@Article{chd.12661,
AUTHOR = {Keri M. Shafer, Alexander R. Opotowsky, Jonathan Rhodes},
TITLE = {Exercise testing and spirometry as predictors of mortality in congenital heart disease: Contrasting Fontan physiology with repaired tetralogy of Fallot},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {13},
YEAR = {2018},
NUMBER = {6},
PAGES = {903--910},
URL = {http://www.techscience.com/schd/v13n6/39074},
ISSN = {3071-1738},
ABSTRACT = {<b>Objective:</b> Risk prediction using cardiopulmonary exercise testing (CPET) in complex
congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single
institution over the same time period to determine CPET variables associated with
mortality.<br/>
<b>Design:</b> All Fontan and tetralogy of Fallot (TOF) subjects with CPET between
November 1, 2002 and December 31, 2014 and subsequently died were identified
(cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and
timing of exercise test.<br/>
<b>Results:</b> Of the 42 cases, 27 had a Fontan circulation and 15 with TOF. All Fontan
patients had a low peak VO<sub>2</sub> but there was no significant difference between cases
and controls (52.5 ± 14.7 v. 57.4% ± 13.5% predicted, P = .11). Spirometry values
were significantly lower in Fontan cases than controls (eg, FVC 67.4 ± 19.1 v
77.6% ± 14.9% predicted, P = .007). Spirometry values were also lower in TOF cases
than controls (% predicted FVC 62.8 ± 16.7 v 75 ± 14, P = .006). In contrast to the
Fontan analysis, both %peak predicted VO<sub>2</sub> and VE/VCO<sub>2</sub> slope were worse in TOF
cases than controls (50.1 ± 13.5 v. 68.5% ± 15.0% predicted VO<sub>2</sub>, P = .0004;
33.9 ± 12.9 v 26.6 ± 4.4, P = .002). Multivariable analysis also identified different
predictors of mortality among the anatomic subgroups. Spirometric data (FVC) correlated most strongly with mortality in Fontan patients while the VE/VCO<sub>2</sub> slope was
most associated with outcome in TOF patients.<br/>
<b>Conclusions:</b> Variables most predictive of mortality in Fontan and TOF patients diverge but spirometry was abnormal and associated with mortality in both groups.
When compared with age‐matched controls, reduced FEV<sub>1</sub> and FVC correlated most
strongly with mortality in Fontan patients while VE/VCO<sub>2</sub> slope correlated with mortality for TOF patients. These findings further support the importance of lung health
in patients with complex congenital heart disease.},
DOI = {10.1111/chd.12661}
}