Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recent reports have suggested an association between primary pulmonary hypertension and human immunodeficiency virus (HIV) infection. This appears to be an accelerated syndrome, associated with a relatively brief duration of symptoms, yet prominent right ventricular failure and severe pulmonary hypertension on presentation. We present a case of a primary pulmonary hypertension in a 35-year-old HIV-seropositive hemophiliac. His accelerated clinical course is consistent with previously reported cases of HIV-related pulmonary hypertension. However, this patient's pulmonary function tests revealed marked hyperinflation, a decreased diffusing capacity, and no airflow obstruction. To our knowledge, this very usual constellation of pulmonary function changes has not been described previously in this syndrome.
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PMID:Marked pulmonary function abnormalities in a case of HIV-associated pulmonary hypertension. 832 1

Pulmonary arterial hypertension (PAH) is a progressive arteriopathy of the pulmonary circulation that can affect a wide group of patients. Presentation and natural history of PAH are intimately linked to progressive right ventricular failure. Historically, survival in PAH has been poor, especially for patients with PAH associated with progressive systemic sclerosis and human immunodeficiency virus. Oral, subcutaneous, and intravenous therapies that have emerged during the last decade can improve symptoms and hemodynamics. This review explores the impact of these therapies on prognosis in PAH, which ultimately rests on the ability to reverse the pulmonary arteriopathy and preserve right ventricular function.
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PMID:Changing the prognosis of pulmonary arterial hypertension: impact of medical therapy. 1612 18

Human immunodeficiency virus-related cardiomyopathy is characterized by global left ventricular (LV) dysfunction commonly associated with biventricular dilation. Human immunodeficiency virus (HIV) cardiomyopathy carries a poor prognosis, and the role of antiretroviral therapy in the reversal of heart failure is not very clear. We report two patients with HIV infection who presented with severe right ventricular (RV) dysfunction in the absence of pulmonary parenchymal, pulmonary arterial and left ventricular myocardial involvement. During the period of intensive antiretroviral therapy, the symptoms of right heart failure progressively and remarkably improved. This was accompanied by normalization of right ventricular size and RV function documented by repeat echocardiograms. Given that the serologic tests for opportunistic infections were negative, and the RV function improvement correlated with a decrement in the viral load, it is likely that the cardiomyopathy was due to direct infection by HIV. These cases illustrate that there can be isolated involvement of the right heart in the absence of lung, significant pulmonary vascular and left ventricular disease, and also that the antiretroviral therapy might reverse the cardiomyopathy.
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PMID:Reversible right ventricular dysfunction in patients with HIV infection. 1655 85

Pulmonary hypertension (PH), defined as a mean pulmonary arterial (PA) pressure of >25 mmHg at rest or >30 mmHg during exercise, is characterized by a progressive and sustained increase in pulmonary vascular resistance that eventually leads to right ventricular failure. Clinically, PH may result from a variety of underlying diseases (Table 1 and Refs. 50, 113, 124). Pulmonary arterial hypertension (PAH) may be familial (FPAH) or sporadic (idiopathic, IPAH), formerly known as primary pulmonary hypertension, i.e., for which there is no demonstrable cause. More often, PAH is due to a variety of identifiable diseases including scleroderma and other collagen disorders, liver disease, human immunodeficiency virus, and the intake of appetite-suppressant drugs such as phentermine and fenfluramine (72). Other, more common, causes of PAH include left ventricular failure (perhaps the most common cause), valvular lesions, chronic pulmonary diseases, sleep-disordered breathing, and prolonged residence at high altitude. This classification, now widely accepted, was first proposed at a meeting in Evian, France, in 1998, and modified in Venice, Italy, in 2003 (124).
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PMID:Mediators and modulators of pulmonary arterial hypertension. 1669 50

Modern health care has greatly increased longevity for patients with congenital hemolytic anemias (such as sickle cell disease and thalassemia) and human immunodeficiency virus (HIV) infection. It is estimated that 10% of patients with hemoglobinopathies and 0.5% of patients with HIV infection develop moderate to severe pulmonary hypertension. Pulmonary hypertension is a relentlessly progressive disease leading to right heart failure and death. Worldwide, there are an estimated 30 million patients with sickle cell disease or thalassemia and 40 million patients with HIV disease. Considering the prevalence of pulmonary vascular disease in these populations, sickle cell disease and HIV disease may be the most common causes of pulmonary hypertension worldwide. In this review, the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies for these diseases are summarized. Because therapy is likely to reduce morbidity and prolong survival, efforts to screen, diagnose, and treat these patients represent a global health opportunity.
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PMID:Pulmonary hypertension: an increasingly recognized complication of hereditary hemolytic anemias and HIV infection. 1846 Jun 61

Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure (PAPm) superior than 25mmHg at rest or superior than 30mmHg with exercise. The classification of PH differentiates between "secondary" PH which results from a well-known disease, such as PH due to thromboembolic disease (obstructive PH), left cardiac failure (passive PH), or chronic respiratory diseases (hypoxic PH), and pulmonary arterial hypertension (PAH). PAH is a rare disease characterized by a progressive increase of pulmonary vascular resistance leading to right ventricular failure. PAH is classified as idiopathic, familial, or associated with various conditions (connective tissue diseases, congenital heart diseases with systemic-to-pulmonary shunts, portal hypertension, infection with the human immunodeficiency virus, or appetite-suppressant drugs). Transthoracic Doppler echocardiography is the investigation of choice for non invasive detection of PAH but right-heart catheterization is necessary to confirm the diagnosis of PAH and determine its mechanism. Pulmonary function tests and chest CT scan may detect an underlying chronic pulmonary disease (hypoxic PH). Lung perfusion scan and contrast-enhanced chest spiral CT scan can lead to the diagnosis of thromboembolic PH, which is to be confirmed by pulmonary angiography. Assessment of the severity of PH is based on clinical parameters (NYHA, right heart failure), functional tests (six-minute walk test), echocardiography and hemodynamics. Characterization of PH is essential in the management of PH because it determines the appropriate treatment: an etiological treatment in passive, obstructive or hypoxemic PH, or vasodilatator and antiproliferative therapies in PAH.
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PMID:[Investigation of pulmonary hypertension]. 1865 91

Pulmonary arterial hypertension (PAH), which can be a complication of human immunodeficiency virus (HIV) infection, is characterized by increased pulmonary arterial pressure and peripheral vascular resistance, subsequently leading to right heart failure. In HIV-infected patients, the management of PAH is challenging given the potential drug interactions between PAH-specific vasodilators and antiretroviral drugs. We describe a 51-year-old female with acquired immunodeficiency syndrome (AIDS) and HIV-associated PAH. She was treated with the oral endothelin receptor antagonist bosentan while taking a nevirapine (a nonnucleoside reverse transcriptase inhibitor)-based antiretroviral regimen. Due to concerns about potential drug interactions with the antiretroviral therapy, her nevirapine plasma concentration, as well as CD4(+) cell count and viral load, were continuously monitored. We observed no interaction between bosentan and nevirapine during a 4-year period. To our knowledge, this report is the first to demonstrate successful, long-term coadministration of bosentan and a nonnucleoside reverse transcriptase inhibitor.
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PMID:Successful bosentan and nonnucleoside reverse transcriptase inhibitor-based therapy in a patient with acquired immunodeficiency syndrome and pulmonary arterial hypertension. 2033 65

Cardiac myxomas are benign tumors of endocardial origin. Symptoms might mimic heart disease as well as infectious disease, immunodeficiency, and malignant processes. We present the case of a 59-year-old female patient with a large right ventricular myxoma that was connected to the basal interventricular septum. Movement in systole provoked the obstruction of the right ventricular outflow tract. She presented clinically with syncope, systolic murmur, and signs of right heart failure. The diagnosis of the right ventricular tumor was based on 2D echocardiography, magnetic resonance, and multislice computed tomography findings.Emergency surgical excision of the myxoma was necessary to prevent embolism and sudden death.
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PMID:Right ventricular myxoma obstructing the outflow tract. 2192 78

The health effects of exposure to household air pollution are gaining international attention. While the bulk of the known mortality estimates due to these exposures are derived from respiratory conditions, there is growing evidence of adverse cardiovascular health effects. Pulmonary hypertension and right heart failure are common conditions in low- and middle-income countries whose etiology may be related to common exposures in these regions such as schistosomiasis, human immunodeficiency virus, tuberculosis infections and other causes. While little is known of the interplay between exposure to household air pollution, right heart function and such conditions, the large burden of pulmonary hypertension and right heart failure in regions where there is significant exposure to household air pollution raises the possibility of a linkage. This review is presented in three parts. First, we explore what is known about pulmonary hypertension and right heart failure in low- and middle-income countries by focusing on eight common causes thereof. We then review what is known of the impact of household air pollution on pulmonary hypertension and posit that when individuals with one of these eight common comorbidities are exposed to household air pollution they may be predisposed to develop pulmonary hypertension or right heart failure. Lastly, we posit that there may be a direct link between exposure to household air pollution and right heart failure independent of pre-existing conditions which merits further investigation. Our overall aim is to highlight the multifactorial nature of these complex relationships and offer avenues for research in this expanding field of study.
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PMID:Waiting to inhale: An exploratory review of conditions that may predispose to pulmonary hypertension and right heart failure in persons exposed to household air pollution in low- and middle-income countries. 2368 34

Antiretroviral therapy has greatly increased longevity for individuals with human immunodeficiency virus (HIV) infection. About 0.5% of patients with HIV infection develop moderate to severe pulmonary arterial hypertension, which is several thousand times higher than the incidence of idiopathic pulmonary arterial hypertension. As more than 30 million individuals are chronically infected, HIV infection could soon become one of the most common causes of pulmonary arterial hypertension worldwide. Pulmonary arterial hypertension is a relentlessly progressive disease leading to right heart failure and death. In this article the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies for HIV-associated pulmonary arterial hypertension are reviewed.
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PMID:Human immunodeficiency virus-associated pulmonary arterial hypertension. 2370 77


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