PURPOSE: Eisenmenger syndrome (ES) represents a specific group of severe irreversible pulmonary arterial hypertension due to congenital heart defect. Typical is right ventricular (RV) adaptation to volume-pressure overload with long-term RV systolic function preservation. The aim of the study was to investigate specific features of RV in ES.
METHODS: Analyzed were 47 patients (34F/13M, mean age 34.6±17.2 years) with invasively confirmed ES. Echocardiographical study was performed, measuring RV hypertrophy, dilatation and function and compared to age-sex matched healthy controls.
RESULTS: Post-tricuspid defects (post-TD) were present in 39 (83%), pre-tricuspid (pre-TD) in 8 patients (17%). 1. Compared to controls, in ES significant RV hypertrophy was present (RV anterior wall (RVAW), RV mass, p<0.001), as well as RV dilatation (RV diastolic diameter (RVD), RV/LV ratio, p<0.001, RV end-diastolic area (EDA), p=0.006). RV fractional area change (FAC) was also significantly lower (p=0.03), although RV FAC<40% was only in 10.5%. 2. Comparison of pre-TD vs post-TD groups showed: a) dominant RV dilatation - RVD (mm) 48 vs 26 (p<0.001), RV/LV ratio 1.5 vs 0.9 (p=0.02), RV mass/chamber ratio 1.65 vs 0.65 (p<0.002), b) significant RV FAC(%) difference - 41.4 vs 60.6 (p=0.004), c) no statistical difference in RV hypertrophy - RVAW, p=0.06, RV mass, p=0.5.
CONCLUSIONS: RV in ES is significantly dilated and hypertrofied with slightly reduced systolic function and substantially expressed ventricular interdependence. In post-TD RV hypertrophy is more expressed; on the contrary, in pre-TD dominant severe RV dilatation with often decreased RV systolic function is present.
CLINICAL IMPLICATIONS: Specific features that are protecting RV from failure in ES are: 1. Presence of “pop-off valve” (defect), allowing relief of RV pressure-volume overload. 2. Massive RV hypertrophy, the adaptation mechanism with positive influence on RV contractility, found as a typical feature of ES heart, regardless of defect type. 3. Important impact on the RV failure must also have the defect location, as shown in pre-TD subgroup, manifesting by a greater degree of RV dilatation and dysfunction.
DISCLOSURE: The following authors have nothing to disclose: Iveta Simkova, Monika Kaldararova
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