+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Right-heart function related to the results of acute pulmonary vasodilator testing in patients with pulmonary arterial hypertension caused by connective tissue disease



Right-heart function related to the results of acute pulmonary vasodilator testing in patients with pulmonary arterial hypertension caused by connective tissue disease



Journal of the American Society of Echocardiography 25(3): 274-279



Acute pulmonary vasodilator testing is important for patients with pulmonary arterial hypertension, but little is known about the predictors of response to such testing. Forty-eight patients (mean age, 41.3 ± 11.6 years; 91.7% women) with pulmonary arterial hypertension associated with connective tissue diseases who underwent right-heart catheterization and acute pulmonary vasodilator testing were prospectively recruited. Echocardiography was performed before and immediately after testing. There were 14 responders (29.2%) to acute pulmonary vasodilator testing. Responders had lower pulmonary vascular resistance, higher peak systolic velocity of the lateral tricuspid valve annulus (right ventricular [RV] S') and tricuspid annular plane systolic excursion, and smaller RV end-diastolic area. After vasodilator testing, mean pulmonary artery pressure and pulmonary vascular resistance decreased significantly in both groups, cardiac index increased significantly in responders, and RV function improved significantly in nonresponders. Receiver operating characteristic curve analysis identified an optimal cutoff value for RV S' of ≥10.5 cm/sec to predict response, with sensitivity of 71% and specificity of 71%. There were more responders among patients with RV S' ≥ 10.5 cm/sec (45.5% vs 15.4%, P = .02). On multivariate logistic regression analysis, RV S' ≥ 10.5cm/sec emerged as an independent predictor of response (odds ratio, 4.58; 95% confidence interval, 1.18-17.79; P = .02). Right-heart function is better in responders to acute pulmonary vasodilator testing than in nonresponders among patients with pulmonary arterial hypertension associated with connective tissue diseases, and pulmonary vasodilators may improve RV function in nonresponders and cardiac index in responders. RV S' is a simple and clinically useful tool for predicting the results of pulmonary vasodilator testing.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 055575586

Download citation: RISBibTeXText

PMID: 22137255

DOI: 10.1016/j.echo.2011.11.007


Related references

Pulmonary capillary endothelial metabolic dysfunction: severity in pulmonary arterial hypertension related to connective tissue disease versus idiopathic pulmonary arterial hypertension. Arthritis and Rheumatism 58(4): 1156-1164, 2008

Left heart abnormalities in connective tissue disease patients with pre-capillary pulmonary hypertension as well as borderline mean pulmonary arterial pressure. Modern Rheumatology 25(5): 744-747, 2015

Comparison of adenosine versus iloprost by acute vasodilator test in patients of pulmonary arterial hypertension complicated with connective tissue diseases. Zhonghua Yi Xue Za Zhi 89(30): 2099-2102, 2009

Acute pulmonary vasodilator testing is a critical step in the management of patients with pulmonary arterial hypertension. Zhonghua Jie he He Hu Xi Za Zhi 31(5): 378-380, 2008

Long-term Outcomes of Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease as Compared With Idiopathic or Heritable Pulmonary Arterial Hypertension Treated With Tadalafil. Chest Journal 140(4): 734a-734b, 2011

Continuous intravenous prostacyclin treatment for primary pulmonary hypertension and pulmonary arterial hypertension associated with either connective tissue diseases or congenital heart disease. Circulation 104(17 Suppl.): II 250, 2001

Red blood cell distribution width predicts responsiveness of acute pulmonary vasodilator testing in patients with idiopathic pulmonary arterial hypertension. Clinica Chimica Acta; International Journal of Clinical Chemistry 446: 272-276, 2015

Bosentan in Pulmonary Arterial Hypertension Related to Connective Tissue Disease with Moderate to Severe Pulmonary Fibrosis. Chest Journal 126(4): 884s-1-884s-2, 2004

Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide. Internal Medicine 56(4): 445-448, 2017

Inhaled iloprost during acute pulmonary vasodilator testing for preoperative assessment of surgical operability of congenital heart disease with severe pulmonary hypertension. Zhonghua Wai Ke Za Zhi 48(10): 727-730, 2010

Acute pulmonary vasodilator testing with inhaled treprostinil in children with pulmonary arterial hypertension. Pediatric Cardiology 34(4): 1006-1012, 2013

Long-term safety profile of bosentan in patients with pulmonary arterial hypertension related to mixed connective tissue disease or systemic lupus erythematosus: Results from the Tracleer post marketing surveillance database. 2006

Comparison of baseline characteristics and survival between patients with idiopathic and connective tissue disease-related pulmonary arterial hypertension. Journal of Heart and Lung Transplantation 28(6): 621-627, 2009

Acute endothelin A receptor antagonism improves pulmonary and systemic haemodynamics in patients with pulmonary arterial hypertension that is primary or autoimmune and related to congenital heart disease. Heart 89(10): 1221-1226, 2003

Connective tissue disease-related pulmonary arterial hypertension. Best Practice and Research. Clinical Rheumatology 30(1): 22-38, 2016