Elizabeth M Shaffer | PubMed Health

PubMed2006

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.

Adolescent↗

PubMed2003

Hepatic dysfunction following the Fontan procedure.

OBJECTIVES: The Fontan procedure offers a palliation for the hemodynamic derangements associated with congenital heart lesions characterized by a single functional ventricle, but it causes a chronically elevated systemic venous pressure that may result in hepatic congestion. The objective of this study was to characterize hepatic function and its relationship to cardiac function in children who had undergone the Fontan procedure. METHODS: In a cross-sectional study of 11 children aged 38 months to 216 months (mean, 149 months), the authors evaluated indices of cardiac and hepatic function, including galactose clearance, Doppler hepatic ultrasonography, synthetic function, and markers of liver injury, at 9 months to 176 months (mean, 100 months) after children had undergone the Fontan procedure. RESULTS: The most common biochemical abnormality of hepatic function was a prolongation of the prothrombin time and a low factor V level. There was a trend toward progressive abnormality in prothrombin time with increasing interval since the Fontan procedure. Galactose elimination half-life and galactose elimination capacity were inversely correlated with the time after Fontan (R2= 0.65, P = 0.004). There was no relationship between cardiac functional measurements and liver function. CONCLUSIONS: Prothrombin time and galactose elimination half-life are abnormal in children who have undergone the Fontan procedure and may be useful markers of hepatic function in the longitudinal assessment of these patients.

Adolescent↗