Noncompaction cardiomyopathy (NCM) results from an arrest of the myocardial compaction process in the embryonic period, leading to the impaired formation of the microvasculature. Predominantly left ventricle is affected, however right ventricle (RV) involvement and its functional impact in NCM remains undefined. We sought to analyze a potential correlation between myocardial ischemia and RV failure in this patient cohort. We enrolled 41 NCM patients (28 male, 13 female), aged 21 to 70 years. At baseline we performed echocardiography to measure RVID (RV inflow diastolic diameter), TAPSE (tricuspid annular plane systolic excursion), Sto (systolic tissue Doppler velocity on tricuspid annulus) and RV FAC (fractional area change). Significant myocardial ischemia was defined as summed difference score (SDS)≥2 on myocardial SPECT at rest and on stress. Myocardial ischemia has been shown in 11 patients (27%, Group A), 30 patients showed no significant ischemic changes (73%, Group B). Groups did not differ in sex (82% male vs. 63% male in Group B, P=0.454), age (48±13 years vs. 47±15 years in Group B, P=0.855), kidney function (serum creatinine 76±11µmol/L vs. 81±26µmol/L in Group B, P=0.565) or liver function (serum bilirubine 12.4±7.1µmol/L vs. 13.3±6.5µmol/L in Group B, P=0.686). When compared to Group B, Group A had significantly lower FAC (30.7±7.7% vs. 44.9±10.2%, P<0.001), greater RVID (3.3±0.6cm vs. 2.9±0.5cm, P=0.016), lower TAPSE (1.9±0.6cm vs. 2.5±0.4cm, P<0.001), higher NT-proBNP levels (1691±1883pg/mL vs. 422±877pg/mL, P=0.006). There was a trend toward lower Sto (10.7±2.6cm/s vs. 12.4±2.4cm/s, P=0.081). Overall, higher SDS was associated with lower FAC (r=-0.51, P=0.006), higher RVID (r=0.41, P=0.032), lower TAPSE (r=-0.49, P=0.008) and higher levels of NT-proBNP (r=0.66, P<0.001). In patients with NCM myocardial ischemia appears to correlate with larger RV size and worse RV function.