Abstract
Background: Pulmonary hypertension is a significant yet rare
disease that can have many long-term consequences, including death.
Cardiac catheterization is the gold standard for measuring pulmonary
artery mean pressures (PAMP), but is invasive and risks potentially
serious complications. This study aimed to create a semi-quantitative,
non-invasive echocardiographic measure of PAMP using septal positioning.
Methods: This study was a retrospective study of patients with
and without pulmonary hypertension who had a transthoracic
echocardiogram and cardiac catheterization. Patients undergoing atrial
septal defect closure represented controls. Two blinded readers
calculated the “Echocardiographically-Derived Septal Positional Angle
(EDSPA)” which was compared to corresponding catheterization data
including mean pulmonary artery pressures.
Results: A total of 159 children were included, of which 150
had useable echocardiographic data. 39 children were identified as
having pulmonary hypertension while 111 children had an atrial septal
defect. Patient age ranged from a minimum of 54 days and maximum of 19
years [mean 7.1 years (SD=5.30)]. Inter-observer variability between
two readers [Pearson correlation coefficient of 0.939 (p
<0.001)] and intra-observer variability were low
[intraclass correlation coefficient (ICC) of 0.95 and 0.96 for each
observer respectively]. An EDSPA of ≤39° predicted a
PAMP>20 mmHg (as measured by cardiac catheterization) with
a 76% sensitivity and 76% specificity (AUC 0.846).
Conclusions: EDSPA is a useful, non-invasive, and reproducible
echocardiographic measure of PAMP that is easy to perform. With a
sensitivity and specificity near 80%, it has significant utility in
screening for pulmonary hypertension and determining which patients
should undergo further invasive diagnostic testing.
Key Words
Pediatric cardiology, pulmonary hypertension, echocardiography, cardiac
catheterization