BackgroundTo investigate the number of patients with congenital thrombophilia who presented an intracardiac thrombosis.MethodsPersonal files were reevaluated together with a time-unlimited search of the literature.ResultsTwenty-five patients with intracardiac thrombosis and congenital thrombophilia have been gathered from the literature including the two personal cases. The distribution observed in thrombophilia patients is similar for left side or right side heart (9 vs 11 cases). The left ventricle and the right ventricle were involved in six or five instances, respectively. In one case, both ventricles were involved. On the contrary, the left atrium was involved in three cases whereas the right atrium was affected in six cases. In the remaining cases, more than one heart chamber was involved.ConclusionsIn “normal” subjects, left side thromboses are predominant once catheter-associated thrombi are excluded. The reason of this discrepancy lies in the greater prothrombotic effect exercised by congenital thrombophilia on venous thrombosis compared to arterial thrombosis. The relative high prevalence of cardiac thrombosis seen in patients with antithrombin and protein C deficiencies indicated that a cardiac evaluation should be carried out in all patients with these two defects.