OBJECTIVES: The goal of this study was to determine whether right ventricular (RV) ischemia is a contributory factor in the development of RV dysfunction in patients with primary pulmonary hypertension (PPH). BACKGROUND: Patients with advanced PPH develop RV dysfunction, characterized by a decreased cardiac output, increased right atrial pressure (RAP) and/or elevated RV end-diastolic pressure, which progresses to heart failure and death. The cause of this dysfunction is unknown. Right ventricular ischemia may play a role in its development. METHODS: From 1992 to 1999, a prospective study involving 23 patients with PPH at the Instituto Nacional de Cardiologia "Ignacio Chave" (Mexico City, Mexico) was undertaken. These patients were evaluated clinically and further studied by echocardiography, right heart catheterization and stress myocardial scintigraphy using technetium 99m sestamibi. RESULTS: Nine patients of 23 were found to have scintigraphic images consistent with RV ischemia. Significant correlation was found between RV ischemia obtained through myocardial perfusion scintigraphy and elevation of RV end-diastolic pressure (p<0.001), elevation of RAP (p<0.037) and a decrease in mixed venous oxygen saturation (p<0.0001). No other clinical or hemodynamic variables showed a significant correlation with RV ischemia. CONCLUSIONS: A direct correlation exists between RV ischemia, as determined by myocardial scintigraphy, and hemodynamic alterations suggestive of RV dysfunction in patients with PPH.