Correlation Between Right Ventricular Echocardiography Measurements and Functional Capacity in Patients With Pulmonary Arterial Hypertension.

BACKGROUND: Accelerometry is an emerging option for real-time evaluation of functional capacity in patients with pulmonary arterial hypertension (PAH). This prospective pilot study assesses the relationship between functional capacity by accelerometry and right ventricular measurements on echocardiography for this high-risk cohort. METHODS: Patients with PAH were prospectively enrolled and underwent 6-Minute Walk Test and cardiopulmonary exercise testing. They were given a Fitbit, which collected steps and sedentary time per day. Echocardiographic data included right ventricular global longitudinal, free wall, and septal strain; tricuspid regurgitant peak velocity; tricuspid annular plane systolic excursion; tricuspid annular plane systolic velocity; right ventricular myocardial performance index; and pulmonary artery acceleration time. Pairwise correlations were performed. RESULTS: The final analysis included 22 patients aged 13 to 59 years. Tricuspid regurgitant peak velocity had a negative correlation with 6-Minute Walk Test (r = -0.58, P = .02), peak oxygen consumption on exercise testing (r = -0.56, P = .03), and average daily steps on accelerometry (r = -0.59, P = .03), but a positive correlation with median sedentary time on accelerometry (r = 0.64, P = .02). Pulmonary artery acceleration time positively correlated with peak oxygen consumption on exercise testing (r = 0.64, P = .002). There was no correlation between right ventricular strain measurements and functional capacity testing. CONCLUSION: In this pilot study, tricuspid regurgitant jet and pulmonary artery acceleration time were the echocardiographic variables that correlated most with accelerometry data. With further echocardiographic validation, accelerometry can be a useful, noninvasive, and cost-effective tool to monitor disease progression in patients with PAH.

Pulmonary artery thromboembolism in a critically ill neonate successfully treated using thrombolytic therapy.

Pulmonary artery thromboembolism is a common and treatable cause of adult pulmonary hypertension. Although rare in children, if undiagnosed, it can result in significant morbidity and mortality. We report a case of a cyanotic neonate found to have bilateral pulmonary arterial thrombi who successfully underwent thrombolysis using tissue plasminogen activator with prompt resolution of right ventricular hypertension.