Patients with tetralogy of Fallot (TOF) have abnormal right ventricular (RV) afterload
because of residual or recurrent outflow tract obstruction, often with abnormal pulmonary
artery (PA) vascular function. The purpose of this study was to determine if RV afterload
was independently associated with death and/or heart transplant in patients with TOF.
This is a retrospective study of TOF patients that underwent cardiac catheterization
for clinical indications at Mayo clinic between 1990 and 2015. Invasively measured
RV systolic pressure (RVSP) was used to define RV afterload. To explore clinical utility
for echocardiographic estimates of invasive data, correlations between invasive and
Doppler-derived indices of RV afterload were examined. Among 266 patients with TOF
(age 35 ± 14 years, TOF-pulmonary atresia 117 [44%]), RVSP was 72 ± 28 mm Hg, PA systolic
pressure 45 ± 19 mm Hg, mean PA pressure 27 ± 10 mm Hg, pulmonary vascular resistance
4.2 ± 3.1 WU, and PA wedge pressure 14 ± 5 mm Hg. Over a mean follow up of 12.9 years,
there were 35 deaths and 4 heart transplants. Invasively measured RVSP (hazard ratio
1.25, 95% confidence interval 1.12 to 1.37; p <0.001) and TOF-pulmonary atresia (hazard
ratio 1.18, 95% confidence interval 1.08 to 1.41; p = 0.023) were independent risk
factors for death and/or transplant. Doppler-derived RVSP was well-correlated with
invasive RVSP (r = 0.92, p <0.001), and was also independently associated with the
combined end point. RVSP, a composite measure of RV afterload, is independently prognostic
in patients with TOF, and can be reliably assessed using Doppler echocardiography.
Further study is required to test whether interventions to reduce RVSP can improve
outcomes in patients with TOF.
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Article info
Publication history
Published online: September 09, 2019
Received in revised form:
August 20,
2019
Received:
July 18,
2019
Footnotes
Funding: Dr. Egbe is supported by National Heart, Lung, and Blood Institute (NHLBI) grant K23 HL141448-01.
Identification
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© 2019 Elsevier Inc. All rights reserved.
