Nitric oxide is an important regulator of vascular tone in the pulmonary circulation. Surgical correction of congenital heart disease limits pulmonary hypertension to a brief period. The study has measured expression of endothelial (eNOS), inducible (iNOS), and neuronal nitric oxide synthase (nNOS) in the lungs from biopsies of infants with ...

BACKGROUND: In idiopathic pulmonary arterial hypertension (IPAH), peripheral airway obstruction is frequent. This is partially attributed to the mediator dysbalance, particularly an excess of endothelin-1 (ET-1), to increased pulmonary vascular and airway tonus and to local inflammation. Bosentan (ET-1 receptor antagonist) improves pulmonary hemodynamics, exercise limitation, and disease severity in ...

INTRODUCTION: Increasing evidence indicates that epidural anesthesia improves postoperative pulmonary function. The underlying mechanisms, however, remain to be determined. Because pulmonary nitric oxide has been identified to play a critical role in pulmonary dysfunction in sepsis, we hypothesized that thoracic epidural anesthesia (TEA) modulates endothelial dysfunction via a nitric oxide-dependent ...

Pulmonary arterial hypertension is defined as a group of diseases characterised by a progressive increase in pulmonary vascular load, leading to marked increase in pulmonary artery pressure, right ventricular failure and premature death. Given the nonspecific nature of its early symptoms and signs, pulmonary arterial hypertension (PAH) is often diagnosed ...

Pulmonary hypertension is a rare condition and in combination with pregnancy, it can result in high maternal mortality. Mitral stenosis is one of the complicated cardiac diseases that may occur during pregnancy. In this report, we describe our management of such a case, which was even more difficult in combination ...

This study was carried out to determine the vasodilator response of inhaled nitric oxide in patients with pulmonary hypertension (PH) after valve replacement surgery for mitral valve disease. This Quesi experimental study was carried out at the intensive care unit of Armed Forces Institute of Cardiology (AFIC) in collaboration with ...

Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by an elevated pulmonary arterial pressure and vascular resistance with a poor prognosis. Various pulmonary and extrapulmonary causes are now recognized to exist separately from the idiopathic form of pulmonary hypertension. An imbalance in the presence of vasoconstrictors and vasodilators plays ...

BACKGROUND: Sedation with alpha2-agonists in the horse is reported to be accompanied by impairment of arterial oxygenation. The present study was undertaken to investigate pulmonary gas exchange using the Multiple Inert Gas Elimination Technique (MIGET), during sedation with the alpha2-agonist detomidine alone and in combination with the opioid butorphanol. METHODS: ...

Pulmonary arterial hypertension (PAH) is a rare fatal disease defined as a sustained elevation of pulmonary arterial pressure to more than 25 mmHg at rest, with a mean pulmonary-capillary wedge pressure and left ventricular enddiastolic pressure of less than 15 mmHg at rest. Histopathology of PAH is founded on structural ...

BACKGROUND: This study aims to present a novel method for using cardiovascular magnetic resonance (CMR) to non-invasively quantify the variation in pulmonary blood volume throughout the cardiac cycle in humans. METHODS: 10 healthy volunteers (7 males, 3 female, age range 21-32 years) were studied. The blood flow in the pulmonary ...

BACKGROUND: Pulmonary endarterectomy (PEA) is an effective treatment for chronic thromboembolic pulmonary hypertension (CTEPH). The present study tested the hypothesis that inflammation, as determined by circulating cytokine levels, may contribute to the difficulty in controlling arterial blood pressure after PEA. MATERIALS AND METHODS: Thirty-six patients with CTEPH (22 males and ...

Long-standing pulmonary hypertension causes significant peripheral and proximal arterial remodeling and right ventricular dysfunction. The clinical metric most often used to assess the progression of PH is the pulmonary vascular resistance (PVR). However, even when measured from multipoint pressure-flow curves, PVR provides information only on the peripheral arterial function, not ...

Bosentan (Tracleer) is an orally administered dual endothelin-1 (ET-1) receptor antagonist approved for use in patients with WHO class II (mildly symptomatic) pulmonary arterial hypertension (PAH). Oral bosentan therapy was beneficial and generally well tolerated in patients with mildly symptomatic PAH. In a well designed, placebo-controlled trial in adolescents and ...

Pulmonary hypertension is characterized by a progressive increase in pulmonary arterial pressure in association with dilatation and hypertrophy of the right ventricle, causing gradual reduction in ejection fraction. The increase in mean pulmonary arterial pressure may be passive, due to increased downstream pressure, hyperkinetic due to increased cardiac output, or ...

BACKGROUND: The optimal oxygen concentration for the resuscitation of premature infants remains controversial. Objectives: We studied the effects of 21 versus 100% oxygen at initial resuscitation and also the effects of 24-hour exposure to 100% oxygen on arterial blood gases, oxidant lung injury, activities of lung antioxidant enzymes (AOEs) and ...

BACKGROUND: Surgical pulmonary endarterectomy is the preferred treatment for chronic thromboembolic pulmonary hypertension. Persistent pulmonary hypertension after pulmonary endarterectomy has been recognized as a major determinant of poor outcome. We tested whether acute vasoreactivity identifies chronic thromboembolic pulmonary hypertension patients prone to develop persistent/recurrent pulmonary hypertension after pulmonary endarterectomy and ...

BACKGROUND: Tetralogy of Fallot with pulmonary atresia is a heterogeneous group of defects, characterised by diverse sources of flow of blood to the lungs, which often include multiple systemic-to-pulmonary collateral arteries. Controversy surrounds the optimal method to achieve a biventricular repair with the fewest operations while basing flow to the ...

BACKGROUND: Idiopathic pulmonary arterial hypertension (IPAH) is a disease characterized by progressively increased resistance of pulmonary arteries. In this study, we evaluated the mechanical property of single pulmonary artery smooth muscles cells (PASMC) from patients with IPAH and tested whether the PASMC showed abnormal response to a vasodilator by use ...

INTRODUCTION: Phosphodiesterase type 5 (PDE5) inhibitors are widely used as first-line therapy for erectile dysfunction (ED). Their efficacy and safety combined with an increasing understanding of cyclic guanosine monophosphate (cGMP)-regulated mechanisms have triggered a number of attempts to determine their effects on the cardiovascular system and their potential benefits in ...

Pulmonary Arterial Hypertension (PAH) is defined by a persistent elevation in pulmonary artery pressure with normal left-sided pressures. It is characterized by increased pulmonary vascular resistance due to increased vascular tone and structural remodeling of pulmonary vessels. PAH is a quite rare condition, thus considering the rarity, subtle presentation, and ...

PURPOSE: To prospectively evaluate the effect of injected ethanol on pulmonary artery pressure during embolosclerotherapy of arteriovenous malformations (AVMs). MATERIALS AND METHODS: This prospective study was conducted in 16 male and 14 female patients (37 sessions; mean age, 34 years; age range, 17-67 years) with AVMs during a 2-year period. ...

Idiopathic pulmonary arterial hypertension (IPAH) is a fatal disease that comprises sustained vasoconstriction, enhanced proliferation of pulmonary vascular cells, and in situ thrombosis. The discovery of several contributing signaling pathways in recent years has resulted in an expanding array of novel therapies; however, IPAH remains a progressive disease with poor ...

Hypoxemia, hypercarbia, and pulmonary arterial hypertension are known complications of advanced COPD. We sought to identify genetic polymorphisms associated with these traits in a population of patients with severe COPD from the National Emphysema Treatment Trial (NETT). In 389 participants from the NETT Genetics Ancillary Study, single-nucleotide polymorphisms (SNPs) were ...

BACKGROUND: We sought to determine the prevalence of pulmonary complications and especially pulmonary arterial hypertension (PAH) in an Australian scleroderma population. METHODS: Between July 2005 and June 2007, physicians in Western Australia were asked to refer patients with scleroderma specifically for pulmonary hypertension screening. All patients were assessed for PAH ...

This study defines the growth patterns for the external diameters of the pulmonary arteries in human foetuses, including relationships with sex, side of body (right-left) and foetal age. Using anatomical dissection, digital-image analysis (system of Leica Q Win Pro 16) and statistical analysis (ANOVA, regression analysis), a range of external ...

Inhaled nitric oxide (iNO) has been used for patients with increased pulmonary vascular resistance (PVR) shortly after Fontan operation, but repeat deterioration of PVR during or shortly after its withdrawal remains a major concern. Milrinone, a phosphodiesterase type 3 (PDE3) inhibitor, can also reduce PVR for postoperative patients with pulmonary ...

We assessed the usefulness of transthoracic Doppler-derived indexes obtained in the proximal pulmonary artery (PA) branch for estimating pulmonary vascular resistance (PVR) in 45 children with congenital heart disease (CHD) and 23 normal control subjects. The acceleration time, inflection time (InT), deceleration index, and peak velocity, which were measured from ...

BACKGROUND: Inhaled nitric oxide (iNO) is a potent pulmonary vasodilator, but therapeutic experience in patients with severe pulmonary hypertension is scarce. METHODS: Eleven patients with severe pulmonary hypertension, 6 due to pulmonary arterial hypertension and 4 due to chronic thromboembolic disease, were selected for iNO therapy. A phosphodiesterase type 5 ...

OBJECTIVE: To evaluate the effect of bed rest with music on the first postoperative day to decrease stress for patients who have undergone heart surgery. METHODS: A repeated-measures randomized controlled trial was used. The study took place in a cardiothoracic intermediary unit of a university hospital in Sweden. Fifty-eight patients ...

BACKGROUND: Shed mediastinal blood during cardiopulmonary bypass (cardiotomy blood) contains fat, particulate matter, and vasoactive mediators that can adversely affect the pulmonary and systemic vasculature, as well as impair gas exchange. Our aim was to evaluate the effects of processing cardiotomy blood on cardiovascular and pulmonary function after cardiac surgery. ...

A 33-year-old-man had severe secondary pulmonary hypertension due to perivalvular leakage at the aortic and mitral positions after aortic and mitral valve replacement. Preoperative cardiac catheterization revealed pulmonary artery pressure of 105/45 mmHg and pulmonary vascular resistance of 929 dynes.s.cm(-5) To save the patient, we performed aortic and mitral valve ...

PURPOSE: To prospectively evaluate the capability of dynamic perfusion MRI for assessment of disease severity and progression to pulmonary arterial hypertension (PAH) in connective tissue disease (CTD) patients. MATERIALS AND METHODS: In all, 18 gender- and age-matched CTD patients without and with PAH and nine healthy volunteers underwent dynamic perfusion ...

Pulmonary regurgitation is a very common phenomenon in pulmonary arteries after repair of patients of Tetralogy of Fallot (TOF) which is the most common complex congenital heart diseases. The aim of this study is to use numerical approaches to simulate flow variations in pulmonary artery after repair of patients of ...

Endogenous nitric oxide (NO) mediates pulmonary vasodilatation at birth, but inhaled NO fails to reduce pulmonary vascular resistance (PVR) in newborns with congenital diaphragmatic hernia (CDH). This study was designed to investigate the effects of ventilation, and the nature of its endogenous mediator, in fetal lambs with experimental CDH. Investigations ...

Aortopulmonary window is an uncommon condition, particularly so in adulthood because it is usually fatal in infancy or childhood if untreated. Very few cases of those who have survived to adulthood and been operated on successfully have been described. Our study aimed to provide clinical, investigative, surgical, and outcome details ...

Idiopathic pulmonary arterial hypertension (IPAH) is associated with lower levels of the pulmonary vasodilator nitric oxide (NO) and its biochemical reaction products (nitrite [NO(2) (-)], nitrate [NO(3) (-)]), in part, due to the reduction in pulmonary endothelial NO synthesis. However, NO levels are also determined by consumptive reactions, such as ...

BACKGROUND: Pulmonary regurgitation is common after repair of tetralogy of Fallot, predisposing to right ventricular dilatation and potentially fatal arrhythmias. Magnetic resonance studies of such patients led us to hypothesize that the amount of regurgitation, in the absence of an effective valve, depends on pulmonary arterial compliance and on the ...

Pulmonary hypertension frequently complicates the postoperative management of patients after congenital cardiac surgery. Inhaled nitric oxide is an effective treatment option, but rebound pulmonary hypertension can occur upon its withdrawal. Sildenafil may facilitate its withdrawal by restoring cyclic guanosine monophosphate availability via phosphodiesterase-5 inhibition. The purpose of this study was ...

RATIONALE: Phosphodiesterase 5 (PDE5) inhibitors (e.g., sildenafil) are selective pulmonary vasodilators in patients with pulmonary arterial hypertension. The mechanism(s) underlying this specificity remains unclear, but studies in genetically modified animals suggest it might be dependent on natriuretic peptide bioactivity. OBJECTIVES: We explored the interaction between PDE5 inhibitors and the natriuretic ...

A very rare combination including tetralogy of Fallot (TOF), absent pulmonary valve, and isolated left pulmonary artery is described. A 4-month-old girl presented with mild cyanosis and severe dyspnea. Echocardiography established the diagnosis of TOF and absent pulmonary valve with severe pulmonary regurgitation and moderate stenosis at the pulmonary valve ...

Children with Down's syndrome (DS)-associated complete atrioventricular septal defect (AVSD) have rapid and aggressive development of pulmonary vascular disease when compared with non-Down's syndrome (ND) children. We aimed to evaluate the role of plasma endothelin-1 (ET-1) and nitrate levels in DS children with complete AVSD-associated pulmonary hypertension (PH) and compare ...

BACKGROUND: Occasionally pulmonary artery banding is necessary to reduce pulmonary arterial blood flow and pressure in patients who cannot be repaired in a single stage. Traditional extraluminal PAB can be associated with significant morbidity. We describe our technique, applications, and results of endoluminal pulmonary artery banding (EPAB) with and without ...

BACKGROUND: The integrity of pulmonary surfactant (PS) is impaired during deep hypothermia and circulatory arrest (DHCA), a preferred bypass strategy for infants undergoing complex cardiac operations, due mainly to bypass-induced systemic inflammation. The requirement of L-arginine, a precursor of nitric oxide, is elevated during acute pulmonary inflammation. We hypothesized that ...

The aim of this study was to evaluate whether pregnancy affects contrast enhancement within the pulmonary arteries during computed tomography pulmonary angiography (CTPA). This was a retrospective analysis of the CTPA examinations of 16 pregnant and 16 non-pregnant female patients, suspected of having an acute pulmonary embolus (PE), during the ...

Over a 7-year period, the chart records of six pregnant women with cardiac diseases at potential risk of thromboembolism were reviewed. All six patients survived and recovered well eventually. LMWH and beta-adrenergic blocker were effective to deal with atrial fibrillation. Digitalis and dobutamine were beneficial to prevent heart failure resulted ...

BACKGROUND: Inhaled treprostinil was recently developed for the treatment of pulmonary arterial hypertension (PAH). We investigated the safety and acute haemodynamic effects of the combination oral sildenafil and inhaled treprostinil in an open label study in patients with precapillary pulmonary hypertension. METHODS AND PATIENTS: Inhaled nitric oxide (20ppm; n=50), sildenafil ...

OBJECTIVE: The bidirectional cavopulmonary connection (BCPC) has shown excellent results as an intermediate step towards permanent palliation in functional single ventricle morphology. The optimal timing of the BCPC, though, remains unclear. This investigation focuses on results in a subgroup of patients under 6 months of age. METHODS: Between 2001 and ...

BACKGROUND: Pulmonary hypertension is a disease characterized by an elevation in pulmonary arterial pressure that is diagnosed invasively via right heart catheterization. Such pathological altered pressures in the pulmonary vascular system should lead to changes in blood flow patterns in the main pulmonary artery. METHODS AND RESULTS: Forty-eight subjects (22 ...

The pulmonary vascular bed is both a source of and target for a number of vasoactive factors. Among the most important for pulmonary vascular homeostasis are factors that utilise cyclic guanosine monophosphate (cGMP) as an intracellular second messenger. These include nitric oxide and the natriuretic peptide family (atrial, brain and ...

We report a case of a giant pulmonary artery aneurysm associated with infundibular pulmonary stenosis. Echocardiography disclosed markedly enlarged main pulmonary artery, but no left to right shunt flow at levels of the atrial septum, ventricular septum and the pulmonary artery. Continuous wave Doppler revealed a maximum velocity of 1.5 ...