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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary arterial hypertension (PAH) is a term which has recently been redefined and includes idiopathic pulmonary arterial hypertension, familial pulmonary arterial hypertension PAH related to specific pathological conditions (e.g. connective tissue diseases), as well as PAH caused by veno-occlusive disease or capillary hemangiomatosis. The clinical manifestation seems to be related to a peculiar pathological anatomy involving small, muscular pulmonary arteries, capillaries and veins. In addition to common hypertrophy of the tunica media, other vascular compartments may also be affected by intimal thickening or adventitial fibrosis. Moreover, complex lesions, such as so called plexiform lesions and arteritis can be present in certain forms of the disease. While the recent identification of responsible gene mutations in subgroups of patients have shed some light on disease evolution, therapeutic strategies must currently rely on vasodilative and antimitogenic drugs acting on the intimal and medial level of the affected pulmonary vessels. The clinical outcome of patients suffering from PAH remains poor, underlining our need for a better comprehension of disease pathophysiology, and thus for the characterization of specific histomorphological patterns.
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PMID:[Update on the pathomorphological assessment of vasculopathies in pulmonary arterial hypertension]. 1645 85

We report 2 cases of capillary hemangioma, each presenting as a solitary nodule in the peripheral lung. Both of the patients were asymptomatic with a small solitary nodule that had revealed by computed tomography. In both cases, the nodule was resected surgically under a clinical diagnosis of early lung cancer. Macroscopically, each lesion was ill defined and irregular in shape with a dark brown cut surface. Microscopically, the alveolar septa in both nodules were thickened by accumulations of numerous thin-walled capillary vessels, which characteristically extended along, or infiltrated, each septum. We diagnosed these lesions as "solitary capillary hemangioma" of the peripheral lung. Tumors or tumorlike lesions of capillary vessels in the lung are rare. Among them, pulmonary capillary hemangiomatosis (PCH) has been described as multiple nodules in the lung parenchyma or bronchovascular walls, comprised of infiltrating thin-walled capillary blood vessels. Moreover, PCH-like foci have been found in a retrospective study of autopsy cases. However, the presented cases should be differentiated from PCH in terms of their clinical setting such as history of hypertension or veno-occlusive disease and multiplicity of the lesion. This is a rare case series of solitary capillary hemangioma discovered incidentally during life, and the lesions were difficult to differentiate radiologically from early lung cancer. After the recent advances in imaging diagnosis for early detection of peripheral lung cancer, these lesions are important to bear in mind for differential diagnosis of bronchioloalveolar carcinoma.
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PMID:Solitary capillary hemangioma of the lung: report of 2 resected cases detected by high-resolution CT. 1672 54

The platelet-derived growth factor receptor inhibitor imatinib has demonstrated clinical and haemodynamical improvement in both animal models of pulmonary hypertension (PH) and patients with PH. It has been suggested that anti-proliferative effects on pulmonary vascular smooth muscle cells are responsible for these beneficial effects. The current study describes a patient with pulmonary arterial hypertension associated with a suspected pulmonary veno-occlusive disease. Treatment with imatinib resulted in rapid clinical improvement and decrease of ground-glass opacities and lobular septal thickening on high-resolution computed tomography. Based on these findings and on in vitro effects of imatinib on permeability of the endothelium, the authors hypothesise that the rapid clinical outcome is partly due to effects of imatinib on vascular integrity.
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PMID:Possible role of imatinib in clinical pulmonary veno-occlusive disease. 1859 41

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension characterised by extensive fibrotic occlusion of pulmonary veins. PVOD has a similar insidious presentation to idiopathic pulmonary arterial hypertension but responds poorly to conventional therapies and has a worse prognosis. The current study reports the case of a Caucasian female with a long history of progressive dyspnoea ultimately diagnosed as focal granulomatous venulitis leading to a pulmonary veno-occlusive disease-like pathology. The present study highlights the challenges in diagnosing and treating this condition.
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PMID:Granulomatous angiitis leading to a pulmonary veno-occlusive disease-like picture. 1925 2

Systemic sclerosis-associated pulmonary arterial hypertension (SScPAH) has a worse prognosis and response to pulmonary arterial hypertension (PAH) therapy than idiopathic PAH (IPAH). These differences have not yet been explained. Knowledge concerning histological pulmonary vasculopathy in SScPAH is limited in contrast to IPAH. Therefore, we explored patterns of vasculopathy in SScPAH compared with IPAH. Parameters of vasculopathy were assessed from lung tissue of eight PAH patients with limited cutaneous systemic sclerosis and 11 IPAH patients. Lung tissue was obtained at autopsy (n = 15), explantation (n = 3) and biopsy (n = 1). Pulmonary arterial/arteriolar intimal fibrosis was identified in all SScPAH patients and in three IPAH patients (p = 0.003). Fibrosis of pulmonary veins/venules was found in all SScPAH patients and in three IPAH patients (p = 0.003). In four SScPAH patients, fibrosis of veins/venules was focal and associated with capillary congestion as in pulmonary veno-occlusive disease (PVOD). Of the IPAH patients, 10 had unequivocal evidence of plexogenic arteriopathy compared with none of the SScPAH patients (p = 0.001). SScPAH is characterised by small vessel intimal fibrosis, which is associated with a PVOD-like pattern in some cases. This might explain its different clinical behaviour from IPAH. Small vessel intimal fibrosis may provide clues to elucidation of differences in pathogenetic mechanisms between the groups.
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PMID:Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy. 2004 65

Haematopoietic stem cell transplantation is currently the only curative option for many haematological malignancies, but is characterized by a wide spectrum of complications, including haemostatic changes. Bleeding and thrombotic events occur in the early and late phases after transplantation. In the early phase, thrombotic events have a variable clinical picture and present either as venous thrombosis, mainly at the site of central venous lines, veno-occlusive disease (also known as sinusoid occlusion syndrome) or transplant-associated microangiopathy. The latter two occur in the context of an acute graft-vs-host reaction, which involves various organs including the endothelium. In the late phase, years or decades after transplantation, thrombotic events present either as common venous thromboses or as arterial occlusions because of the development of premature atherosclerosis combined with diabetes, hypertension and dyslipidaemia, all of which are accelerated under the influence of the post-transplant treatment. This chapter will discuss the incidence, possible causative associations and treatment options of early and late thrombotic events after haematopoietic stem cell transplantation.
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PMID:Thrombotic complications after haematopoietic stem cell transplantation: early and late effects. 1928 80

The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH initially adopted in 1998 during the 2nd World Symposium was slightly modified. During the 4th World Symposium held in 2008, it was decided to maintain the general architecture and philosophy of the previous clinical classifications. The modifications adopted during this meeting principally concern Group 1, pulmonary arterial hypertension (PAH). This subgroup includes patients with PAH with a family history or patients with idiopathic PAH with germline mutations (e.g., bone morphogenetic protein receptor-2, activin receptor-like kinase type 1, and endoglin). In the new classification, schistosomiasis and chronic hemolytic anemia appear as separate entities in the subgroup of PAH associated with identified diseases. Finally, it was decided to place pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis in a separate group, distinct from but very close to Group 1 (now called Group 1'). Thus, Group 1 of PAH is now more homogeneous.
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PMID:Updated clinical classification of pulmonary hypertension. 2479 26

The new classification of pulmonary hypertension proposed in the joint European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines, combines pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) from separate categories into a single subcategory within pulmonary arterial hypertension (PAH) because of specific similarities in their diagnosis, prognosis, and management. These diseases are characterized histologically by their predominant involvement of small pulmonary veins (PVOD) and capillaries (PCH). Their precise prevalence is not known, but they are thought to account for 5 to 10% of the forms of PAH initially considered idiopathic. They cannot be distinguished from idiopathic PAH by their clinical or hemodynamic presentation. Only pathology examination can confirm the diagnosis, but lung biopsies are high-risk procedures and not recommended. A less invasive approach combining chest CT (centrilobular ground-glass opacities, septal lines, and mediastinal adenopathy), blood gases (resting hypoxemia), lung function tests [collapse in carbon monoxide diffusion (DLCO)] and bronchoalveolar lavage (occult intra-alveolar hemorrhage) makes it possible to screen the patients at risk of PVOD or HCP and thus avoid a lung biopsy. These patients have a poor prognosis and are at risk of developing severe pulmonary edema after the initiation of specific treatment for PAH. In view of their limited response to specific treatment and poor prognosis, pulmonary transplantation remains the treatment of choice for PVOD and HCP. In patients with the most severe disease, the prudent use of continuous intravenous epoprostenol, can serve as bridge-therapy while awaiting a lung transplant.
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PMID:[Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis]. 1991 27

The authors present the case of a 22-year-old female patient, white, referred to the cardiovascular outpatient clinic in November 2006 for pulmonary arterial hypertension. Complementary diagnostic exams revealed elevated pulmonary arterial pressure, normal capillary wedge pressure and a reduced cardiac index on invasive hemodynamic study. A high-resolution pulmonary CT scan identified a diffuse ground-glass opacity with a centrilobular pattern, and a marked decrease in CO diffusion on respiratory function assessment. An open lung biopsy was accordingly performed in January 2007, which was compatible with pulmonary arterial hypertension with associated venous lesions: pulmonary veno-occlusive disease. Therapy was begun with oxygen support, warfarin and bosentan (62.5 mg twice a day) Monthly follow-up was maintained, but her clinical and functional status progressively worsened and one year after the diagnosis the patient was admitted to our heart failure clinic for acute right heart failure. She was stabilized with inotropic therapy and diuretics and was subsequently referred to an international pulmonary transplantation center. The authors highlight the diagnostic challenge of this entity and its poor response to medical therapy and dismal prognosis.
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PMID:Pulmonary veno-occlusive disease: a case report. 2039 3

The Ministry of Health, Labour and Welfare (Japan) has approved research into primary pulmonary hypertension (PPH) and pulmonary hypertension due to chronic thromboembolic and/or embolic disease (CTE-PH) to examine their epidemiology, pathophysiology, and develop new therapeutic strategies. The Respiratory Failure Research Group, with grant support from the Ministry of Health, Labour and Welfare, changed the diagnostic names of PPH and CTE-PH. The Specific Diseases Control Division in the Health Service Bureau of the Ministry of Health, Labour and Welfare supported our proposal. One of the major purposes of The Respiratory Failure Research Group has been to maintain and, if possible, promote patient quality of life and prognosis in cases of intractable respiratory diseases. The name PPH has been changed to "pulmonary arterial hypertension (PAH)", and the name CTE-PH has been changed to "chronic thromboembolic pulmonary hypertension (CTEPH)", in keeping with recent worldwide research progress in this field. PAH should be subdivided into different pathophysiologic conditions, such as idiopathic and hereditary PAH, PAH associated with connective tissue diseases, portal hypertension, congenital heart disease, persistent pulmonary hypertension in newborn babies, pulmonary veno-occlusive disease etc. Different therapeutic strategies may be adopted for different subgroups. Pulmonary hypertension due to left heart disease, lung disease and/or hypoxia and CTEPH should be excluded from PAH. Continuous monitoring of PAH and CTEPH is required in patients with these conditions, even if the degree of pulmonary hypertension is improved by therapeutic intervention, because these diseases are incurable.
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PMID:[Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension]. 2080 71


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