Echocardiographic predictors of outcome in eisenmenger syndrome.
BACKGROUND
Eisenmenger syndrome differs significantly from other types of pulmonary arterial hypertension in its physiology and prognosis. We sought to assess the relationship between the echocardiographic characteristics of patients with Eisenmenger syndrome and mortality.


RESULTS
Clinical and echocardiographic variables were assessed in 181 consecutive patients with Eisenmenger syndrome, excluding those with complex congenital heart disease. Patients' mean age was 39.1 ± 12.8 years, 59 (32.6%) were male, 122 (67.4%) were in functional class III or higher, and 74 (40.9%) were on advanced therapies. Mean oxygen saturation at rest was 85.1 ± 7.8%, and median B-type natriuretic peptide was 55.4 ng/L. Over a median follow-up of 16.4 months, 19 patients died; the strongest predictors of mortality were tricuspid annular plane systolic excursion and peak systolic velocity, myocardial performance (expressed as total isovolumic time and ratio of systolic to diastolic duration), and elevated central venous pressure (expressed as right atrial [RA] area, RA pressure, and ratio of RA to left atrial area), even after we accounted for advanced therapies. A composite score based on the strongest echocardiographic predictors of outcome, including 1 point for each of the following: tricuspid annular plane systolic excursion <15 mm, ratio of right ventricular effective systolic to diastolic duration ≥ 1.5, RA area ≥ 25 cm², ratio of RA to left atrial area ≥ 1.5, was highly predictive of clinical outcome (area under the curve 0.90 ± 0.01), with no improvement when B-type natriuretic peptide and resting saturations were added into the model.


CONCLUSIONS
Echocardiographic parameters of right ventricular function and RA area predict mortality in Eisenmenger patients. A new composite echocardiographic score, described herewith, may be incorporated into the noninvasive, periodic assessment of these patients.
Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom. moceri.p@chu-nice.fr
Pamela
P
Moceri
Konstantinos
K
Dimopoulos
Emmanouil
E
Liodakis
Ioannis
I
Germanakis
Aleksander
A
Kempny
Gerhard-Paul
GP
Diller
Lorna
L
Swan
Stephen J
SJ
Wort
Philip S
PS
Marino
Michael A
MA
Gatzoulis
Wei
W
Li
D000328:Adult; D016280:Atrial Function, Right / Q000502:physiology; D016208:Databases, Factual; D003971:Diastole / Q000502:physiology; D004452:Echocardiography / Q000379:methods*; D004541:Eisenmenger Complex / Q000000981:diagnostic imaging* / Q000401:mortality*; D005260:Female; D005500:Follow-Up Studies; D006325:Heart Atria / Q000000981:diagnostic imaging; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000401:mortality; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012307:Risk Factors; D013599:Systole / Q000502:physiology; D014261:Tricuspid Valve / Q000000981:diagnostic imaging; D016278:Ventricular Function, Right / Q000502:physiology