for bilateral lobar transplantations in 2 high-risk patients. Coordination between the graft preparation at the back table and the 2 concomitant lung transplant teams was necessary to minimize the ischemic injury of the grafts and to plan for adequate vascular and bronchial cuffs for both implantations. Read More

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http://dx.doi.org/10.1016/j.athoracsur.2019.08.078DOI Listing
May 2020
1 Read

Pulmonary capillary hemangiomatosis: a lesson learned.

Autops Case Rep 2019 Jul-Sep;9(3):e2019111. Epub 2019 Aug 22.

Cedars Sinai Medical Center, Departments of Pathology & Radiology. Los Angeles, CA, United States of America.

Pulmonary capillary hemangiomatosis (PCH) is a rare and controversial entity that is known to be a cause of pulmonary hypertension and is microscopically characterized by proliferation of dilated capillary-sized channels along and in the alveolar walls. Clinically, it is mostly seen in adults. Clinical features are characterized by nonspecific findings such as shortness of breath, cough, chest pain, and fatigue. Read More

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http://dx.doi.org/10.4322/acr.2019.111DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6709648PMC
August 2019
3 Reads

Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis.

Authors:
W Shi Y Jiao

QJM 2020 May;113(5):371-372

Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan Street, Dongcheng District, Beijing 100730, China.

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http://dx.doi.org/10.1093/qjmed/hcz233DOI Listing
May 2020
2 Reads

A case of early diagnosis of pulmonary capillary hemangiomatosis in a worker with exposure to silica.

BMC Pulm Med 2019 Jul 23;19(1):133. Epub 2019 Jul 23.

Division of Allergy and Pulmonology, Department of Internal Medicine, College of Medicine, Seoul St. Mary's Hospital, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul, 06591, Republic of Korea.

Background: Pulmonary capillary hemangiomatosis (PCH) is a progressive and refractory vascular disease in the lung. Pulmonary hypertension is frequently combined with PCH when capillary proliferation invades to nearby pulmonary vascular systems. It is difficult to differentiate PCH from other diseases such as pulmonary venoocclusive disease and pulmonary arterial hypertension that cause pulmonary hypertension as they frequently overlap. Read More

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http://dx.doi.org/10.1186/s12890-019-0896-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6651969PMC
July 2019
9 Reads

A Case of Pulmonary Veno-Occlusive Disease Following Hepatic Veno-Occlusive Disease After Autologous Hematopoietic Stem Cell Transplantation for Neuroblastoma.

J Pediatr Hematol Oncol 2019 Jul 19. Epub 2019 Jul 19.

Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.

Pulmonary veno-occlusive disease (PVOD) is an uncommon form of pulmonary hypertension that is usually difficult to diagnose and is refractory to conservative treatment. PVOD can occur in connection with high-dose chemotherapy or hematopoietic stem cell transplantation, similar to hepatic veno-occlusive disease (HVOD). Here, we present a case of neuroblastoma with PVOD following HVOD after high-dose chemotherapy that was resolved with conservative treatment. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001566DOI Listing
July 2019
4 Reads

A new palliative surgical technique for high risk Total anomalous pulmonary venous connection (Sarmast-Takriti shunt).

J Cardiothorac Surg 2019 Jul 1;14(1):127. Epub 2019 Jul 1.

Department of Cardiac Surgery Hospital, Damascus university, Mouasat Square Omar ben Abdulaziz Street, Damascus, Syria.

Background: Total Anomalous Pulmonary Venous Connection (TAPVC) is a rare heterogeneous condition That accounting for 1.5-3% of congenital heart diseases. It is characterized by failure of the Pulmonary Venous Confluence (PVC) to be directly connected to the left atrium in combination with a persistent splanchnic connection to the systemic venous circulation. Read More

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http://dx.doi.org/10.1186/s13019-019-0953-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6604196PMC
July 2019
7 Reads

The 6th World Symposium on Pulmonary Hypertension: what's old is new.

F1000Res 2019 19;8. Epub 2019 Jun 19.

Division of Pulmonary and Critical Care Medicine, Stanford University, Stanford, USA.

In February 2018, the 6th World Symposium on Pulmonary Hypertension (WSPH) brought together experts from various disciplines to review the most relevant clinical and scientific advances in the field of PH over the last 5 years. Based on careful review and discussions by members of the different task forces, major revisions were made on the hemodynamic definition for various forms of PH and new genes were added to the list of genetic markers associated with pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease. In addition, the use of risk stratification tools was encouraged as a strategy to reduce one-year mortality risk in PAH patients through early implementation of PAH therapies. Read More

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http://dx.doi.org/10.12688/f1000research.18811.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6584967PMC
June 2020
19 Reads

Real-time three-dimensional trans-oesophageal echocardiography-guided balloon dilation of pulmonary venous pathway obstruction in a patient with dextro-transposition of the great arteries after atrial switch surgery: a case report.

Cardiol Young 2019 Jul 24;29(7):983-985. Epub 2019 Jun 24.

Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

A percutaneous transcatheter balloon dilation of a pulmonary venous pathway obstruction was successfully performed in a 40-year-old patient after a Mustard procedure. During the procedure, real-time three-dimensional trans-oesophageal echocardiography demonstrated the morphology of the obstruction. Our case highlights the usefulness of real-time three-dimensional trans-oesophageal echocardiography as a guide for transcatheter intervention in the increasing number of adults with CHD. Read More

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http://dx.doi.org/10.1017/S1047951119001045DOI Listing
July 2019
7 Reads

Unique wreath-like smooth muscle proliferation of the pulmonary vasculature in pulmonary veno-occlusive disease versus pulmonary arterial hypertension.

J Formos Med Assoc 2020 Jan 13;119(1 Pt 2):300-309. Epub 2019 Jun 13.

Division of Thoracic Surgery, Department of Surgery, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, 10002, Taiwan. Electronic address:

Background/purpose: Pulmonary veno-occlusive disease (PVOD) is a rare but fatal cause of pulmonary hypertension reported to be linked to mutations of eukaryotic initiation factor 2 alpha kinase 4 (EIF2AK4), also known as general control nonderepressible 2 kinase (GCN2). PVOD is difficult to diagnose and often initially misdiagnosed as other types of idiopathic pulmonary arterial hypertension (IPAH). To rapidly and correctly identify PVOD patients and explore the possible pathogenesis, we thoroughly investigated histopathological features and GCN2 protein levels in non-PAH, PVOD and PAH patients. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09296646193028
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http://dx.doi.org/10.1016/j.jfma.2019.05.019DOI Listing
January 2020
14 Reads

Computed tomographic and clinical features of pulmonary veno-occlusive disease: raising the radiologist's awareness.

Clin Radiol 2019 Sep 6;74(9):655-662. Epub 2019 Jun 6.

Royal United Hospital, Combe Park, Avon BA13NG, UK. Electronic address:

Pulmonary veno-occlusive disease (PVOD) is a rare subtype of pulmonary arterial hypertension (PAH) characterised by preferential remodelling of the pulmonary venules. Differentiation from other subtypes of PAH is essential as the management can differ significantly; for example, initiation of vasodilator therapy may cause fatal pulmonary oedema in a patient with PVOD misdiagnosed with idiopathic PAH. PVOD also carries a substantially worse prognosis. Read More

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http://dx.doi.org/10.1016/j.crad.2019.04.023DOI Listing
September 2019
5 Reads

Racial and Ethnic Differences in Pediatric Pulmonary Hypertension: An Analysis of the Pediatric Pulmonary Hypertension Network Registry.

J Pediatr 2019 08 5;211:63-71.e6. Epub 2019 Jun 5.

Computational Health Informatics Program, Harvard Medical School & Boston Children's Hospital, Boston, MA.

Objective: To investigate racial and ethnic differences in pulmonary hypertension subtypes and survival differences in a pediatric population.

Study Design: This was a retrospective analysis of a cohort of patients with pulmonary hypertension (aged ≤18 years) enrolled in the Pediatric Pulmonary Hypertension Network registry between 2014 and 2018, comprising patients at eight Pediatric Centers throughout North America (n = 1417).

Results: Among children diagnosed after the neonatal period, pulmonary arterial hypertension was more prevalent among Asians (OR, 1. Read More

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http://dx.doi.org/10.1016/j.jpeds.2019.04.046DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6776463PMC
August 2019
12 Reads

United States Pulmonary Hypertension Scientific Registry (USPHSR): rationale, design, and clinical implications.

Pulm Circ 2019 Apr-Jun;9(2):2045894019851696

10 Division of Human Genetics, Cincinnati Children's Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.

Diagnostic World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH) and Diagnostic Group 1' pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH) are progressive and fatal disorders. Past registries provided important insights into these disorders, but did not include hormonal exposures or genomic data. The United States Pulmonary Hypertension Scientific Registry (USPHSR) will provide demographic, physiologic, anorexigen and hormone exposure, genomic, and survival data in the current therapeutic era for 499 patients diagnosed with PAH, PVOD, or PCH. Read More

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http://dx.doi.org/10.1177/2045894019851696DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6540712PMC
May 2019
7 Reads

Vascular Anomalies of the Pediatric Liver.

Radiographics 2019 May-Jun;39(3):842-856

From the Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S Kingshighway Blvd, Campus Box 8131-MIR, St. Louis, MO 63110.

The liver is a unique organ as it receives afferent blood supply from the umbilical vein, portal vein, and hepatic artery in the developing embryo but has only one efferent drainage method, through the hepatic veins. In the postnatal period, about 70% of the afferent blood flow into the liver is from the portal venous system, unique vessels that begin and end in a capillary system. Vascular anomalies of the hepatic artery, hepatic veins, portal vein, and/or umbilical vein can be congenital or acquired secondary to inflammation and/or infection, trauma, systemic disorders, or iatrogenic causes. Read More

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http://dx.doi.org/10.1148/rg.2019180146DOI Listing
March 2020
12 Reads

Pulmonary Veno-Occlusive Disease: A Rare Cause of Pulmonary Hypertension.

J Investig Med High Impact Case Rep 2019 Jan-Dec;7:2324709619840375

1 MedStar Washington Hospital Center, Washington, DC, USA.

Pulmonary veno-occlusive disease (PVOD) is a rare entity that is usually mistaken with pulmonary arterial hypertension (PAH) but is considered class I' of PAH. It is important to subclassify PVOD and distinguish it from PAH as treatment with vasodilators in PVOD patients is controversial and may be fatal. In this article, we describe a case of PVOD and how we diagnosed it. Read More

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http://dx.doi.org/10.1177/2324709619840375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6480994PMC
June 2020
6 Reads

Outcomes of Reinterventions for Children with Postoperative Pulmonary Venous Restenosis.

Pediatr Cardiol 2019 Jun 1;40(5):965-972. Epub 2019 Apr 1.

Department of Cardiothoracic Surgery, School of Medicine, Heart Center, Shanghai Children's Medical Center, Shanghai Jiaotong University, Dongfang Road 1678, Shanghai, China.

This report aims to summarize and evaluate the efficacy and experience of reintervention for children with postoperative pulmonary venous restenosis. Clinical data from 61 patients with postoperative pulmonary venous restenosis who underwent reintervention at Shanghai Children's Medical Center (SCMC) from September 2009 to June 2018 were retrospectively analyzed. The patients comprised 38 boys and 23 girls, with a mean age of 15. Read More

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http://link.springer.com/10.1007/s00246-019-02098-z
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http://dx.doi.org/10.1007/s00246-019-02098-zDOI Listing
June 2019
17 Reads

Understanding the Similarities and Differences between Hepatic and Pulmonary Veno-Occlusive Disease.

Am J Pathol 2019 06 26;189(6):1159-1175. Epub 2019 Mar 26.

INSERM UMR_S 999, Pulmonary Arterial Hypertension: Physiopathology and Therapeutic Innovation, Marie Lannelongue Hospital, Le Plessis-Robinson, France; Department of Respiratory Medicine, University Paris-Sud, Medical School, University Paris-Saclay, Hôpitaux de Paris, National Reference Center for Severe Pulmonary Hypertension, Bicêtre Hospital, Le Kremlin Bicêtre, France. Electronic address:

Hepatic veno-occlusive disease (HVOD), alias sinusoidal obstruction syndrome, may develop as a complication of chemotherapy in the setting of hematopoietic stem cell transplantation. HVOD is less frequently described after exposure to chemotherapy in the nontransplant setting and can also be a complication after ingestion of toxins, such as pyrrolizidine alkaloids. Veno-occlusive disease may also affect the lungs, and it is therefore termed pulmonary veno-occlusive disease (PVOD). Read More

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http://dx.doi.org/10.1016/j.ajpath.2019.02.007DOI Listing
June 2019
36 Reads

Inotuzumab ozogamicin versus standard of care in relapsed or refractory acute lymphoblastic leukemia: Final report and long-term survival follow-up from the randomized, phase 3 INO-VATE study.

Cancer 2019 07 28;125(14):2474-2487. Epub 2019 Mar 28.

Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio.

Background: Inotuzumab ozogamicin (InO) is an antibody-drug conjugate used for adults with relapsed/refractory B-cell precursor (BCP) acute lymphoblastic leukemia (ALL). The INotuzumab Ozogamicin trial to inVestigAte Tolerability and Efficacy (INO-VATE) previously reported improved outcomes with InO versus standard-of-care (SoC) chemotherapy. This article reports the final INO-VATE results (≥2 years of follow-up) and additional analyses of patient characteristics associated with improved outcomes. Read More

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http://dx.doi.org/10.1002/cncr.32116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6618133PMC
July 2019
10 Reads

Systematic review of defibrotide studies in the treatment of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS).

Bone Marrow Transplant 2019 12 25;54(12):1951-1962. Epub 2019 Feb 25.

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

Veno-occlusive disease (VOD), also called sinusoidal obstruction syndrome (SOS), is a potentially life-threatening complication of hematopoietic stem cell transplantation (HSCT) conditioning or high-dose nontransplant chemotherapy. VOD/SOS with multi-organ dysfunction (MOD) is associated with a mortality rate of > 80%. Defibrotide (25 mg/kg/day) is approved to treat hepatic VOD/SOS with renal or pulmonary dysfunction post HSCT in the United States and to treat severe hepatic VOD/SOS in patients > 1 month of age in the European Union. Read More

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http://dx.doi.org/10.1038/s41409-019-0474-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6957462PMC
December 2019
5 Reads

Pulmonary veno-occlusive disease is highly prevalent in scleroderma patients undergoing lung transplantation.

ERJ Open Res 2019 Feb 11;5(1). Epub 2019 Feb 11.

Division of Pulmonary Medicine, Dept of Medicine, National Jewish Health, Denver, CO, USA.

http://ow.ly/vPvc30neJZV. Read More

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http://dx.doi.org/10.1183/23120541.00168-2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6368993PMC
February 2019
25 Reads

Comparison of conventional and primary sutureless surgery for repairing supracardiac total anomalous pulmonary venous drainage.

J Cardiothorac Surg 2019 Feb 8;14(1):34. Epub 2019 Feb 8.

Department of Cardiovascular surgery, Chongqing Three Gorges Central Hospital, Chongqing, 404000, China.

Objective: The efficacy of using a sutureless approach in order to surgically manage postoperative pulmonary vein stenosis following total anomalous pulmonary venous drainage (TAPVD) has been reported, though outcomes of primary treatment of supracardiac TAPVD remain unclear. We retrospectively reviewed our cardiac center experience, and compared the differences in mid-term outcomes for those patients that received conventional surgery and those that underwent sutureless technique for the primary repair of supracardiac TAPVD.

Methods: A total of 43 patients (median age, 199 days; range, 35 days to 1572 days) with supracardiac TAPVD underwent surgical treatment at our cardiac center from 2014 to 2018 were studied retrospectively. Read More

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http://dx.doi.org/10.1186/s13019-019-0853-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6368785PMC
February 2019
7 Reads

Tumoral pulmonary hypertension.

Eur Respir Rev 2019 Mar 6;28(151). Epub 2019 Feb 6.

National Pulmonary Hypertension Service, Royal Brompton Hospital, Imperial College London, London, UK.

Tumoral pulmonary hypertension (PH) comprises a variety of subtypes in patients with a current or previous malignancy. Tumoral PH principally includes the tumour-related pulmonary microvascular conditions pulmonary tumour microembolism and pulmonary tumour thrombotic microangiopathy. These inter-related conditions are frequently found in specimens but are notoriously difficult to diagnose The outlook for patients remains extremely poor although there is some emerging evidence that pulmonary vasodilators and anti-inflammatory approaches may improve survival. Read More

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http://dx.doi.org/10.1183/16000617.0065-2018DOI Listing
March 2019
33 Reads

Beneficial Effects of Imatinib in a Patient with Suspected Pulmonary Veno-Occlusive Disease.

Tohoku J Exp Med 2019 02;247(2):69-73

Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine.

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH). The prognosis of PVOD patients remains poor, since no effective medical therapy is yet available. Imatinib is a tyrosine kinase inhibitor specific for platelet-derived growth factor receptor and is expected as a treatment option for pulmonary arterial hypertension (PAH). Read More

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http://dx.doi.org/10.1620/tjem.247.69DOI Listing
February 2019
7 Reads

Comprehensive three-dimensional morphology of neoangiogenesis in pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis.

J Pathol Clin Res 2019 04 27;5(2):108-114. Epub 2019 Feb 27.

Institute of Pathology, Hannover Medical School, Hannover, Germany.

Pulmonary veno-occlusive disease (PVOD) is a rare lung disease characterized by fibrotic narrowing of pulmonary veins leading to pulmonary hypertension (PH) and finally to death by right heart failure. PVOD is often accompanied by pulmonary capillary hemangiomatosis (PCH), a marked abnormal proliferation of pulmonary capillaries. Both morphological patterns often occur together and are thought to be distinct manifestations of the same disease process and accordingly are classified together in group 1' of the Nice classification of PH. Read More

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http://dx.doi.org/10.1002/cjp2.125DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6463863PMC
April 2019
29 Reads

A Combined Targeted and Whole Exome Sequencing Approach Identified Novel Candidate Genes Involved in Heritable Pulmonary Arterial Hypertension.

Sci Rep 2019 01 24;9(1):753. Epub 2019 Jan 24.

Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

The pathogenesis of idiopathic and heritable forms of pulmonary arterial hypertension is still not completely understood, even though several causative genes have been proposed, so that a third of patients remains genetically unresolved. Here we applied a multistep approach to extend identification of the genetic bases of such a disease by searching for novel candidate genes/pathways. Twenty-eight patients belonging to 18 families were screened for BMPR2 mutations and BMPR2-negative samples were tested for 12 additional candidate genes by means of a specific massive parallel sequencing-based assay. Read More

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http://dx.doi.org/10.1038/s41598-018-37277-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6345742PMC
January 2019
17 Reads

Metakaryotic cells linked to pediatric pulmonary vein stenosis.

Cardiovasc Pathol 2019 Mar - Apr;39:51-53. Epub 2018 Dec 21.

Laboratory in Metakaryotic Biology (LIMB), Department of Biological Engineering, Massachusetts Institute of Technology.

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http://dx.doi.org/10.1016/j.carpath.2018.12.005DOI Listing
June 2019
9 Reads

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

Pediatr Radiol 2019 05 16;49(5):575-585. 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
May 2019
11 Reads

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

Eur Respir J 2019 03 14;53(3). Epub 2019 Mar 14.

Département de Génétique, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France.

Background: Heritable forms of pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis (PVOD/PCH) diverge by lung histopathological lesions, clinical and para-clinical presentation, their responsible genes, and mode of transmission. Since the identification of the 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
March 2019
49 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 2019 Apr 18;58(7):955-964. 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6478985PMC
April 2019
5 Reads

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

Eur Respir J 2019 01 24;53(1). Epub 2019 Jan 24.

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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351340PMC
January 2019
71 Reads

Haemodynamic definitions and updated clinical classification of pulmonary hypertension.

Eur Respir J 2019 01 24;53(1). Epub 2019 Jan 24.

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
Publisher Site
http://dx.doi.org/10.1183/13993003.01913-2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351336PMC
January 2019
45 Reads