Detection Of Pulmonary Arterial Hypertension Research Articles

Right Ventricular Systolic Pressure Assessed by Echocardiography: A Predictive Factor of Mortality in Patients With Scleroderma

Pulmonary arterial hypertension (PAH) is a well-known complication of systemic sclerosis (SSc). Doppler echocardiographic screening for the detection of PAH (by measuring right ventricular systolic pressure [RVSP]) is therefore recommended for all patients with SSc. However, the validity of RVSP as a predictor of mortality in patients with SSc is not well established. Doppler-determined PAH identifies a high-risk subset of patients with SSc with decreased survival. We performed echocardiography in 155 consecutive patients with SSc between May 2005 and July 2006 and tested the value of an RVSP level of ≥36 mm Hg to predict mortality. Cox proportional hazards model was used to examine the individual relationship between each variable and the mortality rate. Tricuspid regurgitant jets for RVSP determination were quantified in 129 patients (82.6%), of which 47 (36.4%) had RVSP ≥36 mm Hg. The median follow-up time was 34 months. The 1-, 2-, and 3-year survival rates were significantly lower among SSc patients with RVSP ≥36 vs ≤36 mm Hg (82%, 78%, and 67% vs 98%, 90%, and 86%, respectively, P < 0.01 by Wilcoxon test). In a multivariate analysis including echocardiographic and clinical variables, only an RVSP ≥36 mm Hg and a New York Heart Association III/IV class were associated with increased mortality; the respective Cox hazard ratios were 2.22 (95% confidence interval [CI]: 1.01-4.89, P = 0.048) and 4.77 (95% CI: 2.09-10.90, P = 0.000). Our results indicate that Doppler RVSP identifies a high-risk subset and supports the use of Doppler RVSP as a screening test in patients with SSc who may warrant early treatment of their PAH.

Pulmonary survival study in 91 patients with systemic sclerosis

In systemic sclerosis (SSc), major determinant of morbidity and mortality is pulmonary complication including pulmonary interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). In this study, the natural course of pulmonary involvement in SSc patients was investigated. This was a historical cohort study of SSc patients at a referral center for SSc in Iran between February 1998 and December 2007. Patients had a standardized initial evaluation, and interstitial pulmonary involvement was established by high-resolution CT scan (HRCT). Pulmonary hypertension was assessed by tricuspid gradient on echocardiography. Development of abnormal FVC or DLCO was considered as secondary outcome. Analysis of pulmonary survival was performed for primary and secondary outcomes. Ninety-one SSc patients were included in the study with the mean age of 44.1 (14.8). Among these, 65 (71.4%) patients were classified as limited subtype (lcSSc) and 84 (93.3%) were women. PAH was investigated in 8 (8.2%) patients, 1 (6.7%) in dcSSc and 7 (15.9%) in lcSSc subtype of disease. ILD had developed after a median of 107 (SE = 24.4) months after the first symptom of SSc, and 29 patients (31.9%) developed pulmonary fibrosis. Alveolitis and fibrosis had developed after a median of 129.0 (22.9) and 259.0 (74.2) months, respectively. There was a significant difference in Alveolitis-free pulmonary survival between two subgroups of the disease, which showed pulmonary alveolitis developed later in limited SSc (P = 0.03). The difference was not significant in two subtypes when Cox regression model was used to identify the effect of other prognostic factors on pulmonary survival in patients. In the present study, clinical manifestations of two subtypes of disease were divergent at first; however they became convergent in late stages, and this was the same as results in previous studies. Echocardiography for evaluation of pulmonary hypertension and pulmonary function tests for early detection of ILD and PAH is recommended for SSc patients to detect early stages of pulmonary involvement before significant vascular and fibrotic changes occur.

Pulmonary arterial hypertension in women

Pulmonary arterial hypertension (PAH) is a rare condition characterized by sustained elevation in pulmonary arterial resistance leading to right heart failure. PAH afflicts predominantly women. Echocardiography is the initial investigation of choice for non-invasive detection of PAH but right-heart catheterization is necessary to confirm the diagnosis. Conventional treatment includes non-specific drugs (warfarin, diuretics, oxygen). The endothelin-1 receptor antagonist bosentan, the phosphodiesterase-5 inhibitor sildenafil, and prostanoids have been shown to improve symptoms, exercise capacity and haemodynamics. Intravenous prostacyclin is the first-line treatment for the most severely affected patients. Despite the most modern treatment, the overall mortality rate of pregnant women with severe PAH remains high. Therefore, pregnancy is contraindicated in women with PAH and an effective method of contraception is recommended in women of childbearing age. Therapeutic abortion should be offered, particularly when early deterioration occurs. If this option is not accepted, intravenous prostacyclin should be considered promptly. Recent advances in the management of PAH have markedly improved prognosis and have resulted in more women of childbearing age considering pregnancy. A multidisciplinary approach should give new insights into cardiopulmonary, obstetric and anaesthetic management during pregnancy, delivery and the postpartum period.

The three‐year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France

An algorithm for the detection of pulmonary arterial hypertension (PAH), based on the presence of dyspnea and the findings of Doppler echocardiographic evaluation of the velocity of tricuspid regurgitation (VTR) and right-sided heart catheterization (RHC), which was applied in a large multicenter systemic sclerosis (SSc) population, estimated the prevalence of PAH to be 7.85%. The aim of this observational study was to investigate the incidence of PAH and pulmonary hypertension (PH) during a 3-year followup of patients from the same cohort (the ItinérAIR-Sclérodermie Study). Patients with SSc and without evidence of PAH underwent evaluation for dyspnea and VTR at study entry and during subsequent visits. Patients in whom PAH was suspected because of a VTR of 2.8-3.0 meters/second and unexplained dyspnea or a VTR of >3.0 meters/second underwent RHC to confirm the diagnosis. A total of 384 patients were followed up for a mean+/-SD of 41.03+/-5.66 months (median 40.92 months). At baseline, 86.7% of the patients were women, and the mean+/-SD age of the patients was 53.1+/-12.0 years. The mean+/-SD duration of SSc at study entry was 8.7+/-7.6 years. After RHC, PAH was diagnosed in 8 patients, postcapillary PH in 8 patients, and PH associated with severe pulmonary fibrosis in 2 patients. The incidence of PAH was estimated to be 0.61 cases per 100 patient-years. Two patients who exhibited a mean pulmonary artery pressure of 20-25 mm Hg at baseline subsequently developed PAH. The estimated incidence of PAH among patients with SSc was 0.61 cases per 100 patient-years. The high incidence of postcapillary PH highlights the value of RHC in investigating suspected PAH.

Exploration d’une hypertension pulmonaire

Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure (PAPm) superior than 25mmHg at rest or superior than 30mmHg with exercise. The classification of PH differentiates between "secondary" PH which results from a well-known disease, such as PH due to thromboembolic disease (obstructive PH), left cardiac failure (passive PH), or chronic respiratory diseases (hypoxic PH), and pulmonary arterial hypertension (PAH). PAH is a rare disease characterized by a progressive increase of pulmonary vascular resistance leading to right ventricular failure. PAH is classified as idiopathic, familial, or associated with various conditions (connective tissue diseases, congenital heart diseases with systemic-to-pulmonary shunts, portal hypertension, infection with the human immunodeficiency virus, or appetite-suppressant drugs). Transthoracic Doppler echocardiography is the investigation of choice for non invasive detection of PAH but right-heart catheterization is necessary to confirm the diagnosis of PAH and determine its mechanism. Pulmonary function tests and chest CT scan may detect an underlying chronic pulmonary disease (hypoxic PH). Lung perfusion scan and contrast-enhanced chest spiral CT scan can lead to the diagnosis of thromboembolic PH, which is to be confirmed by pulmonary angiography. Assessment of the severity of PH is based on clinical parameters (NYHA, right heart failure), functional tests (six-minute walk test), echocardiography and hemodynamics. Characterization of PH is essential in the management of PH because it determines the appropriate treatment: an etiological treatment in passive, obstructive or hypoxemic PH, or vasodilatator and antiproliferative therapies in PAH.

Computed Tomographic Screening of Pulmonary Arterial Hypertension in Candidates for Lung Transplantation

Introduction Computed tomography (CT) is essential to evaluate candidates for lung transplantation (LT) We sought to correlate identification of pulmonary arterial hypertension (PAH) via CT to echocardiographic (Eco) and/or hemodynamic observations (Hd) as well as the pathological findings (Path) at posttransplant pneumonectomy. Materials and Methods The study included 24 consecutive months follow-up of 71 patients who were referred for LT evaluation with 54 undergoing LT. Study parameters included CT [diameter of the principal pulmonary artery (PPA) ≥ 29 mm), PPA/diameter of the ascending aorta (AA) ratio > 1; diameter of the lobar artery (LA)/diameter of the lobar bronchiole (LB) ratio > 1 in three or more lobes; thickening of the anterosuperior pericardial recess (>15 mm); mosaic lung density pattern]; PAPs via Eco and/or Hd, and findings Path of PAH. Statistical analysis included chi-square and Pearson correlation coefficient. Results Thirty-five patients had PAH. Significant relationships ( P < .05) were observed between PAH (Eco and/or Hd) and PPA, PPA/AA, and LA/LB. The sensitivity of the finding PPA ≥ 29 mm was 65.9%, and 85.7% when combined with LA/LB > 1. In the Path study, 14/54 patients revealed alterations compatible with PAH. Significant relationships ( P < .05) were observed between PAH via Path and PPA (CT) and between PAH via Path and PAH (Eco and/or Hd). Conclusions CT is useful for the detection of PAH. Findings of PAH via CT were indicative of the need for further confirmatory studies. In contrast, the absence of findings with CT does not exclude the possible presence of PAH.

Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment

Pulmonary arterial hypertension (PAH) is an important cause of mortality in systemic sclerosis (SSc). The symptoms are non-specific and can be ascribed to other features of the disease, so it is often underrecognized until the late stages. Earlier treatment with new agents is associated with better treatment outcomes. The aim of this article is to develop evidence-based guidelines for screening for PAH and interstitial lung disease (ILD) in SSc. PAH occurs in up to 27% of patients with SSc. Abnormal pulmonary function, particularly a disproportionate fall in carbon monoxide diffusing capacity (DLCO), can identify patients in the early stages of PAH, prompting further investigation in high-risk patients (limited SSc of >10 years' duration, symptoms and/or signs of PAH, DLCO <50% predicted, a rapid or large fall in DLCO without evidence of ILD and/or estimated systolic pulmonary artery pressure >45 mmHg on echocardiography). Right heart catheter remains the diagnostic gold standard. An algorithm for screening with regular pulmonary function tests for the early detection of PAH and ILD in SSc is proposed.

Right ventricle Tei-index: A tool to increase the accuracy of non-invasive detection of pulmonary arterial hypertension in connective tissue diseases

To assess the accuracy of echocardiography for predicting pulmonary arterial hypertension (PAH) in a cohort of patients with systemic sclerosis and other connective tissue diseases, and to evaluate whether addition of the right ventricular (RV) Tei-index contributes to the non-invasive diagnosis of PAH in this patient group. Ninety-eight patients with systemic sclerosis and other connective tissue diseases in whom an echocardiography was performed in the period from January 1st 2004 to July 1st 2005 were included. Echocardiographic systolic pulmonary arterial pressure (PAP), end-diastolic PAP and RV Tei-index were calculated. In cases with a high suspicion of PAH right heart catheterization was performed and systolic, diastolic and mean PAP as well as pulmonary wedge pressure, cardiac output and pulmonary vascular resistance were obtained. These results were compared to the echocardiographic measurements. The average RV Tei-index of our patients was substantially above normal values. In 35 (36%) patients a right heart catheterization was performed and PAH was confirmed in 28 patients. In 6 of 7 patients without PAH, the RV Tei-index was below the upper limit of normal. A significant correlation was found between the RV Tei-index and the catheterization parameters such as systolic PAP, diastolic PAP and mean PAP. The accuracy of echocardiography for the detection of PAH increases when the echocardiographic systolic PAP is combined with an elevated RV Tei-index. As a result, by applying the Tei-index, the number of negative catheterizations can be minimized.

1139 Exercise Doppler echocardiography in the detection of pulmonary arterial hypertension in collagen vascular disease

1139 Exercise Doppler echocardiography in the detection of pulmonary arterial hypertension in collagen vascular disease

Early detection of pulmonary arterial hypertension in systemic sclerosis: A French nationwide prospective multicenter study

Screening allows for early management of pulmonary arterial hypertension (PAH), a severe complication of systemic sclerosis (SSc). Since no consensus has been reached on the method and criteria for optimal screening, we sought to develop an algorithm based on symptoms, Doppler echocardiography, and right heart catheterization (RHC) for application to a nationwide multicenter SSc population in France. This prospective study was conducted from September 2002 to July 2003 by experts at 21 SSc centers. At each center, SSc patients without severe pulmonary function abnormalities underwent Doppler echocardiography by an experienced cardiologist. Patients with a peak velocity of tricuspid regurgitation (VTR) of >3 meters/second or 2.5-3 meters/second with unexplained dyspnea were asked to undergo RHC to confirm PAH according to international guidelines. Of the 599 patients analyzed, 29 had known PAH and 33 had suspected PAH, based on Doppler echocardiography, and underwent RHC. Of these 33, 18 were found to have PAH, 3 had left ventricular dysfunction, and 12 had no PAH. Newly diagnosed cases of PAH were of mild severity (mean +/- SD pulmonary artery pressure [mPAP] 30 +/- 9 mm Hg, mean +/- SD total pulmonary resistance [TPR] 524 +/- 382 dynes x second/cm(5)). Hemodynamic findings in patients with known PAH were mPAP 49 +/- 17 mm Hg and TPR 1,007 +/- 615 dynes x second/cm(5). The estimate of PAH prevalence was 7.85% (95% confidence interval 5.70-10.00). This screening algorithm, based on dyspnea, Doppler echocardiographic evaluation of VTR, and RHC, enabled early detection of PAH at a mild stage. Whether mild PAH will evolve to severe PAH in reported cases and whether this early diagnosis translates into improved prognosis for patients with mild PAH will be evaluated in the ongoing 3-year followup of this cohort.

Pulmonary arterial hypertension associated to connective tissue diseases

Pulmonary arterial hypertension is a well-known complication of connective tissue diseases such as systemic sclerosis, systemic lupus erythematosus, mixed connective tissue diseases, and to a lesser extent, rheumatoid arthritis, dermatopolymyositis and primary Sjögren's syndrome. In these patients, pulmonary hypertension may occur in association with left heart disease, interstitial fibrosis or as a result of a isolated pulmonary arteriopathy. The incidence of pulmonary arterial hypertension in the limited form of systemic sclerosis is about 10%. The pathophysiologic mechanisms leading to pulmonary arterial hypertension remain unknown. Symptoms and clinical presentation are very similar to idiopathic pulmonary arterial hypertension but mortality was confirmed to be higher. Echocardiography is the reference investigation for the detection of pulmonary arterial hypertension but the results should be confirmed by right heart catheterization. Treatment appears more complex as compared to idiopathic pulmonary arterial hypertension. Intravenous epoprostenol therapy has been shown to be effective in a special trail. Also, the endothelin receptor antagonists bosentan and sitaxentan, the phosphodyesterase-type-5 sildenafil and subcutaneous treprostinil have shown favourable results.

Hypertension artérielle pulmonaire

Pulmonary arterial hypertension (PAH) is a rare condition characterised by progressively elevated pulmonary arterial resistance leading to right heart failure. A recent classification distinguishes idiopathic PAH, familial PAH and PAH secondary to other conditions (connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus infection or appetite suppressant exposure). Echocardiography is the initial investigation of choice for non-invasive detection of PAH but measurement of pulmonary pressures and cardiac output during right-heart catheterization are necessary to confirm the diagnosis of PAH. Conventional treatment includes non-specific drugs (warfarin, diuretics, oxygen). Intravenous epoprostenol is the first-line treatment for the most severely affected patients. In less severe cases, the first-line treatment may include bosentan or a prostacyclin analogue. Recent advances in the management of PAH have markedly improved prognosis. The avai-lability of novel specific drugs including type 5 phosphodiesterase inhibitors offers novel therapeutic perspectives but their exact role in the treatment of PAH is still uncertain. The evolution of therapy from vasodilators to antiproliferative agents reflects the advancement in our understanding of the mechanisms mediating pulmonary arterial hypertension.

L'hypertension artérielle pulmonaire associée à la sclérodermie systémique : proposition d'un algorithme échocardiographique de dépistage pour un diagnostic précoce (ItinérAIR–Sclérodermie)

Purpose. – Pulmonary arterial hypertension (PAH) is a severe complication of scleroderma. Its prevalence varies from 5% to 35% in the literature. A systematic yearly screening is recommended for early detection and management of PAH, but no precise algorithm is yet available. Methods. – From literature analysis as well as evaluation of medical needs and practices, a multidisciplinary board of experts proposed an algorithm for the screening of PAH in scleroderma. Results. – This algorithm is based on a precise Doppler echocardiography methodology for the purpose of screening scleroderma patients for PAH. Patients are considered as being at high or low risk of PAH depending on the maximal tricuspid regurgitation velocity. High-risk patients undergo right heart catheterization for confirmation of the diagnosis of PAH. A French multicenter transversal observational study (“ItinérAIR Sclérodermie”) will be conducted in 21 hospital centers in France and involved 100 investigators organized as multidisciplinary networks. Future prospects. – Final results will provide confirmation that the screening algorithm is applicable in a real world setting, as well as a better knowledge of the prevalence of PAH in the various sub-groups of scleroderma patients, of the risk profile for PAH and of the value of DLCO as a predictive factor for PAH, and will support elaboration of precise screening guidelines.

Detection of pulmonary hypertension by Doppler echocardiography of the inferior vena cava in chronic airflow obstruction.

Pulsed Doppler echocardiography of the inferior vena cava is an accurate method for the diagnosis of tricuspid regurgitation and impaired right ventricular compliance, two features of pulmonary hypertension. The purpose of this study was to assess the value of Doppler echocardiography of the inferior vena cava for the detection of pulmonary arterial hypertension in patients with chronic obstructive lung disease. Pulse Doppler echocardiography of the inferior vena cava and right heart catheterisation were performed in 29 patients with severe chronic obstructive lung disease. The mean pulmonary arterial pressure was 27 (10) mm Hg for the entire group; 62% of patients (18/29) had pulmonary arterial hypertension (mean pulmonary arterial pressure greater than 20 mm Hg). An adequate Doppler signal could be obtained in 25 of the 29 patients (86%). Pulsed Doppler echocardiography of the inferior vena cava gave normal results in 10 patients and disclosed tricuspid regurgitation in seven patients, impaired right ventricular compliance in seven patients, and both of these abnormalities in one patient. An abnormal Doppler echocardiogram of the inferior vena cava (tricuspid regurgitation or impaired right ventricular compliance, or both) predicted the presence of pulmonary arterial hypertension with a sensitivity of 87% and a specificity of 80%. These results suggest that pulsed Doppler echocardiography of the inferior vena cava may be a useful though imperfect method of detecting pulmonary arterial hypertension in patients with chronic obstructive lung disease.