BACKGROUND: An increased prevalence of splenectomy has been reported in patients with idiopathic pulmonary arterial hypertension. Examination of small pulmonary arteries from these subjects has revealed multiple thrombotic lesions, suggesting that thrombosis may contribute to this condition. Based on these findings, we hypothesised that splenectomy could be a risk factor ...

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 ...

Surgical and interventional therapies for pulmonary arterial hypertension (PAH) in appropriately selected patients have the potential to dramatically improve or, in some cases, cure PAH. These include atrial septostomy, a palliative procedure or bridge to transplantation in patients with refractory right heart failure, pulmonary thromboendarterectomy for pulmonary hypertension associated with ...

This work represents the first application of a statistical mechanics based microstructural orthotropic hyperelastic model to pulmonary artery mechanics under normotensive and hypertensive conditions. The model provides an analogy between the entangled network of long molecular chains and the structural protein framework seen in the medial layer, and relates the ...

The pathobiology of pulmonary arterial hypertension (PAH) reflects a multifactorial process and complex evolution that involves dysfunction of underlying cellular pathways and mediators. Among these, the endothelin system has been shown to be important in the pathogenesis of PAH. Endothelin-1 (ET-1), which is found in high levels in PAH, is ...

Idiopathic pulmonary arterial hypertension (formerly primary pulmonary hypertension) can affect more than one member of the same family. In the past 25 years scientists have exposed the inheritance pattern and a major element of the molecular basis for inherited pulmonary arterial hypertension. Familial pulmonary arterial hypertension is inherited as an ...

Truly reversible pulmonary hypertension is rare. Acquired systemic arteriovenous (A-V) fistulas following spinal surgery (laminectomy) are a less recognized cause of secondary pulmonary hypertension. We describe a patient who presented with symptoms and clinical evidence of pulmonary hypertension and underwent endovascular correction of an acquired A-V fistula, which led to ...

Circumferential pericardial effusion typically results in biventricular tamponade and equalization of intracardiac and pericardial pressure during diastole. However, tamponade may involve the right or left ventricle. While isolated left ventricular cardiac tamponade (LVCT) can occur as a postoperative complication from localized posterior pericardial effusions, circumferential pericardial effusions leading to LVCT ...

The development of pulmonary hypertension is a poor prognostic sign in patients with chronic obstructive pulmonary disease (COPD), affecting both mortality and quality of life. Although pulmonary hypertension in COPD is traditionally viewed as a result of emphysematous destruction of the vascular bed and/or hypoxia, recent studies indicate that neither ...

The modern era in cardiopulmonary medicine began in the 1940s, when Cournand and Richards pioneered right-heart catheterization. Until that time, no direct measurement of central vascular pressure had been performed in humans. Right-heart catheterization ignited an explosion of insights into function and dysfunction of the pulmonary circulation, cardiac performance, ventilation-perfusion ...

We describe a newborn girl with incontinentia pigmenti (IP, MIM308300), unilateral acheiria, and fatal primary pulmonary hypertension. Limb deficiency has not been described previously in IP and pulmonary hypertension only on two previous occasions. A review of the cause of IP shows that these rare manifestations may not be unexpected, ...

Phosphodiesterase-5 (PDE5) inhibitors and other agents that modulate intracellular cGMP are now emerging as promising, safe, and easy to administer therapies for pulmonary hypertension, with relatively few side effects. Recent studies have shown that PDE5 inhibitors are potent acute pulmonary vasodilators in experimental models that partially reverse established pulmonary arterial ...

Hyperplasia of pulmonary artery SMCs (PASMCs) is a pathological hallmark of pulmonary arterial hypertension (PAH). In this issue of the JCI, McMurtry et al. report that adenovirus-mediated overexpression of survivin--a multipotent inhibitor of apoptosis--induces PAH in rats, whereas inhalation of an adenovirus vector encoding a mutant survivin gene with dominant-negative ...

PURPOSE OF REVIEW: Until recently, the diagnosis of idiopathic pulmonary arterial hypertension was virtually a death sentence, particularly for children. Although there is no cure for idiopathic pulmonary arterial hypertension, recent medical advances have dramatically changed the course of this disease in children. A review of some of the latest ...

A Chiari malformation Type I may remain asymptomatic until the patient has reached adulthood and acute presentation of symptoms occurs. In several clinical and experimental studies it has been shown that essential hypertension is associated with vascular compression of the brainstem, particularly of the rostral ventrolateral medulla oblongata. Nevertheless, two ...

Pulmonary hypertension is a devastating complication of various, but rare diseases and can also occur as an isolated entity. It causes morbidity and mortality in all patients. Ongoing research has provided some insight into the pathophysiology and clinical manifestations, and new therapeutic options have recently become available for some types ...

Nitroglycerin and dipyridamole are two commonly available and well tolerated vasoactive medications. Their acute hemodynamic effects in patients with pulmonary arterial hypertension are not well defined in the current literature. The authors retrospectively analyzed the acute hemodynamic effects of IV nitroglycerin, dipyridamole, and epoprostenol in 59 patients with pulmonary arterial ...

Pulmonary hypertension (PH) is a predictor of poor outcome in sarcoidosis. Little is known about the epidemiology of PH in sarcoidosis. The current authors reviewed the records of patients with sarcoidosis listed for lung transplantation in the USA between January 1995 and December 2002. PH was defined as a mean ...

Heart failure (HF) and pulmonary hypertension (PH) coexist frequently and contribute to each other. Because PH often is an insidious disease, the cornerstone of management is the early identification and treatment of its underlying causes, such as left-sided heart disease, left to right shunts, and pulmonary disorders. A practical clinical ...

BACKGROUND: N-terminal pro-Brain Natriuretic Peptide (NT-proBNP) values correlate with mild-moderate pulmonary hypertension assessed by tricuspid insufficiency (TI) gradient>or=30 mm Hg in Gaucher disease. The purpose of this study is to ascertain improved risk stratification based on correlation with NT-proBNP and C-reactive protein (CRP), a standard marker of inflammation. METHODS: Patients ...

Cl- channels have been implicated in essential cellular functions including volume regulation, progression of cell cycle, cell proliferation and contraction, but the physiological functions of the ClC-3 channel are controversial. We tested the hypothesis that the ClC-3 gene (ClCn-3) is upregulated in hypertensive pulmonary arteries of monocrotaline-treated rats, and upregulated ...

Pulmonary hypertension is a common complication of sickle cell disease (SCD). In spite of the mild elevations in pulmonary artery pressures in these patients, the associated morbidity and mortality is high. In fact, in adult patients with SCD, pulmonary hypertension is emerging as the major independent risk factor for death. ...

Given the nonspecific nature of its early symptoms and signs, pulmonary arterial hypertension (PAH) is often diagnosed in its advanced stages. Although clinical assessment is essential when initially evaluating patients with suspected PAH, echocardiography is a key screening tool in the diagnostic algorithm. It not only provides an estimate of ...

Pulmonary arterial hypertension (PAH) results from persistent vasoconstriction, smooth muscle growth and extracellular matrix (ECM) remodelling of pulmonary arteries (PAs). Matrix metalloproteinases (MMPs) are matrix-degrading enzymes involved in ECM turnover, and in smooth muscle cell (SMC) and endothelial cell migration and proliferation. MMP expression and activity are increased in experimental ...

OBJECTIVE: To provide a description and thorough discussion of the diagnostic considerations for a rare case of malignant hypertension and aortic aneurysm in a pediatric patient. DESIGN: Case report. SETTING: A university pediatric intensive care unit. SUBJECT: A young child with a hypertensive crisis and a thoracoabdominal false aortic aneurysm. ...

Intravascular lymphoma (IVL) is a rare and usually fatal disease that belongs to the class of high-grade malignant lymphomas and which is characterized by proliferation of neoplastic lymphoid cells exclusively within the lumina of small blood vessels. Its polymorphic and nonspecific clinical manifestations make antemortem diagnosis very difficult. We report ...

Pulmonary arterial hypertension (PAH) is characterized by the progressive increase in pulmonary vascular resistance potentially leading to right heart failure and death. Since endothelins may play a pathogenic role in the development of the disease, endothelin receptor antagonists have been proposed for the treatment of this condition. Bosentan (Tracleer), an ...

Although pulmonary hypertension secondary to upper airway obstruction caused by adenotonsillar hyperplasia has been well described, the association between laryngeal papillomatosis and pulmonary hypertension has not previously been documented. We report three patients with pulmonary hypertension due to upper airway obstruction caused by laryngeal papillomatosis. Pulmonary hypertension can contribute to ...

OBJECTIVE: To evaluate the efficacy of sildenafil for treatment of pulmonary hypertension. DATA SOURCES: Literature retrieval was accessed through MEDLINE (1977-March 2005), Cochrane Library, and International Pharmaceutical Abstracts (1977-March 2005) using the terms sildenafil and pulmonary hypertension. In addition, reference citations from publications identified were reviewed. STUDY SELECTION AND DATA ...

Asymmetric dimethylarginine (ADMA) is an endogenous inhibitor of nitric oxide synthase (NOS) and has been implicated in endothelial dysfunction. ADMA is metabolized by the enzyme dimethylarginine dimethylaminohydrolase (DDAH), with DDAH2 representing the predominant endothelial DDAH isoform. Symmetric dimethylarginine (SDMA), also originating from arginine methylation by protein arginine methyltransferases, is an ...

Pulmonary hypertension complicates the course of many newborns with congenital diaphragmatic hernia. In the most severe cases, the fetal condition of markedly elevated pulmonary vascular resistance persists after birth and is associated with hypoxemic respiratory failure and severe disturbances in cardiac performance. Late pulmonary hypertension (weeks to months after birth) ...

Although sildenafil, an oral phosphodiesterase type-5 inhibitor, may offer benefits in the pharmacological management of pulmonary hypertension (PH), safety and effectiveness have not been studied during coadministration with beraprost, an oral prostacyclin analogue. To address this issue, we administered oral beraprost (40 microg) on day 1 and beraprost (40 microg) ...

This is a report of the successful angioplastic treatment of an association of renovascular hypertension with renal artery stenosis and the Goldenhar syndrome (a variant of oculoauriculovertebral dysplasia). For the first time to date, this association, which occurred in a 13-year-old girl, is reported. Additionally, increased arterial distensibility in spite ...

We report a case of a patient with anti PL-12 antibody accompanied by interstitial pneumonia and severe pulmonary hypertension. At first presentation, hyperkeratotic skin lesions were found, although the diagnosis of CVD was not conclusive. Lung histology showed diffuse fibrosing interstitial pneumonia predominantly in the subpleural regions. During the seven-year ...

Idiopathic infantile arterial calcification is a rare and usually fatal disorder, which is characterized by widespread vaso-occlusive disease. Presentation is variable, but refractory hypertension is typical, with symptoms of cardiorespiratory failure. Some present in utero with evidence of fetal compromise, which may manifest as hydrops fetalis and premature delivery or ...

Four cases of sudden unexpected death associated with pulmonary hypertension are described. In all cases, the presence of pulmonary hypertension had not been clinically detected. In each case, the underlying etiology of the pulmonary hypertension was different with one case each of occult adult congenital heart disease, pulmonary sarcoidosis, pulmonary ...

OBJECTIVE: To describe a successful transition process from subcutaneous treprostinil to intravenous epoprostenol after the failure of treprostinil in a patient with idiopathic pulmonary arterial hypertension and present an algorithm to achieve the conversion without significant adverse reactions. CASE SUMMARY: A 25-year-old white female receiving subcutaneous treprostinil 97 ng/kg/min was ...

This article focuses on pulmonary arterial hypertension, including both primary pulmonary hypertension (PPH) and those forms of pulmonary arterial hypertension that are related to other factors, including collagen vascular diseases, congenital shunts, portal hypertension, human immunodeficiency viral infection, and exposure to specific drugs and toxins. Risks for different types of ...

Pulmonary hypertension is one of the leading causes of death in adult sickle cell patients, with a prevalence of 20% to 40%. Although these patients have lower pulmonary pressures than patients with primary pulmonary hypertension, both groups suffer high 2-year mortality rates. Pulmonary hypertension may go undetected until the disease ...

Pulmonary hypertension (PAH) is defined as mean pulmonary artery pressure greater than 25 mm Hg at rest, or greater than 30 mm Hg with exercise. While there are a myriad of etiologies for PAH, many times a cardiac etiology may be present. This is particularly important from both diagnostic and ...

Pulmonary arterial hypertension is a severe disease with poor prognosis, caused by obliteration of the pulmonary vasculature as a result of pulmonary-vascular remodeling, active vasoconstriction and in situ thrombosis. Left untreated, pulmonary arterial hypertension results in right-ventricular failure and death. There has been dramatic progress in the treatment of pulmonary ...

Previous studies demonstrated that bacterial lipopolysaccharide (LPS, endotoxin) triggers pulmonary vasoconstriction leading to pulmonary hypertension (PHS, ascites) in broilers. The lungs of broilers are constantly challenged with LPS that can trigger pulmonary vasoconstriction. Among broilers from a single genetic line, some individuals respond to LPS with large increases in pulmonary ...

BACKGROUND: To find chest roentgenographic (CXR) features to help differentiate two representative diseases with severe chronic pulmonary hypertension (PH). STUDY SUBJECTS: Thirty-six consecutive patients with chronic thromboembolic PH (CTEPH), 38 with primary PH (PPH), and 37 with left heart disease and PH. METHODS: CXRs were reviewed about 6 features (left ...

Idiopathic pulmonary arterial hypertension is a rare but serious and life-threatening disease that leads to right heart failure and death within 2.8 years without specific treatment. This review focuses on the stable prostacyclin analog iloprost, its biologic action and pharmacology and, finally, on its clinical development, efficacy and safety in ...

Patients with idiopathic pulmonary arterial hypertension usually undergo acute vasodilator tests with nitric oxide (NO) for haemodynamic evaluation and therapeutical planning. The aim of this study was to evaluate the link between the variation of N-terminal (NT)-pro-brain natriuretic peptide (BNP) levels and haemodynamic parameters during the acute vasodilator test. A ...

PURPOSE: To compare retrospectively the frequency of systemic collateral supply in patients who have chronic thromboembolic pulmonary hypertension with the frequency of systemic collateral supply in patients who have primary pulmonary hypertension by using multi-detector row helical computed tomographic (CT) angiography. MATERIALS AND METHODS: For this review, neither institutional board ...

BACKGROUND: Retinoic acid has antimitogenic effects on smooth muscle cells. Studies on the systemic circulation suggest that it may reduce vascular thickening. Relationships between retinoids and pulmonary hypertension/pulmonary vascular remodeling, however, have not been explored. Thus, the present study examined retinoid levels in plasma of patients with idiopathic pulmonary arterial ...

Homocysteine may contribute to systolic hypertension and cardiac events by decreasing conduit artery compliance and inducing endothelial dysfunction. The effects of the experimental induction of hyperhomocysteinemia on systemic arterial compliance and pulsewave velocity are unclear, with contradictory results from previous studies. The investigators tested whether oral methionine impairs brachial artery ...

Extract: Pulmonary hypertension (PH) was previously termed primary (idiopathic or of unknown origin, i.e., spontaneous) or secondary (as a result of another disorder) pulmonary hypertension. It is now clear, however, that many of the entities labeled as secondary pulmonary hypertension resemble primary pulmonary hypertension in both their histopathological features and ...

PURPOSE OF REVIEW: Options for the surgical treatment of chronic thromboembolic pulmonary hypertension are either lung transplantation or pulmonary endarterectomy. Pulmonary endarterectomy is considered permanently curative and the treatment of choice. The procedure dramatically improves functional status and provides an excellent immediate and long-term survival, much better than transplantation. Pulmonary ...