Echocardiography May Help Detect Pulmonary Vasculopathy
Echocardiography May Help Detect Pulmonary Vasculopathy
Background: Pulmonary arterial hypertension (PAH) in patients with systemic sclerosis is associated with a poor prognosis, but this can be improved by early disease detection. Abnormal pulmonary and cardiac function can be detected early by means of echocardiography, whereas right heart catheterization is usually performed later.
Objectives: The purpose of this prospective study was to detect early the presence of pulmonary artery vasculopathy in patients with verified systemic sclerosis without significant pulmonary fibrosis, normal lung volumes and a mildly reduced lung diffusion capacity of carbon monoxide (DLCO).
Methods: Nineteen consecutive female NYHA class I-II patients with scleroderma and a PAPs of < 35 mm/Hg measured by echocardiography, were enrolled between September 2007 and September 2009. They had a mean age of 51 ± 13 years, body mass index of 25 ± 5 kg/m). They all underwent complete Doppler echocardiography, CPET, a pulmonary ventilation test (carbon monoxide lung diffusion, DLCO), HRCT. To investigate PAH by means of complete resting Doppler echocardiography estimates of systolic pulmonary artery pressure (PAPs) derived from tr icuspid regurgitation, mean PAP derived from pulmonary regurgitation, pulmonary vessel resistance (PVR) derived from the acceleration time of the pulmonary outflow tract (ACTpo), and right ventricular function derived from tricuspid annular plane systolic excursion (TAPSE). Right heart catheterisation was conducted only, if pulmonary hypertension was suggested by echocardiography and an abnormal ventilator test.
The data are given as mean values ± SD, unless otherwise stated. The correlations between the variables were analysed using Pearson's r coefficient, and the predictive value of the variables was calculated using linear regression analysis. A p value of > 0.05 was considered significant.
Results: Right heart catheterization detected PAH in 15/19 patients; mean PAP was 30.5 mm/Hg and RVP 3.6 UW. Coronary angiography of the patients aged more than 55 years showed some evidence of significant coronary artery disease. Echocardiography showed high systolic PAP values (46 ± 8 mmHg), whereas right ventricular function was normal (TAPSE 23 ± 3 mm), and in line with the NYHA class. ACTpo was reduced in the patients with a systolic PAP of < 46 mm/Hg (p > 0.001) and positively correlated with DLCO (p > 0.001) and the hemodynamic data.
There was a good correlation between ACTpo and PVR (hemodynamic data) (r = −0615; p > 0.01).
Conclusions: Although they need to be confirmed by studies of larger series of patients, our findings suggest that, in comparison with hemodynamic data, non-invasive echocardiographic measurements are an excellent means of identifying early-stage PAH.
Systemic sclerosis is a chronic disease essentially due to microvascular abnormalities, antibodies and a fibroblast production that leads to excessive collagen levels, and skin and viscera fibrosis.
The most recent data show that the prevalence of systemic sclerosis from 1977 to 1980 was 12.6-25/100,000, with an incidence of 0.06-1.9/100,000. The recorded mean age of the patients at the time of onset is between 45 and 65 years, this data does not reflect the real age as the symptoms may appear many years before the first specialist assessment. The main pulmonary complications are pulmonary fibrosis and pulmonary arterial hypertension (PAH): interstitial lung disease is observed in 70% of the patients with diffused systemic sclerosis, and pulmonary hypertension in 35% of those with limited systemic sclerosis. The patients have no symptoms at rest during the initial stages of the disease, but soon develop slight dyspnea during physical exercise or rest; some may also experience chest pain, asthenia and loss of conscience under stress.
As pulmonary hypertension in systemic sclerosis may not only be due to PAH, but also to interstitial lung disease and cardiac involvement, it is very important to classify it adequately in order to determine the best treatment. According to the 2009 guidelines, PAH describes an increase in mean pulmony artery pressure (PAPm) to more than 25 mmHg. Echocardiography and respiratory function testing are recommended every year, even if the patient is asymptomatic, and right heart catheterisation is usually performed in late stages.
The published frequencies of PAH in systemic sclerosis vary widely (from 5% to 35%), depending on the study population, and diagnostic methods (cardiac echo-Doppler or right heart catheterisation) and criteria. Echocardiography plays a central role in the non-invasive screening and management of patients with suspected PAH as it guarantees the early identification and treatment of a condition that is usually diagnosed at least two or three years after its onset.
PAH and interstitial lung disease are the leading causes of death in systemic sclerosis and, as their early detection and treatment should improve morbidity and mortality. The aim of this prospective study was to detect the presence of pulmonary artery vasculopathy early in patients with verified systemic sclerosis and normal lung volumes, no significant pulmonary fibrosis, and mildly reduced lung carbon monoxide diffusing capacity (DLCO) by: 1) evaluating resting pulmonary function at rest, and maximum and sub-maximum exercise capacity; 2) detecting systolic pulmonary artery pressure (PAPs) values of < 35 mmHg using thoracic echocardiography; and 3) investigating the relationships between PAP and the acceleration time of the pulmonary outflow tract (ACTpo), right ventricular function (tricuspid annular plane systolic excursion; TAPSE), and right heart catheterisation (PAPs, PAPm and pulmonary vessel resistance [PVR]).
Abstract and Introduction
Abstract
Background: Pulmonary arterial hypertension (PAH) in patients with systemic sclerosis is associated with a poor prognosis, but this can be improved by early disease detection. Abnormal pulmonary and cardiac function can be detected early by means of echocardiography, whereas right heart catheterization is usually performed later.
Objectives: The purpose of this prospective study was to detect early the presence of pulmonary artery vasculopathy in patients with verified systemic sclerosis without significant pulmonary fibrosis, normal lung volumes and a mildly reduced lung diffusion capacity of carbon monoxide (DLCO).
Methods: Nineteen consecutive female NYHA class I-II patients with scleroderma and a PAPs of < 35 mm/Hg measured by echocardiography, were enrolled between September 2007 and September 2009. They had a mean age of 51 ± 13 years, body mass index of 25 ± 5 kg/m). They all underwent complete Doppler echocardiography, CPET, a pulmonary ventilation test (carbon monoxide lung diffusion, DLCO), HRCT. To investigate PAH by means of complete resting Doppler echocardiography estimates of systolic pulmonary artery pressure (PAPs) derived from tr icuspid regurgitation, mean PAP derived from pulmonary regurgitation, pulmonary vessel resistance (PVR) derived from the acceleration time of the pulmonary outflow tract (ACTpo), and right ventricular function derived from tricuspid annular plane systolic excursion (TAPSE). Right heart catheterisation was conducted only, if pulmonary hypertension was suggested by echocardiography and an abnormal ventilator test.
The data are given as mean values ± SD, unless otherwise stated. The correlations between the variables were analysed using Pearson's r coefficient, and the predictive value of the variables was calculated using linear regression analysis. A p value of > 0.05 was considered significant.
Results: Right heart catheterization detected PAH in 15/19 patients; mean PAP was 30.5 mm/Hg and RVP 3.6 UW. Coronary angiography of the patients aged more than 55 years showed some evidence of significant coronary artery disease. Echocardiography showed high systolic PAP values (46 ± 8 mmHg), whereas right ventricular function was normal (TAPSE 23 ± 3 mm), and in line with the NYHA class. ACTpo was reduced in the patients with a systolic PAP of < 46 mm/Hg (p > 0.001) and positively correlated with DLCO (p > 0.001) and the hemodynamic data.
There was a good correlation between ACTpo and PVR (hemodynamic data) (r = −0615; p > 0.01).
Conclusions: Although they need to be confirmed by studies of larger series of patients, our findings suggest that, in comparison with hemodynamic data, non-invasive echocardiographic measurements are an excellent means of identifying early-stage PAH.
Introduction
Systemic sclerosis is a chronic disease essentially due to microvascular abnormalities, antibodies and a fibroblast production that leads to excessive collagen levels, and skin and viscera fibrosis.
The most recent data show that the prevalence of systemic sclerosis from 1977 to 1980 was 12.6-25/100,000, with an incidence of 0.06-1.9/100,000. The recorded mean age of the patients at the time of onset is between 45 and 65 years, this data does not reflect the real age as the symptoms may appear many years before the first specialist assessment. The main pulmonary complications are pulmonary fibrosis and pulmonary arterial hypertension (PAH): interstitial lung disease is observed in 70% of the patients with diffused systemic sclerosis, and pulmonary hypertension in 35% of those with limited systemic sclerosis. The patients have no symptoms at rest during the initial stages of the disease, but soon develop slight dyspnea during physical exercise or rest; some may also experience chest pain, asthenia and loss of conscience under stress.
As pulmonary hypertension in systemic sclerosis may not only be due to PAH, but also to interstitial lung disease and cardiac involvement, it is very important to classify it adequately in order to determine the best treatment. According to the 2009 guidelines, PAH describes an increase in mean pulmony artery pressure (PAPm) to more than 25 mmHg. Echocardiography and respiratory function testing are recommended every year, even if the patient is asymptomatic, and right heart catheterisation is usually performed in late stages.
The published frequencies of PAH in systemic sclerosis vary widely (from 5% to 35%), depending on the study population, and diagnostic methods (cardiac echo-Doppler or right heart catheterisation) and criteria. Echocardiography plays a central role in the non-invasive screening and management of patients with suspected PAH as it guarantees the early identification and treatment of a condition that is usually diagnosed at least two or three years after its onset.
PAH and interstitial lung disease are the leading causes of death in systemic sclerosis and, as their early detection and treatment should improve morbidity and mortality. The aim of this prospective study was to detect the presence of pulmonary artery vasculopathy early in patients with verified systemic sclerosis and normal lung volumes, no significant pulmonary fibrosis, and mildly reduced lung carbon monoxide diffusing capacity (DLCO) by: 1) evaluating resting pulmonary function at rest, and maximum and sub-maximum exercise capacity; 2) detecting systolic pulmonary artery pressure (PAPs) values of < 35 mmHg using thoracic echocardiography; and 3) investigating the relationships between PAP and the acceleration time of the pulmonary outflow tract (ACTpo), right ventricular function (tricuspid annular plane systolic excursion; TAPSE), and right heart catheterisation (PAPs, PAPm and pulmonary vessel resistance [PVR]).
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