The right ventricle, compared to the left ventricle, is in clinical practice at present not fully appreciated, though the right ventricle seems to be the crucial determinant of prognosis, exercise tolerance and life quality in a broad spectrum of diseases. The authors present an overview of rare causes of right ventricular dysfunction with right ventricle pressure overload due to chronic thromboembolic pulmonary hypertension, sclerodermia and Eisenmenger syndrome