Right ventricular function in late-onset Pompe disease
While right ventricular (RV) function and size are important clinical markers in several cardiac conditions, the assessment of RV function by two-dimensional (2D) echocardiography remains challenging, due to the complexity of RV geometry. We therefore sought to compare an easily-measured parameter, peak systolic velocity of tricuspid annulus (TAPSV) obtained by tissue Doppler imaging (TDI), to right ventricular ejection fraction (RVEF) measured by real time three-dimensional echocardiography (RT3DE) and to explore what TAPSV cutoff values would be useful in detecting global RV dysfunction. We enrolled 20 patients affected by primary pulmonary hypertension and 30 consecutive healthy volunteers, who underwent transthoracic echocardiography, RT3DE and tissue Doppler evaluation. TAPSV had a statistically significant correlation with RVEF (r = 0.66, P < 0.001). With RV dysfunction defined as RVEF <40%, a TAPSV cutoff value of 9.5 cm/sec yielded the best compromise between sensitivity, specificity, and positive predictive value and negative predictive value. In conclusion, a TAPSV cutoff value of 9.5 cm/sec yields significantly high sensitivity and specificity and appears to be a valid compromise in detecting RV dysfunction, TAPSV values however are not useful in evaluating the severity of RV dysfunction.