Echocardiographic Indexes for the Non-Invasive Evaluation of Pulmonary Hemodynamics

Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics

“…Obtaining the tricuspid regurgitation jet by echocardiogram is the best method to periodically screen for elevated right-side heart pressures. 163 Because the reported cases of GSD I patients with PAH were mostly children older than 10 years of age, screening by echocardiography can likely be delayed until patients are 10 years of age or older. 33,84 Periodic screening echocardiograms could be performed every 3 years or at shorter intervals if there are any suggestive clinical symptoms.…”

“…To date, nine GSD I patients with PAH have been reported. 49,50,[157][158][159][160][161][162][163] Of these, five had concomitant conditions that were also associated with the development of PAH, including three patients with portocaval shunts, one patient with an atrial septal defect, and one patient with hereditary hemorrhagic telangiectasia. 33,49,84,[157][158][159]161 It has been suggested that abnormal handling of serotonin might be one event in a multistep process leading to PAH in GSD I patients.…”

A new method to estimate pulmonary vascular resistance using diastolic pulmonary artery-right ventricular pressure gradients derived from continuous-wave Doppler velocity measurements of pulmonary regurgitation

“…It has been reported that the early-diastolic pressure gradient derived from the peak early-diastolic PR velocity is useful for estimating the mean PA pressure [7][8][9][10], whereas the end-diastolic pressure gradient calculated from the end-diastolic PR velocity reflects end-diastolic PA pressure [7,9]. It was also reported that the end-diastolic PA pressure measured by Doppler echocardiography correlated well with invasively measured PA wedge pressure and might be used as its surrogate [10].…”

“…However, the invasive nature of catheterization makes it difficult or impossible to perform daily or repetitive measurements of PVR. Although several noninvasive methods to estimate PVR based on echocardiographic measurements have been proposed [2][3][4][5][6][7], these methods used various empirical constants and/or ignored the left ventricular filling pressure, and thus their accuracy may be limited. The guidelines of the American Society of Echocardiography recommended not to use noninvasive estimations of PVR as a substitute for the invasive evaluation of PVR [8].…”

“…The guidelines of the American Society of Echocardiography recommended not to use noninvasive estimations of PVR as a substitute for the invasive evaluation of PVR [8]. It has been reported that the early-diastolic and end-diastolic pressure gradients between the PA and the right ventricle (RV) derived from pulmonary regurgitation (PR) velocities measured by using continuous-wave Doppler echocardiography can reflect the mean PA pressure and the PA wedge pressure, respectively [7][8][9][10]. We therefore postulated that, by using these parameters, we could establish a simpler and more accurate noninvasive method to estimate PVR.…”