Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic pulmonary arterial hypertension (PAH) is included in Group 1 of PAH classification, while pulmonary veno-occlusive disease (PVOD), pulmonary capillary hemangiomatosis (PCH), portopulmonary hypertension (PoPH) and HIV-associated PAH constitute the subgroups of Group I PAH disorders. PVOD has similar clinical presentation, hemodynamic characteristics and genetic background with PAH although main pathology is in venules and definite diagnosis is done by biopsy although not recommended due to high risk of bleeding. PCH, a rare idiopathic lung disorder, causes PAH in young adults and mostly is diagnosed after death by autopsy. PoPH is defined as PAH due to primary chronic liver disease associated with intrapulmonary vascular dilatations and hypoxia. HIV associated PAH is a rare complication of HIV-infection, and it is seen especially in HIV-infected patients with intravenous drug abuse.
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PMID:[Other causes of pulmonary arterial hypertension: pulmonary veno-occlusive disease, pulmonary capillary hemangiomatosis, porto-pulmonary hypertension, HIV- associated pulmonary arterial hypertension]. 2081 69

A 27-yr-old female with a 6-month diagnosis of idiopathic pulmonary arterial hypertension (PAH) confirmed elsewhere was referred to our centre with worsening dyspnoea. On examination, the patient had low systemic oxygen saturation despite high oxygen flow and reduced exercise capacity. Haemodynamics were indicative of severe pre-capillary PAH. High-resolution computed tomography revealed diffuse ground-glass opacity with thickening interlobular septa, and haemosiderin-laden macrophages were identified by bronchoalveolar lavage. Based on clinical and diagnostic findings, the patient was re-diagnosed with pulmonary veno-occlusive disease (PVOD). Treatment with high-dose diuretics and the endothelin-receptor antagonist bosentan improved the patient's exercise capacity, haemodynamics and quality of life. However, 1 yr later there was a progressive, slow deterioration in the patient's functional capacity and oxygen saturation, and inhaled prostanoid and oxygen therapy were initiated. Despite some subjective improvements, the patient's haemodynamics and oxygen saturation continued to decline and she underwent lung transplantation. This case emphasises that PVOD is an under-recognised and often misdiagnosed form of pulmonary hypertension. Therefore, accurate diagnosis of PVOD requires comprehensive clinical and diagnostic work-up. While lung transplantation remains the treatment of choice for patients with PVOD, targeted therapies for PAH in addition to high doses of diuretics merit evaluation.
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PMID:Pulmonary veno-occlusive disease misdiagnosed as idiopathic pulmonary arterial hypertension. 2095 40

Pulmonary hypertension (PH) comprises a heterogeneous group of disorders characterised by increased pulmonary vascular resistance that results in progressive right ventricular failure. In order to translate current evidence into routine clinical practice, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) have recently jointly proposed evidence-based guidelines for the optimal management of different PH patient groups. This article describes a series of clinical cases of PH due to various aetiologies that were referred to a large national PH expert referral centre. In each case, the assessment and therapeutic approach undertaken is described in the context of the new ECS/ERS guidelines. The routine diagnostic work-up of suspected idiopathic pulmonary arterial hypertension (PAH) and recommended treatments for patients with functional class II, III and IV disease is emphasised. Familial screening and management of heritable PAH is discussed. Appropriate investigation and therapeutic strategies for patients with chronic thromboembolic disease and PH that is associated with congenital heart disease, pulmonary veno-occlusive disease and systemic sclerosis are also highlighted.
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PMID:Implementing the ESC/ERS pulmonary hypertension guidelines: real-life cases from a national referral centre. 2095 52

Pulmonary hypertension may primarily affect either the arterial (precapillary) or the venous (postcapillary) pulmonary circulation. Pulmonary arterial hypertension may be idiopathic or arise in association with chronic pulmonary thromboembolism; pulmonary embolism caused by tumor cells, parasitic material, or foreign material; parenchymal lung disease; liver disease; vasculitis; human immunodeficiency virus infection; or a left-to-right cardiac shunt. Its histologic characteristics include vascular changes-medial hypertrophy, intimal cellular proliferation, intraluminal thrombosis, and the development of plexiform lesions-that manifest primarily in the muscular pulmonary arteries. Features of pulmonary arterial hypertension that may be seen at computed tomography (CT) are central pulmonary artery dilatation, abrupt narrowing or tapering of peripheral pulmonary vessels, right ventricular hypertrophy, right ventricular and atrial enlargement, dilated bronchial arteries, and a mosaic pattern of attenuation due to variable lung perfusion. Pulmonary venous hypertension may result from pulmonary veno-occlusive disease, pulmonary venous compression by extrinsic lesions (eg, mediastinal fibrosis), left-sided cardiac disease, or pulmonary vein stenosis. Its histologic hallmarks include venous intimal cellular proliferation, medial hypertrophy, and thickening of the internal elastic lamina; capillary congestion and proliferation; interlobular septal thickening; lymphatic dilatation; and, sometimes, venous infarction and vascular changes characteristic of pulmonary arterial hypertension. CT scans in patients with pulmonary venous hypertension show pulmonary interstitial and alveolar edema with signs of pulmonary arterial hypertension. High-resolution CT with standard axial and angiographic acquisitions is useful for identifying underlying disorders and differentiating among the various causes of secondary pulmonary hypertension.
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PMID:CT findings in diseases associated with pulmonary hypertension: a current review. 2105 19

Pulmonary hypertension (PH) is a serious complication of connective tissue diseases. The prevalence of PH in systemic lupus erythematosus (SLE) ranges from 0.5 to 17.5%, depending on whether echocardiography or right heart catheterization is used as the gold standard for diagnosis. The recent guidelines for the diagnosis and treatment of pulmonary hypertension include several potential causes of PH in SLE, including: a primary vasculopathy similar to idiopathic pulmonary arterial hypertension (PAH); left heart diseases; post-thromboembolic disease; hypoxia and fibrosis resulting from interstitial lung disease; and the infrequent SLE-associated pulmonary veno-occlusive disease. The pathogenesis of PAH associated with lupus is yet unclear, but likely includes a role for the genetic background, the presence of antiphospholipid antibodies, and some level of endothelial dysfunction. The evolution of SLE-associated PH is highly variable and difficult to elicit because the published series have used heterogeneous inclusion criteria. Optimal therapeutic management of PAH associated with lupus is unclear because no dedicated randomized controlled trial is yet available. Treatment usually includes arterial pulmonary vasodilators and immunosuppressive agents when the patients have NYHA functional class II, III or IV dyspnea.
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PMID:[Pulmonary arterial hypertension in systemic lupus erythematosus]. 2137 32

Although pulmonary arterial hypertension (PAH) is usually considered as a late complication in limited subsets of systemic sclerosis (SSc), PAH can also occur in diffuse SSc and in early SSc. The evaluation of the velocity of the tricuspid regurgitation on echocardiography is so far the most effective screening tool for pulmonary hypertension (PH) in SSc. Nevertheless, it is not measurable in about 20% of SSc patients ; In that case, pulmonary arterial pressure can be evaluated by using the velocity of a pulmonary insufficiency and indirect signs of PH should be analyzed: pulmonary ejection time, right heart cavities dilatation and aspect of the inferior vena cava. About 10% of SSc patients with a suspected of PAH on echocardiography have post-capillary PH at right heart catheterization mainly due to left ventricular diastolic dysfunction. In some SSc patients, PAH and post-capillary PH due to left ventricular diastolic dysfunction may be associated. This can be suspected in case of post-capillary PH with a transpulmonary gradient > 12 mmHg. Lung fibrosis is another cause of PH in SSc. The distinction between PH associated to lung fibrosis from PAH is sometimes difficult, particularly in patients with a FVC between 60 and 70% of expected. Pulmonary veno-occlusive disease is a possible cause of precapillary PH in SSc. The diagnosis of pulmonary veno-occlusive disease should be discussed in each case of PAH associated ScS due to the high risk of lung edema at the introduction of specific PAH drugs.
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PMID:[Systemic sclerosis associated pulmonary arterial hypertension: the pitfalls]. 2153 80

Recognition of causes of pulmonary hypertension other than congenital heart disease is increasing in children. Diagnosis and treatment of any underlying cause of pulmonary hypertension is crucial for optimal management of pulmonary hypertension. This article discusses the available knowledge regarding several disorders associated with pulmonary hypertension in children: idiopathic pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, hemoglobinopathies, hepatopulmonary syndrome, portopulmonary hypertension and HIV. Three classes of drugs have been extensively studied for the treatment of IPAH in adults: prostanoids (epoprostenol, treprostinil, iloprost, beraprost), endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan), and phosphodiesterase inhibitors (Sildenafil, tadalafil). These medications have been used in treatment of children with pulmonary arterial hypertension, although randomized clinical trial data is lacking. As pulmonary vasodilator therapy in certain diseases may be associated with adverse outcomes, further study of these medications is needed before widespread use is encouraged.
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PMID:Non-congenital heart disease associated pediatric pulmonary arterial hypertension. 2185 94

Pulmonary hypertension is a significant cause of morbidity and mortality. Unfortunately, non-specific presentation and lack of awareness of the disease frequently lead to significant delay in diagnosis, often with the onset of right heart failure, when prognosis is poor and therapy is of limited effectiveness. The classification of pulmonary hypertension is a clinical one grouping diseases into categories with similar patho-physiological mechanism and therapeutic options. Pulmonary biopsy can provide a definitive diagnosis but is hazardous in patients with pulmonary hypertension. Imaging has emerged as an invaluable tool in differentiating the aetiology, assessing disease severity and directing further management. One of the most important roles of imaging is to differentiate diseases resulting from obstruction of the large pulmonary arteries from those secondary to diffuse small vessel disease, as these have very different prognosis and are also treated differently. Small vessel diseases causing pulmonary arterial hypertension most commonly result from diffuse remodelling of the pulmonary arterioles. There are multiple causes of arteriolar remodelling which share similar histopathological, clinical and imaging features. In a subgroup of small vessel diseases causing pulmonary hypertension the predominant site of increased vascular resistance is at the level of the capillaries or venules. Correct diagnosis of pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis is essential since poor prognosis and inadvertent administration of vasodilators (conventional therapy for arteriolar predominant disease) can result in fatal pulmonary oedema. Multimodality imaging plays an important role in suggesting a diagnosis, guiding further investigation and directing treatment.
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PMID:Imaging in pulmonary hypertension, part 3: small vessel diseases. 2226 25

A pulmonary hypertension (PH) registry (Spanish Registry of Pulmonary Arterial Hypertension) was undertaken to analyse prevalence, incidence and survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Spain, and to assess the applicability of recent survival prediction equations. Voluntary reporting of previously diagnosed and incident PAH or CTEPH cases (July 2007-June 2008) was performed. Demographic, functional and haemodynamic variables were evaluated. 866 patients with PAH and 162 with CTEPH were included. PAH associated with toxic oil syndrome and pulmonary veno-occlusive disease were reported for the first time in a PAH registry. Estimated prevalences were as follows: PAH, 16 and CTEPH, 3.2 cases per million adult inhabitants (MAI). Estimated incidences were as follows: PAH, 3.7 and CTEPH, 0.9 cases per MAI per yr. 1-, 3- and 5-yr survival was 87%, 75% and 65%, respectively, with no differences between PAH and CTEPH. Male sex, right atrial pressure and cardiac index were independent predictors of death. Matching between observed survival and that predicted by different equations was closer when the characteristics of the cohorts were similar. Epidemiology and survival of PAH patients in the Spanish registry are similar to recent registries. Characteristics of the population from which survival prediction equations are derived influence their applicability to a different cohort. CTEPH is much less prevalent than PAH, although has a similar survival rate.
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PMID:Survival in pulmonary hypertension in Spain: insights from the Spanish registry. 2236 43

Despite the development of specific therapies for pulmonary arterial hypertension (PAH) some patients fail to respond to such treatment. One of the potential reasons for the unresponsiveness to targeted therapies may be the presence of fibrous occlusion of small pulmonary veins that accompanies pre-capillary arteriopathy. This type of pathologic change is called pulmonary veno-occlusive disease (PVOD). Underdiagnosed PVOD occurs probably in 5-10% of idiopathic pulmonary hypertension (IPAH) and in a substantial proportion of PAH related to connective tissue diseases (mainly in scleroderma). A definite diagnosis of PVOD requires histological examination of lung sample, but surgical lung biopsy in pulmonary hypertension is combined with high risk of bleeding. Thus major interest is focused on a non-invasive diagnostic approach enabling early recognition of PVOD and referral for lung transplantation. The present review is focused on the radiological features suggestive of PVOD-like vasculopathy in PAH.
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PMID:In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scans. 2295 11


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