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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary hypertension is a complex and multidisciplinary disorder. The classification of pulmonary hypertension includes 5 groups. Pulmonary arterial hypertension is a rare disorder that can be idiopathic or heritable in nature, or associated with other conditions, such as scleroderma or congenital heart disease. The recent decades have realized advances in the treatment of this once devastating disease. More commonly, pulmonary hypertension is associated with other disorders, such as those that elevate left heart filling pressures and hypoxemic lung disease. Chronic thromboembolic disease can result in pulmonary hypertension. To determine the etiology, a thorough and methodical evaluation must be completed. Often, an echocardiogram is the first test to suggest the diagnosis of pulmonary hypertension. Studies to identify potential associated causes are important. The diagnostic evaluation culminates in right heart catheterization. Over recent years, advances in therapies, including the prostacyclins, the endothelin receptor antagonists, and the phosphodiesterase type 5 inhibitors, have resulted in an improved quality of life and outlook for patients with what is often a progressive disease.
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PMID:Pulmonary arterial hypertension. 2203 8

Congenital heart disease (CHD) is a common structural defect of the heart or major blood vessels. Patients with adult congenital heart disease (ACHD) have medical needs that are distinct from those of pediatric patients with CHD, and the transition into adult health care is important for management of the patient with ACHD. A large proportion of patients with CHD develop diseases and complications associated with the long-term stress of intracardiac shunts. Pulmonary arterial hypertension (PAH) is a significant complication of some CHD lesions. The treatment of these patients remains challenging due to their combined heart and lung disease, and multidisciplinary care is ofen necessitated for a variety of secondary conditions. A number of treatment options are available for the management of PAH associated with CHD, including prostanoids, phosphodiesterase type-5 inhibitors, and endothelin receptor antagonists. This article discusses the diagnosis and management of such ACHD patients with PAH.
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PMID:Adult congenital heart disease and pulmonary arterial hypertension: the Texas Adult Congenital Heart Program experience. 2210 52

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension (PH) have been adopted for Germany. While the guidelines contain detailed recommendations regarding pulmonary arterial hypertension (PAH), they contain only a relatively short paragraph on other, much more frequent forms of PH including PH owing to left heart disease. The guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors) have not been sufficiently investigated in other forms of PH. However, despite the lack of respective efficacy data an uncritical use of targeted PAH drugs in patients with PH associated with left heart disease is currently observed at an increasing rate. This development is a matter of concern. On the other hand, PH is a frequent problem that is highly relevant for morbidity and mortality in patients with left heart disease. It that sense, the practical implementation of the European Guidelines in Germany requires the consideration of several specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH due to left heart disease. This commentary describes in detail the results and recommendations of the working group which were last updated in October 2011.
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PMID:Pulmonary hypertension due to left heart disease: updated Recommendations of the Cologne Consensus Conference 2011. 2222 72

Sexual activity represents a light physical effort in most subjects with or without heart disease. In a small number of patients with heart disease sexual activity may trigger cardiac symptoms and even induce serious cardiac problems, such as myocardial infarction or sudden cardiac death due to malignant ventricular arrhythmias. However, the global risk remains very low. Thus, it is important to stratify patients with heart disease into risk groups which may help to counsel them to resume sexual activity, to identify those patients at higher risk for cardiac events and to treat those with sexual dysfunction, particularly when using 5-phosphodiesterase inhibitors for erectile dysfunction.
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PMID:[Sexuality and heart disease]. 2250 45

Pulmonary arterial hypertension (PAH) is a disease that leads to characteristic vascular wall remodeling and hemodynamic alterations. Consequently, this pulmonary vascular disease contributes to substantial morbidity and mortality in afflicted patients. PAH may be idiopathic in nature or associated with connective tissue disease, chronic liver disease, human immunodeficiency virus, congenital heart disease, and a growing list of other conditions. There are currently nine Food and Drug Administration-approved therapies for specific PAH treatment. Therapeutic targets include prostacyclin replacement, endothelin-1 antagonism, and phosphodiesterase-5 inhibition. This article focuses on the prostanoid treprostinil and explores its role in the management of patients with PAH.
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PMID:Clinical utility of treprostinil and its overall place in the treatment of pulmonary arterial hypertension. 2287 90

Sildenafil is a selective phosphodiesterase-5 inhibitor and causes vasodilatation, particularly in pulmonary circulation. Since left heart failure may be associated with pulmonary hypertension "out of proportion to left heart disease," sildenafil may have beneficial effect in such patients. The present investigation was designed as a 12-week, single-center, randomized, double-blind, placebo-controlled study evaluating the effects of sildenafil on mean blood pressure (primary endpoint) in patients with left systolic heart failure. Secondary endpoints included exercise capacity assessed by 6-minute walk test. A total of 106 patients were randomized 1:1 to sildenafil (n=53) or placebo (n=53). Patients received sildenafil 25 mg twice a day or matching placebo for the first 2 weeks and 50 mg 3 times a week for the remainder of the trial. The placebo-corrected effect on mean blood pressure was 1.16 mm Hg (95% confidence interval, -1.6 to 5.1, P>.05), demonstrating that sildenafil did not decrease mean blood pressure. Compared with placebo, sildenafil increased the 6-minute walk test by a nonsignificant treatment effect of 14 m (P=.67). Adverse effects occurred in a comparable proportion of patients taking sildenafil and placebo, and none of the patients needed to discontinue therapy. Sildenafil is well tolerated in left heart failure patients and does not decrease blood pressure. It can be safely added to standard heart failure therapy.
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PMID:Is chronic sildenafil therapy safe and clinically beneficial in patients with systolic heart failure? 2324 Oct 3

The association of pulmonary atresia and ventricular septal defect (PA/VSD) can be considered the most severe form of tetralogy of Fallot. The main feature of this congenital heart disease is represented by discontinuity between the right ventricle and pulmonary trunk or its branches; the anatomy of central pulmonary arteries is often abnormal, consequently the type and the amount of sources of pulmonary blood flow are variable. Due to evolution in surgical techniques, definitive correction is now also considered in more complex cases. A small rate of unoperated patients with PA/VSD can survive until adulthood and the arterial blood supply to the lungs, provided by major aorto-pulmonary collateral arteries (MAPCAs), is one of the main determinants of survival. We report two unoperated cases of PA/VSD and MAPCAs with long-term survival. Giant MAPCAs can occasionally be found by chest radiography in adults with unrepaired PA/VSD. Moreover, non-invasive assessment of the pulmonary arterial bed with computer tomography or MRI is helpful in these patients during follow-up. Finally, we discuss the use of oral anticoagulants and/or 5-phosphodiesterase inhibitors in these patients.
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PMID:Giant aorto-pulmonary collaterals in pulmonary atresia and ventricular septal defect: long-term survival in unoperated adults. 2331 39

miRNAs are short regulatory RNAs that can regulate gene expression through interacting with the 3'UTR of target mRNAs. Although the role of miRNAs has been extensively studied in adult human and animal models of heart disease, nothing is known about their expression in pediatric heart failure patients. Different than adults with heart failure, pediatric patients respond well to phosphodiesterase inhibitor (PDEi) treatment, which is safe in the outpatient setting, results in fewer heart failure emergency department visits, fewer cardiac hospital admissions and improved NYHA classification. We have recently shown that pediatric heart failure patients display a unique molecular profile that is different from adults with heart failure. In this study we show for the first time that pediatric heart failure patients display a unique miRNA profile, and that expression of some miRNAs correlate with response to PDEi treatment. Moreover, we show that expression of Smad4, a potential target for PDEi-regulated miRNAs, is normalized in PDEi-treated patients. Since miRNAs may be used as therapy for human heart failure, our results underscore the importance of defining the molecular characteristics of pediatric heart failure patients, so age-appropriate therapy can be designed for this population.
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PMID:miRNA expression in pediatric failing human heart. 2333 38

There has been no established medical therapy to ameliorate pulmonary hypertension (PH) owing to left heart disease (LHD-PH). It has recently been shown that the left ventricular assist device (LVAD) can improve LHD-PH and therefore has the potential to become a major bridge tool for heart transplantation (HTx). However, some patients still have persistent PH even after LVAD treatment. It is essential to demonstrate the reversibility of end-organ dysfunction, including PH, prior to implantable LVAD treatment, especially in Japan, because implantable LVAD treatment is indicated only as bridge to transplantation. Here we report a patient with LHD-PH whose PH was demonstrated to be reversible by the acute pulmonary vasoreactivity test (APVT) with nitrogen monoxide (NO) and the phosphodiesterase-5 inhibitor sildenafil. Both inhaled NO and sildenafil reduced pulmonary vascular resistance, but pulmonary capillary wedge pressure was increased by NO, which was conversely decreased under increased cardiac output by sildenafil. After the patient was listed as an HTx recipient, pulmonary vascular resistance recovered down to an acceptable range with LVAD treatment. Based on these findings, we suggest that the APVT with sildenafil may be a useful and safe tool to predict improvement of PH after LVAD treatment.
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PMID:Acute pulmonary vasoreactivity test with sildenafil or nitric monoxide before left ventricular assist device implantation. 2355 49

World Health Organization (WHO) group 2 pulmonary hypertension (PH) due to left-side heart disease (ie, heart failure or left-sided valvular heart disease) is the most common form of PH in western countries. Distinguishing patients with WHO group 2 PH, particularly the subset of patients with PH due to heart failure with preserved ejection fraction (HFpEF), from those with WHO group 1 pulmonary arterial hypertension (PAH) is challenging. Separating the two conditions is of vital importance because treatment strategies differ completely. Furthermore, therapies that are indicated for WHO group 1 PAH may be harmful in patients with WHO group 2 PH. We review the somewhat confusing PH nomenclature and the WHO classification system and rationale behind it. We then focus on left-side heart disorders that cause PH. An aging population and advances in the medical management of common cardiovascular disorders have caused the prevalence of heart failure to rise significantly, with more than one-half of patients having HFpEF. We review contemporary studies that focus on clinical and echocardiographic findings that help to distinguish HFpEF from PAH in the patient with PH. We discuss the typical, and sometimes atypical, hemodynamic profiles that characterize these two groups, review challenges in the interpretation of data obtained by right-sided heart catheterization, and highlight special maneuvers that may be required for accurate diagnosis. Finally, we review the largely disappointing studies on the use of PAH-specific therapies in patients with WHO group 2 PH, including the use of prostacyclins, endothelin receptor antagonists, and the more promising phosphodiesterase-5 inhibitors.
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PMID:WHO's in second?: A practical review of World Health Organization group 2 pulmonary hypertension. 2449 47


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