BACKGROUND: Reports have linked anorexigen intake to an increased risk of pulmonary arterial hypertension (PAH). With the rise in anorexigen use in the latter half of the last decade, we established a surveillance network within the United States to monitor temporal trends in the number of reported cases of PAH. ...

Persistent pulmonary hypertension of the newborn (PPHN) remains an important cause of mortality and morbidity in the term neonate. Preliminary but limited data suggest that there may be a role for sildenafil in the treatment of PPHN. We report the successful treatment of PPHN caused by pulmonary hypoplasia in a ...

Pulmonary arterial hypertension is a life-threatening, rare disease characterised by vasoconstriction and vascular remodelling of pulmonary artery vessels. Pulmonary arterial hypertension can occur without an obvious cause or can be secondary. Until several years ago, therapeutic approaches were represented mainly by 'conventional therapy' (anticoagulants, calcium channel blockers, diuretics and digoxin, ...

Portopulmonary hypertension (PPHT) is a rare but devastating complication in patients with portal hypertension, characterized by pulmonary arterial obliterative disease with a concomitant rise in pulmonary vascular resistance. A broad body of evidence has accumulated, indicating that endothelin (ET) peptides and their cognate receptors are causally involved in the pathophysiology ...

We describe a case of pulmonary hypertension and high-output heart failure in a 61-year-old woman suffering from relapsing Graves disease. The patient experienced prompt hemodynamic and symptomatic recovery after normal thyroid function was restored. Possible mechanisms for the development of pulmonary arterial hypertension due to hyperthyroidism include damage to pulmonary ...

A number of controversies exist regarding the pathophysiology, natural history, diagnosis, and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). Although CTEPH is regarded by many to be a complication of pulmonary embolism (PE) arising subsequent to venous thromboembolism (VTE)-the embolic hypothesis-it has been suggested that PE is rarely the sole ...

BACKGROUND: Children with pulmonary hypertension secondary to large left-to-right, post-tricuspid valve shunts can eventually have severe and/or irreversible pulmonary vascular disease, yielding them inoperable for conventional surgery. It has been shown, however, that unloading of the pulmonary hypertension can result in remodeling of the pulmonary vasculature and, thus, improvement of ...

Pulmonary arterial hypertension (PAH) is found in 2-20% of cirrhosis patients who have portal hypertension (portopulmonary hypertension, PPHT). Endothelin (ET), a potent vasoconstrictor, is likely to play a role in the pathogenesis of portal hypertension. We describe the long-term successful use of the dual ET(A)/ET(B) receptor antagonist bosentan in a ...

Pulmonary arterial hypertension is a recognized clinical component of systemic autoimmune diseases, especially systemic sclerosis. Mutations in the bone morphogenetic protein receptor 2 gene reported in sporadic and familial primary pulmonary arterial hypertension have failed to be detected in patients with either scleroderma spectrum disease or underlying connective tissue diseases. ...

Pulmonary arterial hypertension (PAH) is characterized by abnormal proliferation of smooth muscle cells (SMCs), leading to occlusion of pulmonary arterioles, right ventricular (RV) hypertrophy, and death. We investigated whether mycophenolate mofetil (MMF), a potent immunosuppresssant, prevents the development of monocrotaline (MCT)-induced PAH in rats. MMF effectively decreased RV systolic pressure ...

Proliferation of pulmonary artery smooth muscle cells (PASMCs) appears to play a significant role in chronic pulmonary hypertension. The proliferation of PASMCs is strongly inhibited by some commercial heparin preparations. Heparin fragments were prepared by periodate treatment, followed by sodium borohydride reduction, to enhance potency. The tributylammonium salt of this ...

Pulmonary hypertension represents a significant disease burden in both the developed and developing worlds. Certain forms of pulmonary hypertension are more common in some countries than others but people of all races, all ages and both sexes are affected. Treatment options are limited and expensive. The development of new therapies ...

Chronic thromboembolic pulmonary hypertension is a commonly overlooked cause of physical incapacity and dyspnoea, with a higher incidence than is generally appreciated and a poor prognosis. The diagnosis can be suspected based on echocardiographic examinations and ventilation perfusion scanning of the lung. Pulmonary angiography still remains the gold standard for ...

The role of thrombotic arteriopathy in the pathophysiology of idiopathic pulmonary arterial hypertension (IPAH) and the use of anticoagulants in the treatment of IPAH are currently controversial issues. This article reviews the evidence for a role of vascular thrombosis in the pathophysiology of IPAH. There is sufficient biological rationale to ...

Pulmonary hypertension can occur as an isolated disease affecting the lung vessels only, in association with underlying hypoxic lung disorders, or due to chronic thromboembolic disease. Pulmonary hypertension caused by pulmonary venous congestion will not be focused on in this review. Regardless of the underlying disease, chronic cor pulmonale is ...

BACKGROUND: In non-thromboembolic pulmonary hypertension, endothelin (ET)-1 levels are increased and correlate with the hemodynamic severity of the disease. Whether such correlations exist in chronic thromboembolic pulmonary hypertension (CTEPH) is unknown, nor whether ET-1 levels correlate with hemodynamic outcome after pulmonary endarterectomy (PEA). METHODS AND RESULTS: ET-1 levels were determined ...

BACKGROUND: Pulse wave velocity (PWV), a relevant indicator of arterial stiffness, can be measured noninvasively with a variety of automatic devices, but most are complexly equipped. We developed a novel index for estimating arterial stiffness as "QPV interval," which was determined by means of surface electrocardiogram and Doppler ultrasound of ...

Idiopathic pulmonary arterial hypertension is a disease involving small muscular pulmonary arteries and arterioles. Treatment with prostacyclin analogs and endothelin receptor antagonists is the cornerstone of therapy in these patients. Recent evidence suggests that phosphodiesterase-5 inhibitors such as sildenafil may improve functional capacity and hemodynamics in patients with pulmonary arterial ...

Pulmonary manifestations of adult-onset Still's disease (AOSD) include aseptic pneumonitis, pleural effusions, rarely acute respiratory distress syndrome, and restrictive lung disease. Pulmonary arterial hypertension (PAH) occurs with several rheumatologic diseases, however, has only been reported once in AOSD. We describe a 29-year-old woman with a 9-year history of AOSD, who ...

In recent years, the process of the programmed cell death has gained much interest because it has important pathophysiological consequences contributing to the deletion of unwanted cells in the vessel wall, loss of pulmonary smooth muscle cells and therefore in reversing the pulmonary pressure. For the reason that most patients ...

The objective of this study was to use tissue Doppler parameters to identify pulmonary embolism (PE) in patients with echocardiographic signs of pulmonary hypertension. One hundred fifty patients with echocardiographic signs of pulmonary hypertension were enrolled, 50 of whom had PE on multidetector row computed tomography of the chest. Another ...

Pulmonary arteriopathy (PA) is the pathologic hallmark in human medicine of diffuse constrictive (medial and intimal remodeling) or multifocal complex (plexiform and dilatative lesions) arterial lesions, or both, that lead to irreversible obliteration of the arterial lumen. Clinically, PA leads to pulmonary arterial hypertension (PAH), of which idiopathic (IPAH) is ...

In recent years, many authors have described several cases revealing an association between hyperthyroidism and pulmonary hypertension. This observational study was designed to evaluate the prevalence of pulmonary hypertension in hyperthyroidism and to determine the role of methimazole in regulating pulmonary vascular resistance in these patients. We studied 114 patients, ...

Angiotensin-converting enzyme (ACE) inhibitors are viewed by many as important treatment options for both the management of hypertension and the provision of end-organ protection. ACE inhibitors have not been looked on as having any clearly recognizable direct pulmonary benefits; rather, their pulmonary effects have been felt to be a nuisance ...

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension, regarded by some as distinct entities. However, their presentations are similar and both are associated with poor prognoses. We therefore reviewed 38 specimens [autopsies (n=15), surgical biopsies (n=15), explants (n=7), and pneumonectomy (1 case)] from ...

In patients with sickle cell disease, anemia is a recognized risk factor for stroke, death, and the development of pulmonary hypertension. We have proposed that hemolytic anemia results in endothelial dysfunction and vascular instability and can ultimately lead to a proliferative vasculopathy leading to pulmonary hypertension. Consistent with this mechanism ...

Two-dimensional strain echocardiography is a new method for the assessment of regional contractility. Thirty-seven patients with pulmonary arterial hypertension (mean age 56.4 +/- 11 years) and 38 normal subjects (mean age 58.3 +/- 12 years) underwent 2-dimensional echocardiography and tissue Doppler echocardiographic evaluation of right ventricular (RV) global function and ...

BACKGROUND: The POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal immunoglobulin, Skin changes) syndrome is a rare disease that entails a specific risk for pulmonary arterial hypertension. METHODS AND RESULTS: We report on 2 patients who suffered from POEMS syndrome and pulmonary arterial hypertension for whom a detailed hemodynamic pulmonary study was obtained ...

Pulmonary hypertension is a common complication of beta-thalassemia major. We report a case of successful treatment of pulmonary hypertension in a patient with beta-thalassemia major and review the literature on pulmonary hypertension and beta-thalassemia major. A 28-year-old man with beta-thalassemia major, splenectomy, hepatitis C, and hemosiderosis who presented with increasing ...

PURPOSE OF REVIEW: The prevalence of pulmonary arterial hypertension is rising worldwide. Significant progress in our understanding of the pathobiology of pulmonary arterial hypertension has resulted in a shift from vasodilator therapy to the development of specific drugs targeting seminal molecular derangements of this disorder. This review highlights the recent ...

Adrenomedullin (ADM) is a potent vasodilator peptide that was originally isolated from human pheochromocytoma. Its vasodilatory effect is mediated by cyclic adenosine 3',5'-monophosphate- and nitric oxide-dependent mechanisms. Earlier studies have demonstrated that ADM is secreted from various tissues, including vessels, heart, and lungs. In addition, there are specific receptors for ...

Chronic thromboembolic pulmonary hypertension may occur in the context of incomplete lysis of acute pulmonary emboli, resulting in the obstruction of pulmonary blood flow, as well as progressive right ventricular dysfunction and failure. The treatment of choice for this condition is surgical removal of the obstructing material. However, in many ...

RATIONALE: HIV-infected patients with pulmonary arterial hypertension have histologic manifestations that are indistinguishable from those found in patients with idiopathic pulmonary arterial hypertension. In addition, the role of pleiotropic viral proteins in the development of plexiform lesions in HIV-related pulmonary hypertension (HRPH) has not been explored. Simian immunodeficiency virus (SIV) ...

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 ...

Angiotensin II-forming chymase is expressed in the pulmonary arteries of the monocrotaline-induced pulmonary hypertensive rats, but its actual role is unclear. We studied chymase-dependent angiotensin II formation in the pulmonary arteries of the monocrotaline-induced pulmonary hypertensive rats and observed the effects of an angiotensin II receptor blocker on vascular remodeling. ...

We report a case of severe pulmonary hypertension associated with sarcoidosis with a unique histopathological presentation. This 43-year old obese patient first presented eight years ago with complaint of dyspnea on exertion for four years and was diagnosed as primary pulmonary hypertension. Six years later, a skin biopsy performed on ...

Pulmonary hypertension is a devastating disorder, characterized by vascular proliferation, intimal hypertrophy and vasoconstriction. In this disorder, alterations in the nitric oxide pathway have borne out to be important in not only vascular proliferation, but also in the maintenance of vascular tone. After synthesis by soluble guanylate cyclase, cGMP effects ...

OBJECTIVE: To evaluate the prevalence of pulmonary hypertension associated with severe acute respiratory distress syndrome (ARDS) and to asses the value of pulmonary artery trunk diameter (PAT) to predict pulmonary hypertension. DESIGN: Prospective study SETTING: University teaching hospital and ARDS referral center. PATIENTS: 103 patients with ARDS, who received both ...

The treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA). However, many patients develop a severe progressive small vessel pulmonary arteriopathy that is inaccessible to surgical intervention and is associated with poor survival. The purpose of the present study was to evaluate the medium-term efficacy and ...

The pathogenesis of pulmonary hypertension remains incompletely understood. Many factors have been implicated; however, there has been great interest in the potent pulmonary vasoconstrictor serotonin (5-HT) due to episodes of primary pulmonary hypertension in humans triggered by serotoninergic appetite-suppressant drugs. Pulmonary hypertensive patients have elevated blood 5-HT levels and pulmonary ...

The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17-mo-old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic-range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. Diagnosis of classic ...

BACKGROUND: Patients with pulmonary arterial hypertension are believed to experience severe postoperative complications after major surgery. METHODS: We retrospectively analyzed the data of 21 patients with pulmonary arterial hypertension who underwent 28 surgical procedures at our institution between 1996 and 2002. RESULTS: The average mean pulmonary arterial pressure was 53 ...

Systolic pulmonary arterial hypertension (PAH) was diagnosed in a 15-year-old intact male Yorkshire terrier presented for progressive dyspnoea and coughing. Several examinations were performed (thoracic radiographs, faecal analysis, heartworm antigen test, tracheal fluoroscopy, abdominal ultrasound, complete blood cell count, urine and serum biochemistry) but the PAH remained of unknown origin. ...

The compression of the left main coronary artery (LMCA) secondary to pulmonary artery trunk dilatation is a relatively new entity that has been associated with severe pulmonary hypertension. It is associated with acyanogen congenital cardiopathies or idiopathic pulmonary arterial hypertension. The natural history is unknown and its treatment has been ...

CASE DESCRIPTION: A 13-year-old llama was examined because of lethargy, inappetence, and syncope. CLINICAL FINDINGS: Physical examination revealed muffled heart and lung sounds and peripheral edema. Clinicopathologic abnormalities included lymphopenia, hyperglycemia, prerenal azotemia, mild hyponatremia, mild hypoalbuminemia, and high gamma-glutamyltransferase and creatine kinase activities. On ultrasonography, the liver appeared hyperechoic ...

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by the presence of telangiectases and arteriovenous malformations. In some families in whom a form of idiopathic pulmonary arterial hypertension cosegregated with HHT, mutations in the ACVRL1 gene were present. PURPOSE: We noninvasively measured the pulmonary artery systolic pressure ...

Severe thromboembolic pulmonary hypertension is an infrequent but potentially fatal complication of effort thrombosis (Paget-Schroetter syndrome) of the upper extremity. It has been sporadically reported in the literature, but in most cases has affected young, otherwise healthy athletes. Because this syndrome has been rarely described, it is likely underdiagnosed, and ...

Pulmonary arterial hypertension is a disease characterized by progressive obliteration of the pulmonary vasculature leading to right-ventricular failure and if untreated, death. Several effective therapies are now available for pulmonary arterial hypertension. These therapies target specific abnormalities in the endothelium, including prostacyclin and nitric oxide deficiencies, and endothelin excess. Sildenafil, ...

Sildenafil attenuates acute pulmonary embolism (APE)-induced pulmonary hypertension. However, the hemodynamic effects of sildenafil in combination with other vasodilators during APE have not been examined yet. In the present study, we examined the hemodynamic effects of combined diethylenetriamine/nonoate (DETA-NO, 1microMol kg(-1), i.v.) and sildenafil (0.25mg/kg, i.v.) in an anesthetized dog ...

We report a case of a patient with myelofibrosis with myeloid metaplasia (MMM) who presented with progressive dyspnea of unexplained origin. Splenomegaly, blood smear, and bone marrow findings allowed diagnosis of MMM. High-resolution CT chest scan revealed diffuse septal thickening, while echocardiography and electrocardiogram showed no indirect evidence of pulmonary ...