To date, the usage of exercise echocardiography in patients with pulmonary or congenital heart disease has been limited despite its potential for broader applications in different clinical scenarios. Exercise echocardiography can be utilized to determine the extent of pulmonary vascular damage in patients with chronic obstructive pulmonary disease by demonstrating the presence of exertional pulmonary hypertension in subjects with normal pulmonary artery pressures (PAPs) at rest. It is also useful in patients with connective tissue disease to screen for lung involvement by identifying exertional pulmonary hypertension, and in patients with established pulmonary hypertension to choose and monitor the effects of therapeutic interventions on the PAPs. Moreover, the measurement of aortic flow velocity and acceleration by exercise echocardiography may be helpful in the study of dyspnea in patients at risk for both pulmonary disease and congestive heart failure. In patients with congenital heart disease, the measurement of PAP and ventricular function both at rest and during exercise by echocardiography is the cornerstone in the investigation of the etiology of exercise intolerance in these individuals. Lastly, exercise echocardiography can also screen for residual narrowing in patients after repair of the coarctation of the aorta by detecting a significant diastolic gradient in the descending aorta during exercise provocation.