New predictors of outcome in idiopathic pulmonary arterial hypertension

Kawut, Steven M.; Horn, Evelyn M.; Berekashvili, Ketevan; Garofano, Robert P.; Goldsmith, Rochelle L.; Widlitz, Allison; Rosenzweig, Erika B.; Kerstein, Diane; Barst, Robyn J.

doi:10.1016/j.amjcard.2004.09.006

Cited by 223 publications

(207 citation statements)

References 27 publications

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“…Changes in NYHA functional class, cardiac index, SvO 2 and NT-proBNP level better predict outcome than their baseline measurement [22,23]. Improvement in RVEF at 3-6 months after therapy was the best predictor of cardiovascular and overall survival.…”

Section: Discussionmentioning

confidence: 86%

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

et al. 2014

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Right ventricle ejection fraction (RVEF) evaluated with magnetic resonance imaging is a strong determinant of patient outcomes in pulmonary arterial hypertension. We evaluated the prognostic value of RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change 3-6 months after initiating pulmonary arterial hypertension-specific therapy.In a prospective cohort of newly diagnosed patients with idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension, RVEF was measured at baseline (n=100) and 3-6 months after initiation of therapy (n=78). After a median follow-up of 4.1 years, 41 deaths occurred, including 35 from cardiovascular causes. Patients with a (median) baseline RVEF >25% had better survival than those with a RVEF <25% using Kaplan-Meier analysis ( p=0.010). RVEF at baseline was an independent predictor of all-cause and cardiovascular mortality in adjusted Cox regression model ( p=0.002 and p=0.007, respectively; HR 0.93 for both). Patients with stable or increased RVEF at 3-6 months had a trend for improved all-cause survival (HR 2.43, p=0.086) and had less cardiovascular mortality (HR 3.25, p=0.034) than those in whom RVEF decreased despite therapy.RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change in RVEF 3-6 months after therapy initiation independently predict outcomes in patients with pulmonary arterial hypertension. @ERSpublications RVEF assessed with CPERA at baseline, and its changes on therapy, independently predict outcome in patients with PAH http://ow.ly/DsCS4

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Section: Discussionmentioning

confidence: 86%

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

et al. 2014

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“…In general, mean right atrial and pulmonary artery pressures as well as cardiac output have been shown to be prognostic of outcome in patients with PAH. 1,7,8 In the case of evaluating our cohort for increased risk of catheterization-related complications, only mean right atrial pressure was associated with increased complication rate, whereas other hemodynamic measures were unrelated.…”

Section: Discussionmentioning

confidence: 99%

“…6 It is also a useful prognosticator that is used to help monitor treatment response and guide management decisions. 1,5,7,8 However, in a population as fragile as PAH patients, there is often hesitance to perform invasive procedures such as catheterization, especially when it includes the use of GA. Much of this hesitation comes from anecdotal reporting and higher rates of mortality and serious complications from older series in the 1980s, 17,18 as there is very little recent literature regarding the risks and complication rates of catheterization in PAH patients. Hofmann et al 11 reported their 10-year single-center experience performing pulmonary angiography in 202 mostly adult patients with PAH in 2004 and found an overall complication rate of 2.0%, although all complications were major and 3 were fatal.…”

Section: Discussionmentioning

confidence: 99%

“…3,4 Cardiac catheterization, including acute vasodilator testing, is extremely important in the management of PAH; it is used for diagnosis, prognosis, and therapy guidance as well as to perform interventions such as balloon atrial septostomy and coil embolization of aortopulmonary collateral vessels. 1,[5][6][7][8] Many centers are reluctant to perform invasive procedures in such a fragile patient population because of the potential for serious complications. However, there are scant data regarding the risk of catheterization in PAH patients.…”

Section: Introductionmentioning

confidence: 99%

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Safety of Cardiac Catheterization at a Center Specializing in the Care of Patients with Pulmonary Arterial Hypertension

et al. 2013

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Cardiac catheterization is important for the management of patients with pulmonary arterial hypertension (PAH). It is used for diagnosis, assessment, and monitoring of PAH patients, as well as to perform interventions such as balloon atrial septostomy and coil embolization of collateral vessels. Although reports on the risks of catheterization in PAH patients are scarce, many centers hesitate to perform these procedures in such fragile patients. We performed a retrospective chart review of all cardiac catheterizations performed in PAH patients over 10 years at our pulmonary hypertension center. Demographic, hemodynamic, and outcome data were collected. Complication rates were determined, and multivariate proportional hazards modeling was performed to identify predictors of catheterization-related complications. There were 1,637 catheterizations performed in 607 patients over 10 years. Pediatric patients accounted for 50% of these cases, 48% were performed in patients with idiopathic PAH, and 49% were performed under general anesthesia. While the overall complication rate was 5.7%, the rate of major complications was only 1.2% (n = 20). Although there were 8 deaths during the admission following catheterization, only 4 of these were related to the procedure, yielding a catheterization-related mortality of 0.2%. In conclusion, when performed at a pulmonary hypertension center with expertise in the care of PAH patients, cardiac catheterization is associated with low complication rates and mortality, and it should remain an important tool in the management of these patients.

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“…Patients with familial PAH or PAH in association with drug use were considered to have idiopathic PAH; this approach has been employed previously. 19 Patients with PH due to left heart disease (PH-LHD; group 2) were identified on the basis of echocardiographic and MRI assessment of LV and left atrium morphology and function, valvular sufficiency, left atrium size, and presence of LV hypertrophy in addition to PCWP. Patients with significant lung disease were subclassified accordingly as group 3.…”

Section: Clinical Subgroup Classificationmentioning

confidence: 99%

Longitudinal and Transverse Right Ventricular Function in Pulmonary Hypertension: Cardiovascular Magnetic Resonance Imaging Study from the ASPIRE Registry

et al. 2015

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Right ventricular (RV) function is a strong predictor of outcome in cardiovascular diseases. Two components of RV function, longitudinal and transverse motion, have been investigated in pulmonary hypertension (PH). However, their individual clinical significance remains uncertain. The aim of this study was to determine the factors associated with transverse and longitudinal RV motion in patients with PH. In 149 treatment-naive patients with PH and 16 patients with suspected PH found to have mean pulmonary arterial pressure of <20 mmHg, cardiovascular magnetic resonance imaging was performed within 24 hours of right heart catheterization. In patients with PH, fractional longitudinal motion (fractional tricuspid annulus to apex distance [f-TAAD]) was significantly greater than fractional transverse motion (fractional septum to free wall distance [f-SFD]; P = 0.002). In patients without PH, no significant difference between f-SFD and f-TAAD was identified (P = 0.442). Longitudinal RV motion was singularly associated with RV ejection fraction independent of age, invasive hemodynamics, and cardiac magnetic resonance measurements (P = 0.024). In contrast, transverse RV motion was independently associated with left ventricular eccentricity (P = 0.036) in addition to RV ejection fraction (P = 0.014). In conclusion, RV motion is significantly greater in the longitudinal direction in patients with PH, whereas patients without PH have equal contributions of transverse and longitudinal motion. Longitudinal RV motion is primarily associated with global RV pump function in PH. Transverse RV motion not only reflects global pump function but is independently influenced by ventricular interaction in patients with PH.Keywords: pulmonary hypertension, magnetic resonance imaging, right ventricle. Right ventricular (RV) dysfunction is considered a key determinant of adverse outcomes in several cardiopulmonary disease states. RV dilatation 1-3 and progressive reduction in RV ejection fraction (RVEF) predicts mortality in patients with pulmonary arterial hypertension (PAH). 1,3 In addition, RV dysfunction predicts mortality in patients following myocardial infarction 4,5 and in nonischemic cardiomyopathy. 6 The transverse and longitudinal components of RV function have been studied previously in patients with pulmonary hypertension (PH). 7,8 However, there are conflicting data regarding the importance of transverse and longitudinal shortening in relation to global RV function in PH. Longitudinal shortening measured using tricuspid annular plane systolic excursion (TAPSE) has been shown to be a strong predictor of adverse outcomes in patients with PH, 9,10 and it has been postulated that longitudinal motion, rather than transverse motion, is the main determinant of RVEF in both healthy subjects and patients with PH. 8 In direct contrast, Kind et al. 7 demonstrated a stronger association between transverse RV motion and RVEF than between longitudinal RV motion and RVEF in patients with PH. Moreover, Mauritz et al. 11 identi...

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New predictors of outcome in idiopathic pulmonary arterial hypertension

Cited by 223 publications

References 27 publications

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

Safety of Cardiac Catheterization at a Center Specializing in the Care of Patients with Pulmonary Arterial Hypertension

Longitudinal and Transverse Right Ventricular Function in Pulmonary Hypertension: Cardiovascular Magnetic Resonance Imaging Study from the ASPIRE Registry

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