Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary arterial hypertension is a malignant disease with a median survival of 3 years. Uric acid levels are elevated in severe heart failure and in states of hypoxemia. Early data suggest a correlation between hyperuricemia and severe pulmonary arterial hypertension. We studied 29 patients with pulmonary arterial hypertension diagnosed and treated between 1998 and 2001. Clinical characteristics (6 min walk test and New York Heart Association class) and hemodynamic parameters (pulmonary artery pressure, pulmonary vascular resistance and cardiac output) were evaluated and correlated to uric acid level in a retrospective study. Uric acid levels correlated positively with New York Heart Association class (r=0.66, P<0.001) and negatively with 6 min walk test (r=-0.35, P=0.03). Uric acid levels were higher in patients who died than in patients who survived at the end ofthe follow-up period (8.8 vs. 5.7 mg/dl, P=0.001). This study shows that uric acid levels are elevated in severe pulmonary arterial hypertension and can be used as a prognostic marker of disease severity.
 ...
PMID:Hyperuricemia as a prognostic factor in pulmonary arterial hypertension. 1258 62

Pulmonary arterial hypertension is a life-threatening disorder that refers to a group of diseases characterized by an abnormal elevation of the blood pressure within the pulmonary circulation due to a vasculopathy of the pulmonary microcirculation (1). If left untreated, the overall prognosis of pulmonary arterial hypertension is poor, with a 5-year survival rate of 34%. The most common cause of death is progressive right-sided heart failure (2). There is no pharmacologic cure for pulmonary arterial hypertension, and a team approach is required for the proper care of these patients (3). Treatment is directed at improving clinical symptoms, increasing exercise tolerance and extending survival. Until just a few years ago, standard treatment options for the treatment of pulmonary arterial hypertension were limited and included coumarin derivatives, calcium channel blockers, diuretics, digoxin and oxygen supplementation (4). In the last decade, the therapeutic options for pulmonary arterial hypertension have made considerable advances. In at least three major randomized controlled trials, the continuous intravenous infusion of epoprostenol, a synthetic salt of prostacyclin with potent vasodilatory ability, has been shown to improve exercise tolerance, hemodynamics, survival and quality of life in patients with New York Heart Association (NYHA) functional classes III and IV with either primary pulmonary hypertension or pulmonary arterial hypertension associated with scleroderma (5-9). Epoprostenol has now become a mainstay therapy in the long-term treatment of primary pulmonary hypertension or pulmonary arterial hypertension associated with scleroderma. Nevertheless, epoprostenol is far from an ideal form of therapy. Issues such as the short half-life of the drug, need for continuous central intravenous access and significant side effects have led to the development of more stable prostacyclin analogues as alternative therapies for primary pulmonary hypertension. These alternative therapies include iloprost (inhaled delivery) and treprostinil (subcutaneous delivery). As a result, the Food and Drug Administration (FDA) recently approved treprostinil for the treatment of pulmonary arterial hypertension in patients with NYHA classes II-IV. This review will offer a discussion of the basic pharmacology of treprostinil, its similarities to and differences from epoprostenol, the animal studies as well as the initial investigational studies leading to its FDA approval, and clinical uses of the drug alone or in combination with newer therapies directed at pulmonary arterial hypertension developed over the last decade.
 ...
PMID:Overview of treprostinil sodium for the treatment of pulmonary arterial hypertension. 1514 31

Pulmonary arterial hypertension (PAH) is a rare and rapidly progressing disease characterized by an increase in pulmonary resistance, which, if untreated, inevitably progresses to right ventricular heart failure and death. New treatment options were developed in the last 10 years. Endothelin receptor antagonists (ERAs) and prostanoids are an effective treatment with a better survival. Phosphodiesterase inhibitors (PDIs) are of a great interest, and first results in the treatment of patients are promising. PDIs are different in pulmonary selectivity and half-life.Right heart catheterization is the "gold standard" in the diagnosis of pulmonary hypertension. Direct classification of the pulmonary hypertension is feasible, and the effects of the drugs are directly measurable. The results of right heart catheterization are comparable for inhalative and oral drugs. New generations of drugs resulted in special testing protocols. Furthermore, individual concepts of therapy can be developed.
 ...
PMID:[Clinical and pharmacological testing of patients with pulmonary hypertension for treatment decision]. 1596 5

The availability of selective inhibitors of the cyclic guanosine monophosphate (cGMP)-specific type 5 phosphodiesterase (PDE5) has created increasing interest in unlocking the therapeutic potential of PDE5 inhibition in cardiovascular diseases that are marked by dysfunction of nitric oxide (NO)-cGMP signaling. Pulmonary arterial hypertension (PAH) and heart failure (HF) are characterized by pulmonary arterial vasoconstriction that is thought to be caused by relative deficiencies of vasodilators such as NO and exaggerated production of vasoconstrictors such as endothelin. PDE5 is abundant in the pulmonary vasculature where it catabolizes cGMP, the second messenger of NO. Inhibition of PDE5 has been shown to lower pulmonary vascular resistance in PAH and HF by augmenting local cGMP. This review outlines the therapeutic potential of PDE5 inhibition for the treatment of PAH and HF.
 ...
PMID:Type 5 phosphodiesterase inhibition in heart failure and pulmonary hypertension. 1603 43

The thalassemias are common monogenic disorders of hemoglobin synthesis. beta-thalassemias are the most important among the thalassemia syndromes and have become a worldwide clinical problem due to an increasing immigrant population. In beta-thalassemia major, regular blood transfusions are necessary early in life. Beta-thalassemia intermedia refers to a less severe phenotype, whereas beta-thalassemia/hemoglobin E disease encompasses a broad phenotypic spectrum. Blood transfusions and increased gastrointestinal iron absorption result in iron overload and tissue damage. Among patients with beta-thalassemia major, biventricular, dilated cardiomyopathy remains the leading cause of mortality. In some patients, a restrictive type of left ventricular cardiomyopathy or pulmonary hypertension is noted. The clinical course, although variable and occasionally fulminant, is more benign in recent than in older series. Myocarditis has been described as a cause of left-sided heart failure in younger patients. Pulmonary arterial hypertension is the principal cause of heart failure in beta-thalassemia intermedia. Chelation therapy has improved prognosis in beta-thalassemia major both by reducing the incidence of heart failure and by reversing cardiomyopathy. Estimation of the patient's cardiac risk is mainly based on clinical criteria and serial echocardiography. A new cardiovascular magnetic resonance technique will probably fulfill the need for more precise risk stratification in beta-thalassemia syndromes. By increasing the proportion of patients on optimal chelation, survival in beta-thalassemia major may further improve. Recent advances in gene therapy are expected to result in the long-awaited cure of this disease.
 ...
PMID:Heart failure in beta-thalassemia syndromes: a decade of progress. 1616 78

Pulmonary arterial hypertension (PAH) is a clinical condition characterized by elevated vascular resistance, associated with a poor prognosis and usually diagnosed in late stage. Echocardiographic assessment of PAH includes early disease detection and functional heart evaluation, in order to introduce a more accurate surveillance at an early stage of the disease and to contribute to prognostic stratification of advanced disease. Detection involves pulmonary artery systolic pressure (PASP) estimation. There is no clear consensus on defining normal distribution, but a PASP of 36 mmHg has been widely assumed as a cut-off value for mild PAH, requiring a more aggressive surveillance to detect further progression. Functional heart evaluation requires an accurate characterization of morphologic and hemodynamic changes, secondary to PAH development, which involves description of dimensional parameters, ventricular interdependency, intracardiac flow patterns, and right ventricular systolic performance. A valid assessment of these issues results in a useful evaluation of cardiac function, supporting clinical context in defining heart failure condition.
 ...
PMID:Pulmonary hypertension: echocardiographic assessment. 1627 Apr 77

Pulmonary arterial hypertension is a serious and severe complication of liver cirrhosis and portal hypertension. We present a case of a 47 year old female who developed pulmonary hypertension and right ventricular heart failure symptoms 6 years from the diagnosis of liver biliary cirrhosis and portal hypertension.
 ...
PMID:[Pulmonary arterial hypertension complicating portal hypertension and liver biliary cirrhosis--a case report]. 1650 74

Pulmonary arterial hypertension represents an absolute contraindication for heart transplantation. We report the case of a 30-year-old man with end-stage heart failure due to restrictive cardiomyopathy and pulmonary arterial hypertension. A complex multidrug therapy improved pulmonary haemodynamics to the point that orthotopic heart transplantation could be carried out. At 18-month follow-up after heart transplantation, the patient's cardiac function made a full recovery. Larger prospective studies are warranted to support these results.
 ...
PMID:Complex multidrug therapy in a patient with pulmonary hypertension before and after orthotopic heart transplantation. A case report. 1790 84

Pulmonary arterial hypertension (PAH) is a progressive disease, with right-heart failure being the main cause of death. In patients refractory to conventional drug therapy, atrial septostomy can serve as palliative treatment or as a bridge to transplantation. A 41-year-old woman with a 15-year history of PAH associated with a corrected atrial septal defect presented with severe deterioration of symptoms. Echocardiography confirmed reocclusion of an atrial septal stoma that had been created several months before. After performing a repeat atrial septostomy, we implanted a custom-made atrial septostomy device, an Amplatzer septal occluder that had been fenestrated to serve as a custom-made atrial septostomy device. This resulted in an improvement in cardiac output and a marked symptomatic relief. During the 6-year follow-up, the patient was clinically stable with limited but constant exercise tolerance, under specific medical therapy. Repeated echocardiography confirmed long-term patency of the device.
 ...
PMID:Long-term follow-up of a fenestrated Amplatzer atrial septal occluder in pulmonary arterial hypertension. 1818 54

Pulmonary arterial hypertension (PAH) is a severely disabling disorder characterized by sustained elevations of pulmonary vascular resistance, ultimately leading to right-heart failure and death. Intravenous epoprostenol has been widely used in patients with PAH, leading to long-term clinical benefits and improved survival. Epoprostenol has to be delivered through a permanently implanted intravenous catheter, with the potential of life-threatening complications. Thus, treprostinil, a stable prostacyclin analog suitable for subcutaneous administration, has been developed. Treprostinil is chemically stable at room temperature, has a long half-life (2-4 hours), and has similar pharmacologic properties with comparable hemodynamic effects as epoprostenol.A large, double-blind, placebo-controlled, multicenter, 12-week study confirmed the efficacy of subcutaneous treprostinil, by improving exercise capacity, Borg Dypnea Score (BDS), New York Heart Association (NYHA) class, and clinical signs and symptoms in patients with PAH. Subsequently, multiple observational studies reported the long-term effects of subcutaneous treprostinil. The long-term survival of patients treated with subcutaneous treprostinil was similar to that reported with intravenous epoprostenol. Pain at the infusion site has been a major drawback of subcutaneous treprostinil, hampering dose titration and leading to an 8-10% discontinuation rate. In addition, studies have examined the efficacy of intravenous treprostinil in the treatment of patients with PAH. An open-label study demonstrated that intravenous treprostinil improved exercise capacity, BDS, NYHA functional class, and hemodynamics at week 12 compared with baseline. Transitioning from intravenous epoprostenol to intravenous treprostinil is safe and effective. The dose of intravenous treprostinil has to be adjusted to approximately twice the dose of intravenous epoprostenol. Most patients have reported less severe adverse effects with intravenous treprostinil compared with intravenous epoprostenol. The assessment of the long-term efficacy and safety of continuous intravenous treprostinil requires further studies.
 ...
PMID:The role of treprostinil in the management of pulmonary hypertension. 1869 Jul 55


1 2 3 4 5 6 7 8 Next >>