|| Checking for direct PDF access through Ovid
Background: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension related to congenital heart disease. Several studies have suggested that the location of the intracardiac shunt causes differences in natural course and circulatory physiology between patients with ES. We therefore aimed to compare echocardiographic parameters in various types of ES.Methods: We studied 186 patients with ES, 27 with pre-tricuspid and 151 with post-tricuspid shunt (72 VSD, 56 complete AVSD, 17 PDA, 4 truncus arteriosus, 2 aorto-pulmonary window). We excluded 8 patients with systemic right ventricle (RV), double outlet RV and double inlet left ventricle.Results: RV dilatation is prevalent in pre-tricuspid ES (mean RV inlet diameter 49.1mm ± 8.7) compared to post-tricuspid shunts (43.3mm ± 6.9, p = 0.003) despite no significant difference in peak tricuspid regurgitation gradient or pulmonary valve acceleration time. However, RV dilatation is also common in post-tricuspid ES,with 49.7% having an RV inlet>42mm. Right atrial area is also larger in pre-tricuspid shunts (24.3±8.6 cm2 versus 19.8±5.2cm2, p = 0.03) whereas,within post-tricuspid shunts, VSDs have a higher RA area. Pretricuspid shunts have a trend for a higher end-systolic eccentricity index (2.14 ± 0.8 vs1.87 ± 0.6, p = 0.08). No significant difference was found in peak tricuspid TDI S-wave (11.1±4.3 vs 9.9±2.8, p=ns),TAPSE (18.0± 5.0 vs 17.8 ± 5.2, p=ns) and fractional area change (41 ± 11% vs 33 ± 10,5 %, p = ns) between pre and post-tricuspid ES. Tissue Doppler myocardial performance index is abnormal overall (0.68 ± 0.30), but was not different between subgroups. TDI-derived isovolumic myocardial acceleration is higher in the pre-tricuspid group (4,4±1.6 cm/s2 vs 2,4±1.0cm/s2,p = 0.01), even when corrected for the heart rate (IVA/√RR), suggesting that early RV dysfunction was more frequent in patients with post-tricuspid shunt, despite no difference in other functional parameters.Conclusion: RV adaptation differs between types of ES, but RV dilatation with near-normal RV systolic function is common and likely accounts for the better survival of ES compared to other types of pulmonary hypertension.