Over the past 2 decades, pulmonary arterial hypertension has evolved from a uniformly fatal condition to a chronic, manageable disease in many cases, the result of unparalleled development of new therapies and advances in early diagnosis. However, none of the currently available therapies is curative, so the search for new ...

The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH initially adopted in 1998 during the 2nd ...

OBJECTIVES: Systemic sclerosis-related pulmonary arterial hypertension (SSc PAH) is a major complication of both limited and diffuse systemic sclerosis and leads to substantial morbidity and mortality. Natriuretic peptides (NP) are clinically useful markers of right ventricular dysfunction and pulmonary hypertension. The aim of our review was to examine the evidence ...

The sophisticated and expensive treatment modalities of persistent pulmonary hypertension of the newborn (PPHN), such as nitric oxide, are limited in developing countries. Alternative (less expensive) treatments are being sought and bosentan, an oral dual endothelin-1 receptor antagonist, may be an option for the treatment of PPHN. We report our ...

Pulmonary hypertension is a complication of sickle cell disease that is associated with increased mortality. Whether this complication is associated with hemolysis has been questioned. Systolic pulmonary artery blood pressure can be estimated from echocardiography-determined tricuspid regurgitation velocity (TRV). A velocity of 2.5 m/s or higher suggests possible pulmonary hypertension. ...

Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by progressive obliteration of the pulmonary vasculature, leading to right heart failure and death if left untreated. Prior to the current treatment era, pulmonary hypertension carried a poor prognosis with a high mortality rate, but its prognosis has changed over the ...

Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arterioles, which, in the absence of effective therapy, progresses rapidly to right heart failure and death. Opening of a patent foramen ovale (PFO) is common in patients with severe pulmonary hypertension (PH), resulting in resistive hypoxemia. We report the ...

Despite limited evidence from clinical studies, anticoagulant drugs such as vitamin K antagonists (VKA) (e.g., warfarin or phenprocoumon) are widely used in the background treatment of patients with pulmonary arterial hypertension (PAH). According to current guidelines, they are generally accepted as efficacious drugs, although their efficacy is neither supported by ...

Although pulmonary arterial hypertension is usually associated with advanced stages of sarcoidosis, its occurrence in early stage disease is rare. Herein, a case of associated pulmonary arterial hypertension in the setting of Hashitoxicosis and stage II pulmonary sarcoidosis is reported. The case of associated pulmonary arterial hypertension occurred in a ...

Currently, prostacyclin, endothelin receptor antagonists and phosphodiesterase inhibitors are recommended therapy in idiopathic pulmonary arterial hypertension. However, each of these drugs has limited efficacy in the improvement of clinical symptoms, pulmonary hemodynamics and long-term survival. Levosimendan, a novel calcium-sensitizing inodilator agent, has been shown to have pulmonary vasodilatory action. However, ...

Vasculitis can occur either as a primary condition or secondary to CTDs, infection, medication or malignancy. This article reviews the clinical presentation and management of vascular disease associated with SLE and SS, as well as the primary necrotizing vasculitides. Although pulmonary arterial hypertension (PAH) has traditionally been considered a rare ...

Significant progress has been made in the medical therapy of pulmonary hypertension in recent years, but long term results are still disappointing. We describe the theoretical underpinnings of a surgical procedure which we suggest might prevent or delay the occurrence of right ventricular failure and thereby improve long-term survival in ...

Pulmonary arterial hypertension (PAH) is a severe clinical and pathophysiologic syndrome with no effective treatment at present. Mycophenolate mofetil (MMF), a prodrug of mycophenolic acid (MPA), has been applied widely to the treatment of connective tissue diseases with the effect of immunosuppressant. Its anti-proliferation has been found recently. Thereby, we ...

The case of a 65 years old lady, diabetic, hypertensive, reduced functional class with history of episodic dyspnoea lasting for few weeks for the last two years. On presentation she was tachypnoiec with reduced oxygen saturation. Jugular venous distension was present with bibasal crackles. Her ECG was normal. Echocardiogram revealed, ...

Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder, characterised by the presence of vascular malformations. The pulmonary vascular complications of HHT include pulmonary arteriovenous malformations, pulmonary hypertension associated with high-output heart failure and liver vascular malformations and, finally, pulmonary arterial hypertension secondary to HHT. In the present review, ...

We examined inflammatory mediators in patients with pulmonary hypertension related to congenital systemic-to-pulmonary shunts and the change in these markers during treatment with bosentan. Inflammatory mechanisms probably play a pathogenic role in idiopathic pulmonary arterial hypertension. Their involvement in pulmonary hypertension related to congenital systemic-to-pulmonary shunts is largely unknown. Plasma ...

BACKGROUND: Knowledge of the causative reasons for pulmonary arterial hypertension, a major category of pulmonary hypertension, has expanded dramatically over the past 10 years. This has led to heightened research across a range of potential new mechanistic approaches and resulted in the identification of further treatment options, together with several ...

OBJECTIVE: In order to provide an overview of current knowledge, the literature was systematically examined for clinical studies, which evaluate the safety and effectiveness of bosentan in pediatric pulmonary arterial hypertension (PAH). SOURCES: 3 databases (MEDLINE, EMBASE and BIOSIS) were searched for the period January 2000 - October 2007 using ...

(1) In patients with mildly symptomatic pulmonary hypertension (stage III), there is no firm evidence that ambrisentan reduces mortality or slows disease progression. In contrast, ambrisentan has frequent and sometimes serious adverse effects; (2) In patients whose physical activity is markedly restricted by pulmonary hypertension, it is better to continue ...

We describe a 54-year-old woman with isolated pulmonary arterial hypertension accompanied by hyperthyroidism due to Graves' disease. Her pulmonary artery hypertension resolved spontaneously after restoration of euthyroidism. This case suggests that hyperthyroidism should be considered a reversible cause of pulmonary arterial hypertension.

The serotonin hypothesis of pulmonary arterial hypertension (PAH) arose owing to anorexigens, acting as indirect serotinergic agonists, causing PAH. However, it is now thought that serotonin plays an important role in the pathobiology of PAH per se. The rate-limiting enzyme in the synthesis of peripheral serotonin is tryptophan hydroxylase 1 ...

BACKGROUND: Schistosomiasis is a highly prevalent disease with >200 million infected people. Pulmonary hypertension is one of the pulmonary manifestations in this disease, particularly in its hepatosplenic presentation. The aim of this study was to determine the prevalence of pulmonary hypertension in schistosomiasis patients with the hepatosplenic form of the ...

It is known that essential hypertension may be implicated in the development of cognitive impairment that is associated to microvascular disease of the brain. It has been hypothesized that increased arterial stiffness of the large arteries may lead to microvascular changes due to increased pulsatile flow. Our study tests the ...

Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening condition in which organised thrombi obstruct the pulmonary vessels, causing increased pulmonary vascular resistance, progressive pulmonary hypertension (PH) and right heart failure. The treatment of choice is pulmonary endarterectomy, which restores pulmonary haemodynamics with acceptable periprocedural mortality rates in the majority of ...

PURPOSE: To investigate, through an immunohistochemical method, whether there is deposition of plasma proteins in the wall of lenticulostriate, cortical and leptomeningeal arteries of hypertensive patients, with and without lipohyalinosis. METHOD: Forty patients with essential hypertension were selected at random, 20 with lipohyalinosis in the lenticulostriate arteries (HH group) and ...

Among the most commonly used therapies to treat pulmonary arterial hypertension (PAH) are the prostanoids. Epoprostenol, a relatively effective therapy for PAH, has its limitations, which are largely attributable to its pharmacokinetic properties and its requirement for intravenous administration. The development of an equally effective oral prostanoid would be an ...

The prognosis for patients with pulmonary hypertension remains poor despite recent treatment advances, and there is a need for therapies with new modes of action. Nitric oxide (NO) is an endogenous vasodilator, the levels of which are regulated throughout the lung to ensure preferential perfusion of well-ventilated regions. Drugs that ...

Between the April and July of 2007, patients undergoing treatment for schistosomal liver fibrosis, at a university hospital in north-eastern Brazil, were examined by transthoracic Doppler echocardiography (TTE). The main aim was to determine the prevalence of pulmonary hypertension in the patients. The thorax of each patient who had such ...

Pulmonary hypertension is defined by a mean pulmonary artery pressure of greater than 25 mmHg at rest or 30 mmHg with exercise. It can occur in association with a variety of medical conditions. The most serious elevation in pulmonary artery pressures are seen in a group of conditions that share ...

INTRODUCTION: The true incidence of chronic thromboembolic pulmonary hypertension (CTPH) remains a matter of debate. Symptomatic CTPH is probably more common than previously reported, whereas the occurrence of asymptomatic CTPH has not been defined since very limited evidence on the incidence of asymptomatic CTPH diagnosed with echocardiography Doppler are currently ...

BACKGROUND: Despite the development of targeted therapies for pulmonary arterial hypertension (PAH), some patients fail to respond to medical therapy. There are a number of types of PAH, one of which is pulmonary venoocclusive disease (PVOD). Unlike other PAH types, the hallmark pathology of PVOD is fibrous occlusion of the ...

Pulmonary hypertension is an important complication of COPD. A small subset of patients with COPD have severe pulmonary hypertension (PH) that is out of proportion to the mild increase in pulmonary arterial pressure observed commonly. Severe PH associated with COPD is associated with increased morbidity and mortality. Treatment options in ...

Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. Effective management requires timely recognition and accurate diagnosis of the disorder and appropriate selection among therapeutic alternatives. Despite progress in treatment, obstacles remain that impede the ...

The pathogenesis of pulmonary arterial hypertension (PAH) is complex, involving multiple modulating genes and environmental factors. Multifactorial impairment of the physiologic balance can lead to vasoconstriction, vascular smooth muscle cell and endothelial cell proliferation/fibrosis, inflammation, remodeling and in-situ thrombosis. These are the likely mechanisms that lead to narrowing of the ...

Sitaxentan, a highly-selective endothelin receptor antagonist (ETRA) and bosentan a non-selective ETRA are both approved for the treatment of idiopathic pulmonary arterial hypertension (iPAH). Sildenafil is a phosphodiesterase-5 (PDE-5) inhibitor used in the treatment of iPAH. Tadalafil is a long acting PDE-5 inhibitor largely unexplored for the treatment of iPAH. ...

Pulmonary arterial hypertension (PAH) has evolved from an untreatable condition to a disease for which several classes of drugs have now been approved, including various prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors. Because the pathogenesis of pulmonary hypertension is increasingly understood, various new substances are now under clinical investigation, including ...

Pulmonary arterial hypertension is a rare disorder in childhood, the two most common types being idiopathic pulmonary arterial hypertension and pulmonary hypertension associated with congenital left-to-right shunt lesions, together accounting for almost 90% of cases. The clinical presentation of idiopathic pulmonary arterial hypertension (familial and non familial) is essentially non-specific ...

Pulmonary arterial hypertension (PAH) is a devastating disease characterized by progressive elevation of pulmonary arterial pressure and vascular resistance due to pulmonary vasoconstriction and vessel remodeling as well as inflammation. Rho-kinases (ROCKs) are one of the best-described effectors of the small G-protein RhoA, and ROCKs are involved in a variety ...

Studies on the role of connexins (Cxs) in the pathogenesis of right ventricular (RV) hypertrophy in pulmonary arterial hypertension (PAH) have not been reported to date. Therefore, we established a rat model of PAH induced by monocrotaline (MCT), and they were randomized to three groups: Control, MCT, and MCT+bosentan. Through ...

Left vocal cord palsy is a well-recognised rare complication of SLE. We encountered a gentleman who during an acute flare developed left vocal fold palsy (VCP). Immunosuppression was increased, but this only produced partial improvement. VCP is commonly but not exclusively caused by recurrent laryngeal nerve palsy. Other causes may ...

OBJECTIVE: Heart rate-corrected QT interval (QTc) and QTc dispersion (QTcd) are increased and associated with ventricular arrhythmia and an increase in sudden death in a variety of diseases. This study aimed to examine QTc and QTcd in pulmonary hypertension and assess their relationship with pulmonary arterial pressure. PATIENTS AND METHODS: ...

Pulmonary arterial hypertension is a complication of systemic lupus erythematosus. Mortality in pregnant patients with pulmonary arterial hypertension related to connective tissue disease is as high as 56%. The authors report the first case of a successful maternal-fetal outcome in a pregnant patient with systemic lupus erythematosus-associated pulmonary arterial hypertension ...

Endothelin receptor antagonism has emerged as an important therapeutic approach in pulmonary arterial hypertension (PAH). Bench to bedside scientific research has clearly shown that endothelin-1 (ET-1) is over-expressed in several forms of pulmonary vascular disease and plays an important pathogenetic role in the development and progression of PAH. Oral endothelin ...

Pulmonary arterial hypertension (PAH) afflicts thousands of children worldwide. The pathophysiology involves intravascular proliferation and remodeling leading to an increase in pulmonary vascular resistance which if left untreated results in right heart failure and death. Signs and symptoms are subtle as the disease progresses to irreversible lung damage. There is ...

Since the Food and Drug Administration (FDA) approved the use of buprenorphine hydrochloride (Subutex) for the treatment of opiate dependence in 2002, there has been a global trend of its IV abuse which led to life-threatening medical complications such as infective endocarditis (IE), cardiac failure, and death. First episode IE ...

Fibromuscular dysplasia (FMD) is predominantly a disease of younger women, but it can occur and cause refractory hypertension in the elderly. We present here classic angiographic and intravascular ultrasound images of FMD in a 70-year-old woman with refractory hypertension. Renal artery FMD should be included in the differential diagnosis of ...

Ambrisentan is an endothelin receptor antagonist (ERA) that was recently approved for treatment of pulmonary arterial hypertension (PAH). Endothelin (ET) is a potent vasoconstrictor with mitogenic, hypertrophic and pro-inflammatory properties that is upregulated in pulmonary hypertensive diseases. The biologic effects of ET are mediated by 2 cell surface receptors termed ...

Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon consequence of acute pulmonary embolism. We report CTEPH in a 58-year-old male who had pleurisy with a small haemorrhagic pleural effusion three months ago. The six-month course of anti-coagulation therapy failed to resolve thromboemboli completely or improve pulmonary hypertension. Computed tomographic pulmonary ...

This review highlights a number of nitric oxide (NO)-related mechanisms that contribute to coronary vascular function and that are likely affected by hypertension and thus become important clinically as potential considerations in prevention, diagnosis, and treatment of coronary complications of hypertension. Coronary vascular resistance is elevated in hypertension in part ...

Major advances have been made in the understanding and treatment of pulmonary hypertension in the last few years. Without treatment (medication) for idiopathic pulmonary arterial hypertension, which is a rare and potentially fatal condition, the survival time is only about 3 years after diagnosis. However, if pulmonary hypertension is secondary ...