Use of myocardial performance index in pediatric patients with idiopathic pulmonary arterial hypertension.
BACKGROUND
The myocardial performance index (MPI) correlates with clinical status in adults with idiopathic pulmonary arterial (PA) hypertension (IPAH). This pediatric study used MPI to assess response to bosentan therapy.


METHODS
The study included 12 children with IPAH and 12 healthy control subjects. MPI was correlated with catheterization data at initiation of bosentan and at a median follow-up of 9 months. Therapy responders were defined by a greater than 20% decrease in mean PA pressure.


RESULTS
Right ventricular MPI for patients with IPAH was 0.64 +/- 0.30 versus 0.28 +/- 0.03 in control subjects (P < .01). It had a strong correlation with mean PA pressure (R = 0.94; P < .001). Right ventricular MPI decreased significantly in responders (range 20%-44%, mean 25%) with a 5% increase in nonresponders.


CONCLUSIONS
Right ventricular MPI in pediatric IPAH correlates with mean PA pressure and response to therapy. This study suggests that this noninvasive Doppler index may be useful to follow up children with IPAH, particularly when tricuspid regurgitation data are insufficient.
Dyer
Karrie L
KL
University of Colorado Health Sciences Center--The Children's Hospital, Denver, Colorado, USA. dyer.karrie@tchden.org
Pauliks
Linda B
LB
Das
Bibhuti
B
Shandas
Robin
R
Ivy
Dunbar
D
Shaffer
Elizabeth M
EM
Valdes-Cruz
Lilliam M
LM
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler / Q000379:methods*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000000981:diagnostic imaging*; D007090:Image Interpretation, Computer-Assisted / Q000379:methods*; D008297:Male; D010372:Pediatrics / Q000379:methods; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging* / Q000209:etiology