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 ...

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 ...

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. ...

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 ...

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 ...

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 ...

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: 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 ...

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 ...

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 ...

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 ...

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 ...

Abstract Introduction: Asthma is known to be associated with the structural alterations of the airways - airway remodelling. The features of these alterations had been well described; however, the effects of airway inflammation on the adjacent vessels have been virtually unknown. One exception is a study from 1922 by Huber ...

The available treatment strategies against pulmonary hypertension include the administration of endothelin-1 (ET-1) receptor subtype blockers (ET(A) and ET(B) antagonists); vasoactive intestinal polypeptide (VIP) has recently been suggested as a potential new therapeutic agent. We set out to investigate the ability of these agents to protect against the vasoconstriction and ...

RATIONALE: Growth-differentiation factor (GDF)-15 is a stress-responsive, transforming growth factor-beta-related cytokine. Circulating levels of GDF-15 provide independent prognostic information in patients with acute pulmonary embolism and chronic left-sided heart failure. OBJECTIVES: To assess the prognostic value of GDF-15 in idiopathic pulmonary arterial hypertension. METHODS: GDF-15 levels were determined in 76 ...

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) causes physical plugging of large pulmonary arteries as well as a distal micro-vasculopathy. Pulmonary endothelium is an active metabolic tissue in normal humans. The effects of CTEPH on pulmonary endothelial metabolism are unknown. OBJECTIVES: We studied pulmonary capillary endothelium-bound angiotensin converting enzyme (ACE) activity ...

PURPOSE: The purpose of this study was to determine the normal range of the main pulmonary artery diameter (MPAD) by computed tomography (CT) in persons with normal pulmonary artery pressure, and then to evaluate the relationship of the diameter with age, gender, and body surface area (BSA). MATERIALS AND METHODS: ...

Pulmonary hypertension remains a significant complication of several systemic and cardiothoracic diseases. It is important to emphasize that the hemodynamic relevance relates to the effect of pulmonary hypertension on right ventricular function and right-left ventricular interaction. The goal of pulmonary vasodilation should focus on optimizing right ventricular function and improving ...

This study was undertaken to determine the diastolic Doppler echocardiographic correlates of pulmonary vascular resistance calculated on cardiac catheterization in patients with secondary pulmonary arterial hypertension. Thirty-eight consecutive patients with congenital heart disease, pulmonary artery hypertension and pulmonary regurgitation were studied. Continuous-wave Doppler-derived pulmonary artery diastolic gradients were measured at ...

OBJECTIVES: Sildenafil, a phosphodiesterase-5 inhibitor, significantly improves oxygenation when used in animal models and patients with pulmonary hypertension. Tadalafil is a new and clinically available phosphodiesterase-5 inhibitor that, aside from causing pulmonary vasodilation, has been shown to increase cardiac output in pulmonary hypertensive adults. Its hemodynamic effects on the newborn, ...

AIM: The aim of the present study was to identify the possible genotypic association of 3'UTR Hind III polymorphism of Plasminogen activator Inhibitor-1 (PAI-1) gene with idiopathic pulmonary arterial hypertension (IPAH). BACKGROUND: IPAH is a disorder with abnormally raised mean pulmonary arterial pressure and increase in the resistance to blood ...

The aim of this study was to test whether the simple ratio of right ventricular (RV) end-systolic pressure (Pes) to stroke volume (SV), known as the effective arterial elastance (Ea), provides a valid assessment of pulmonary arterial load in case of pulmonary embolism- or endotoxin-induced pulmonary hypertension. Ventricular pressure-volume (PV) ...

Increasing evidence supports the use of embolisation to treat pulmonary arteriovenous malformations (AVMs). Most pulmonary AVM patients have hereditary haemorrhagic telangiectasia (HHT), a condition that may be associated with pulmonary hypertension. The current authors tested whether pulmonary AVM embolisation increases pulmonary artery pressure (P(pa)) in patients without baseline severe pulmonary ...

OBJECTIVES: To compare iomeprol 400 and iodixanol 320 in pulmonary artery MDCTA in subjects with suspected pulmonary embolism. METHODS: Eighty randomized subjects received equi-iodine intravenous doses (48 g) of iomeprol 400 (n=40) or iodixanol 320 (n=40), via power injector at 4 mL/s. Four-row (35 subjects) and 64-row (45 subjects) scanners ...

AIM: The aim of this study was to determine the presence of the neuronal nitric oxide synthase (nNOS) in near full-term lambs with congenital diaphragmatic hernia (CDH) and its role in the modulation of pulmonary vascular basal tone. METHODS: We surgically created diaphragmatic hernia on the 85th day of gestation. ...

Pulmonary arterial hypertension (PAH) associated with congenital heart disease remains a major problem despite advances in cardiac surgery. Recently, advanced therapies for PAH have become available and have been effective in reducing pulmonary vascular resistance and symptoms in patients with near-systemic pulmonary arterial pressures, previously thought to have irreversible pulmonary ...

Pulmonary arterial hypertension (PAH) is associated with significant perioperative risk for major complications, including pulmonary hypertensive crisis and cardiac arrest. Several mechanisms of hemodynamic deterioration, including acute increases in pulmonary vascular resistance (PVR), alterations of ventricular contractility and function and coronary hypoperfusion can contribute to morbidity. Anesthetic drugs exert a ...

Prostacyclin and prostacyclin analogues are potent vasodilators and possess antithrombotic, anti-inflammatory and antiproliferative properties. These properties qualify them as efficient drugs for the treatment of pulmonary hypertension, a life-threatening illness characterized by an increase in artery pressure and vascular resistance in the pulmonary circulation. Diseased pulmonary vessels show specific remodeling ...