Key result
Eisenmenger syndrome was associated with preserved short-axis right ventricular function compared to pulmonary arterial hypertension (median fractional shortening 21% vs 14%, P=0.002).
Why the study?
Does quantitative echocardiography reveal differences in right ventricular adaptation between patients with Eisenmenger syndrome and pulmonary arterial hypertension?
Cross-Sectional (n=73)
Does quantitative echocardiography reveal differences in right ventricular adaptation between patients with Eisenmenger syndrome and pulmonary arterial hypertension?
Absolute Event Rate: 21% vs 14%
p-value: p=0.002
Patients with Eisenmenger syndrome have preserved short-axis right ventricular function compared to those with PAH, which may explain their more favorable outcomes and is not detected by conventional echocardiographic measures.
Supports differential RV adaptation in Eisenmenger syndrome; leaves open prospective validation for risk stratification.
BACKGROUND: The favorable outcomes of Eisenmenger syndrome (ES) relative to other forms of pulmonary arterial hypertension (PAH) have been partially attributed to a unique adaptation of the right ventricle (RV). However, conventional measures of RV function may not adequately express this adaptation. METHODS: We studied 23 patients with ES (age 43 ± 17 years, 16 women, pulmonary artery systolic pressure [PASP] 93 ± 26 mmHg), 25 patients with PAH (age 44 ± 13 years, 17 women, PASP 92 ± 19 mmHg), and 25 subjects without known structural disease (age 45 ± 16 years, 17 women). We evaluated long- and short-axis function of the RV with two-dimensional strain and anatomical M-mode echocardiography, respectively. RESULTS: Long-axis function of the RV was comparable between patients with ES and PAH although depressed relative to controls (global strain, -15.6 ± 4.7, -14.9 ± 4.3, and -22.4 ± 2.8%, respectively, P < 0.001; global RV systolic strain rate, -0.77 ± 0.26, -0.84 ± 0.24, and -1.11 ± 0.21 1/sec, respectively, P < 0.001). However, short-axis RV function was significantly better in patients with ES versus those with PAH and preserved relative to controls (RV fractional shortening by anatomical M-mode, median [interquartile range], 21%[14-33%], 14%[10-16%], and 26%[22-36%], respectively, P = 0.002 for ES vs. PAH, P = 0.09 for ES vs. controls). This differential was not reflected in conventional measures of RV function (fractional area change, 32 ± 10 vs. 29 ± 8% in ES and PAH, respectively, P = 0.26). CONCLUSION: In patients with ES, the RV is characterized by preserved short-axis function, despite a depressed long-axis function. Thus, conventional assessment of RV function might not be suitable for patients with ES.
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Kalogeropoulos et al. (2010) conducted a cross-sectional in Eisenmenger syndrome and pulmonary arterial hypertension (n=73). Eisenmenger syndrome vs. Pulmonary arterial hypertension was evaluated on Short-axis right ventricular function (fractional shortening) (p=0.002). Eisenmenger syndrome was associated with preserved short-axis right ventricular function compared to pulmonary arterial hypertension (median fractional shortening 21% vs 14%, P=0.002).
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