Rationale: Transthoracic Doppler echocardiography is recommended for screening for the presence of pulmonary hypertension (PH). However, some recent studies have suggested that Doppler echocardiographic pulmonary artery pressure estimates may frequently be inaccurate. Objectives: Evaluate the accuracy of Doppler echocardiography for estimating pulmonary artery pressure and cardiac output. Methods: We conducted a prospective study on patients with various forms of PH who underwent comprehensive Doppler echocardiography within 1 hour of a clinically indicated right-heart catheterization to compare noninvasive hemodynamic estimates with invasively measured values. Measurements and Main Results: A total of 65 patients completed the study protocol. Using Bland-Altman analytic methods, the bias for the echocardiographic estimates of the pulmonary artery systolic pressure was 20.6 mm Hg with 95% limits of agreement ranging from 138.8 to 240.0 mm Hg. Doppler echocardiography was inaccurate (defined as being greater than 610 mm Hg of the invasive measurement) in 48% of cases. Overestimation and underestimation of pulmonary artery systolic pressure by Doppler echocardiography occurred with a similar frequency (16 vs. 15 instances, respectively). The magnitude of pressure underestimation was greater than overestimation (230 6 16 vs. 119 6 11 mm Hg; P 5 0.03); underestimates by Doppler also led more often to misclassification of the severity of the PH. For cardiac output measurement, the bias was 20.1 L/min with 95% limits of agreement ranging from 12.2 to 22.4 L/min. Conclusions: Doppler echocardiography may frequently be inaccurate in estimating pulmonary artery pressure and cardiac output in patients being evaluated for PH.Keywords: echocardiography; pulmonary hypertension; pulmonary systolic pressure; cardiac output; accuracy Pulmonary hypertension (PH), a syndrome characterized by increased pulmonary vascular resistance and remodeling, is associated with significant morbidity and mortality, which are directly related to cardiac function (1). Although the definitive diagnosis of PH is currently established through right-heart catheterization, accurate noninvasive assessment of pulmonary arterial pressure and cardiac output (CO) is desirable both for diagnostic purposes and to assess response to therapy.Transthoracic Doppler echocardiography (DE) is recommended as the initial noninvasive modality in the screening and evaluation of PH (2). Echocardiography can be used to evaluate right-sided chamber size and function and the presence of pericardial effusion, which are known to impact survival (3-5). Frequently, DE is used to estimate the right ventricular systolic pressure by estimating the pressure gradient between the right ventricle and the right atrium using the modified Bernoulli equation, 4v 2 , where v equals the velocity of the tricuspid regurgitant jet. An estimated right atrial pressure is added to this number to approximate the right ventricular systolic pressure, which equals the pulmonary artery systolic pres...

TAPSE powerfully reflects RV function and prognosis in PAH.

Objective. Pulmonary arterial hypertension related to scleroderma (PAH-Scl) is associated with high morbidity and mortality as well as poorer response to therapy and worse outcomes compared with the idiopathic form of PAH (IPAH). Scleroderma is an autoimmune disease that can affect left and right heart function directly through inflammation and fibrosis and indirectly through systemic and pulmonary hypertension. This study tested the hypothesis that an increased prevalence of left heart disease might explain the higher mortality in patients with PAH-Scl compared with patients with IPAH.Methods. The study was designed as a retrospective cohort study comparing the baseline clinical data from 91 consecutive patients (41 with IPAH and 50 with PAH-Scl). Cox proportional hazards models were used to predict the effect of clinical covariates on patient survival.Results. Patients with PAH-Scl had a lower mean pulmonary artery pressure (46.6 mm Hg versus 54.4 mm Hg in patients with IPAH; P ‫؍‬ 0.002) despite similar levels of cardiac dysfunction (cardiac index 2.2 and 2.1 liters/minute/m 2 , respectively; P ‫؍‬ 0.19). Echocardiography revealed similar degrees of right ventricular dysfunction in the 2 groups, whereas a predominance of left heart dysfunction was observed in patients with PAH-Scl. One-and three-year survival estimates were 87.8% and 48.9%, respectively, in patients with PAH-Scl and 95.1% and 83.6%, respectively, in those with IPAH. Patients with PAH-Scl were 3.06 times more likely to die than were patients with IPAH, after controlling for the presence of pericardial effusion; there was no significant change in increased risk of death in PAH-Scl after controlling for left heart disease.Conclusion. The results confirm that there are significant clinical and survival differences between IPAH and PAH-Scl. The presence of left heart disease, although more common in PAH-Scl, was not predictive of the higher mortality in these patients.