Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic pulmonary arterial hypertension is a rare and potentially fatal condition. Without treatment, survival is only approximately 2.8 years from diagnosis. However, if the pulmonary hypertension is secondary to other causes, especially to congenital heart disease, it is possible to survive for 30 years or more without treatment. In recent years, remarkable progress has been made, risk factors have been identified and improved imaging techniques, including echocardiography, computer tomography and magnetic resonance imaging, are available. The condition can affect children at any age from fetal life through to adulthood. Patients can present to the respiratory pediatrician with unresponsive asthma, to the neurologist with faints or to the general pediatrician with failure to thrive. Over the last few years there have been significant developments in the available therapy for managing this complicated disease, which have improved the prognostic outlook, such as oral bosentan and sildenafil, intravenous epoprostenol and interventional catheterization with atrial septostomy. This article reviews the current knowledge about causation, investigation and treatment of children with pulmonary hypertension in the clinical setting.
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PMID:Management and therapeutic options in pediatric pulmonary hypertension. 1671 97

Idiopathic pulmonary arterial hypertension (IPAH) is an incurable disease of multifactorial origin. Inflammation is frequently observed in IPAH, but its role in the pathobiology is unclear. In this study the distribution, nature and number of inflammatory cells in periarterial infiltrates in lungs of children with IPAH, pulmonary arterial hypertension associated with congenital heart disease (APAH) and in normal lung tissue were characterised and compared using immunohistochemistry The influence of treatment with combined prostacyclin and endothelin receptor blockers was also studied. In children with IPAH, both treated and untreated, but not in children with APAH or normal children, extensive periarterial infiltrates were present comprising macrophages and T lymphocytes with S100A4- and bone morphogenetic protein receptor type-2 (BMPR2)-positive cells. Although rarely co-expressing macrophage-specific antigens, BMPR2-positive cells were frequently closely associated with macrophages and lymphocytes. They were more abundant around peripheral arteries of children with IPAH than in APAH or normal lungs (15.1 (3.5), 2.3 (0.9) and 2.3 (0.9) cells/mm external elastic lamina, respectively; p<0.01 for IPAH vs APAH or normal lungs). Prostacyclin with endothelin receptor blockade resulted in a significant reduction in endothelial cell activation as indicated by human leucocyte antigen (HLA)-DR expression (treated 17% vs untreated 100%, p<0.002). This study shows that pulmonary inflammation is present in the lungs of children with IPAH. This may indicate a role for inflammation in the pathobiology of IPAH and provide the rationale for novel therapeutic intervention.
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PMID:Contribution of inflammation to the pathology of idiopathic pulmonary arterial hypertension in children. 1952 66

Liver cirrhosis is a complex and progressive disease associated with high mortality. In developing countries, alcoholic liver disease is the most common form of liver cirrhosis, followed by chronic viral disease, especially hepatitis C virus infection. Cirrhosis is associated with systemic and splanchnic hemodynamic abnormalities, including increased vascular volume, decreased systemic vascular resistance, and increased cardiac output. At the splanchnic vascular bed, increases in portal flow and intrahepatic resistance have been described, inducing portal hypertension. Pulmonary arterial hypertension is a progressive disease of pulmonary circulation, without left ventricle and valvular heart disease; it is closely related with structural changes in pulmonary arteries. Idiopathic pulmonary arterial hypertension is related to abnormalities in cellular signals, inducing arterial hypertrophy and increased vascular tone. Porto-pulmonary hypertension includes simultaneous portal and pulmonary arterial hypertension. To confirm disease, it is important to exclude concomitant heart disease. Porto-pulmonary hypertension requires important components: portal hypertension, shear vascular stress, and cellular activation with pulmonary arterial hypertrophy. In this short manuscript, the epidemiology, patho-physiology, and diagnostic criteria of the disease are reviewed to optimize early diagnosis and treatment.
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PMID:[Epidemiology, physiopathology and diagnosis of pulmonary hypertension in hepatic (PH) cirrhosis]. 2262 15

Pulmonary hypertension is defined as 25mmHg or higher mean pulmonary arterial pressure measured by right heart catheterization. Miscellaneous diseases may cause PH. Among these are pulmonary arterial hypertension (PAH), left heart disease, pulmonary disorders and hypoemia, and chronic thromboembolic disease. Pulmonary hypertension (PH) is seen frequently in the elderly. Although diseases resulting in systolic and diastolic dysfunction with accompanying pulmonary venous hypertension (PVH) account for a large percentage of PH in this population, PH associated with other respiratory diseases, such as chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), and sleep apnea can also be seen. Idiopathic pulmonary arterial hypertension (IPAH) is, although rarely, seen and requires careful exclusion in older patients. PAH associated with connective tissue disease is the most encountered form of PAH. Since the treatment to all these disease is different, differential diagnosis has the utmost importance.
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PMID:[Pulmonary hypertension in the elderly]. 2897 95

Heart-lung transplantation (HLTx) is currently the best treatment for patients who have end-stage heart and lung failure. Idiopathic pulmonary arterial hypertension (IPAH) was the main indication for HLTx in the 1980s. However, when studies showed resolution of right ventricular dysfunction after double-lung transplantation (DLTx), this last procedure became the preferred option for end-stage IPAH. Currently, the main indication of HLTx is congenital heart disease (CHD), followed by acquired heart disease combined with pulmonary hypertension and/or intrinsic lung disease. Although early posttransplant survival remains lower after HLTx than after lung transplantation, careful patient selection combined with surgical advances are producing improvements. Here, we review the practice patterns, trends, and outcomes of HLTx worldwide.
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PMID:Heart-lung transplantation: current indications, prognosis and specific considerations. 3050 5