Background: Compared to primary pulmonary hypertension (PPH), the right ventricular (RV) contractile function is preserved for a long time in patients with Eisenmenger syndrome and is likely the most important determinant of relatively higher survival. The differences in myocardial perfusion have been purported to explain this discrepancy. The exact prevalence of myocardial perfusion abnormalities in Eisenmenger syndrome is not known. We sought to examine the prevalence of myocardial perfusion abnormalities in patients with Eisenmenger syndrome.
Methods: In this prospective study, 20 consecutive adult patients with Eisenmenger syndrome were subjected to clinical assessment, six-minute walk test and echocardiography. Myocardial perfusion was assessed using one day stress-rest Gated Technetium-99 m Sestamibi single-photon emission computed tomography.
Results: Nineteen (95%) patients were in New York Heart Association functional class I or II. All patients had RV hypertrophy. Five (25%) patients had RV systolic dysfunction. Left ventricular systolic function was normal in all except in three patients. Two (10%) patients had perfusion defects in the RV and 4 (20%) patients had perfusion defects in the left ventricle (LV).
Conclusion: Myocardial perfusion defects, both in RV and LV, occur even in asymptomatic or mildly symptomatic patients with Eisenmenger syndrome.