Faculty Collaboration Database

Active right atrial emptying fraction predicts reduced survival and increased adverse events in childhood pulmonary arterial hypertension. Int J Cardiol 2018 Nov 15;271:306-311

Date

09/19/2018

Pubmed ID

30223361

DOI

10.1016/j.ijcard.2018.04.125

Scopus ID

2-s2.0-85046660223 (requires institutional sign-in at Scopus site)   10 Citations

Abstract

BACKGROUND: Right atrial (RA) function has been studied rarely in childhood pulmonary arterial hypertension (PAH). We sought to determine if RA and right ventricular (RV) area changes measured by echocardiography predicted outcomes.

METHODS: We reviewed data from children with PAH undergoing cardiac catheterization and echocardiography. RA and RV areas were obtained from the apical 4-chamber view. Clinical worsening indicated initiation of parenteral prostanoid therapy, heart and/or lung transplantation, Potts shunt surgery or death.

RESULTS: We studied 57 children (27 females), median age 3 years (range 0.30-17 years), body surface area 0.56 m2 (0.2-1.8), follow up 3 years (0.21-8.35), time to clinical worsening was 1.14 years (0.03-6.14) and mortality was 1.55 years (range 0.88-4.95). We determined from receiver operator curves that RA active emptying fraction (RA EaF) ≥60% predicted clinical worsening (sensitivity 78%, specificity 69%, AUC 0.7) and mortality (sensitivity 100%, specificity 65%, AUC 0.82). RV fractional area change (RVFAC) <25% predicted clinical worsening (sensitivity 72%, specificity 79%, AUC 0.85) and death (sensitivity 67%, specificity 69%, AUC 0.77). The combination of RA EaF ≥60% and RVFAC <33% were best predictors of clinical worsening (sensitivity 72%, specificity 82%, partial AUC 0.65) and mortality (sensitivity 100%, specificity 77%, partial AUC 0.75).

CONCLUSION: In childhood PAH, RA EaF ≥ 60% and RVFAC <25% were associated with poor outcomes. RA EaF ≥60% and RVFAC <33% were best predictors of clinical worsening and may be useful markers in children with PAH who require closer observation and more intensive therapy.

Author List

Kumar S, Vadlamudi K, Kaddoura T, Bobhate P, Goot BH, Elgendi M, Jain S, Colen T, Khoo NS, Adatia I

Author

Ben Goot MD Associate Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Atrial Function, Right
Cardiac Catheterization
Child
Child, Preschool
Female
Humans
Hypertension, Pulmonary
Infant
Male
Predictive Value of Tests
Retrospective Studies
Survival Rate