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Background The presence and size of a pericardial effusion in pulmonary arterial hypertension (PAH) and its association with outcome is unclear. Methods In this single-center cohort study of 577 patients with group 1 PAH seen between January 1, 1995, and December 31, 2005, all patients underwent transthoracic echocardiography and were followed for ≥ 5 years. Echocardiography-guided pericardiocentesis was performed as needed. Results Pericardial effusions on index echocardiography occurred in 150 patients (26%); 128 patients had small and 22 had moderate-sized or larger effusions. Most of the moderate or greater effusions occurred in patients who had connective tissue disease (82%). Mean right atrial pressure was 13.4 ± 4.4 mm Hg (no effusion), 15.1 ± 4.4 mm Hg (small effusion), and 17.0 ± 4.0 mm Hg (moderate or greater effusion) ( P Conclusions Pericardial effusions are relatively common but rarely of hemodynamic significance in patients with PAH. However, even modest degrees of pericardial fluid are associated with a significant increase in mortality and appear to reflect the presence of associated collagen vascular disease and high right atrial pressure.

Background Pulmonary arterial hypertension (PAH) is a devastating illness of pulmonary vascular remodeling, right-sided heart failure, and limited survival. Whether strain-based measures of right ventricular (RV) systolic function predict future right-sided heart failure and/or death is untested. Methods RV longitudinal systolic strain and strain rate were evaluated by echocardiography in 80 patients with World Health Organization group 1 pulmonary hypertension (PH) (72% were functional class [FC] III or IV). Survival status was assessed over 4 years. Results All patients had a depressed RV systolic strain (−15% ± 5%) and strain rate (−0.80 ± 0.29 s−1). Of the parameters assessed, average RV free wall systolic strain worse than −12.5% identified a cohort with greater severity of disease (82% were FC III/IV), greater RV systolic dysfunction (RV stroke volume index 26 ± 9 mL/m2), and higher right atrial pressure (12 ± 5 mm Hg). Patients with an RV free wall strain worse than −12.5% were associated with a greater degree of disease progression within 6 months, a greater requirement for loop diuretics, and/or a greater degree of lower extremity edema, and it also predicted 1-, 2-, 3-, and 4-year mortality (unadjusted 1-year hazard ratio, 6.2; 2.1–22.3). After adjusting for age, sex, PH cause, and FC, patients had a 2.9-fold higher rate of death per 5% absolute decline in RV free wall strain at 1 year. Conclusions Noninvasive assessment of RV longitudinal systolic strain and strain rate independently predicts future right-sided heart failure, clinical deterioration, and mortality in patients with PAH.

The presence and size of a pericardial effusion in pulmonary arterial hypertension (PAH) and its association with outcome is unclear.In this single-center cohort study of 577 patients with group 1 PAH seen between January 1, 1995, and December 31, 2005, all patients underwent transthoracic echocardiography and were followed for ≥ 5 years. Echocardiography-guided pericardiocentesis was performed as needed.Pericardial effusions on index echocardiography occurred in 150 patients (26%); 128 patients had small and 22 had moderate-sized or larger effusions. Most of the moderate or greater effusions occurred in patients who had connective tissue disease (82%). Mean right atrial pressure was 13.4 ± 4.4 mm Hg (no effusion), 15.1 ± 4.4 mm Hg (small effusion), and 17.0 ± 4.0 mm Hg (moderate or greater effusion) (Plt; .0001). Median survival for patients with moderate or greater effusion, mild effusion, or no effusion was 11.3 months, 42.3 months, and 76.5 months, respectively. Four of the 22 patients with moderate or greater pericardial effusions eventually required echocardiography-guided pericardiocentesis because of clinical and echocardiographic evidence of hemodynamic impact. When drained, the effusions were large (858 ± 469 mL) and generally serous. All pericardiocenteses were performed cautiously under echocardiographic guidance by a highly experienced echocardiologist, with low immediate morbidity and mortality.Pericardial effusions are relatively common but rarely of hemodynamic significance in patients with PAH. However, even modest degrees of pericardial fluid are associated with a significant increase in mortality and appear to reflect the presence of associated collagen vascular disease and high right atrial pressure.