The importance of the right ventricle (RV) in pulmonary arterial hypertension (PAH) has been gaining increased recognition. This has included a reconceptualization of the RV as part of an RV-pulmonary circulation interrelated unit and the observation that RV function is a major determinant of prognosis in PAH. Noninvasive imaging of RV size and function is critical to the longitudinal management of patients with PAH, and continued understanding of the pathophysiology of pulmonary vascular disease relies on the response of the RV to pulmonary vascular remodeling. Echocardiography, in particular the newer echocardiographic measurements and techniques, allows easy, readily accessible means to assess and follow RV size and function.Keywords: imaging, three-dimensional echocardiography, tricuspid annular plane systolic excursion, right heart failure, right ventricular function. Pulmonary arterial hypertension (PAH) is characterized by severe remodeling of distal pulmonary arterioles due to a complex interplay between genetic and molecular factors.1,2 This remodeling is characterized by intimal hyperplasia, vasoconstriction, medial hypertrophy, and the development of plexiform lesions, all of which contribute to and result in higher pulmonary artery pressure. The prevalence of PAH is 7-15 individuals per million people. 3,4 A diagnosis of PAH is initially suggested by symptoms including dyspnea, syncope, and exertional intolerance and is usually evaluated first with echocardiography. 5 Echocardiography may suggest elevated pulmonary artery pressures (PAPs) and help formulate a working hypothesis regarding the etiology of the presumed pulmonary hypertension (PH), but right heart catheterization remains essential to provide final hemodynamic classification of PH and, with that knowledge in hand, to guide appropriate World Health Organization (WHO) PH group-specific therapy. 6 Once the diagnosis of PAH is established (mean PAP ≥ 25 mmHg and pulmonary capillary wedge pressure ≤ 15 mmHg), most clinicians rely on a combination of frequent clinical evaluations and echocardiography to follow therapeutic response and to give insight into the effects of elevated PAP on the structure and function of the right ventricle (RV). The right ventricle's adaptation or maladaptation to the increased afterload is often a sign of the severity of PH.
7The relevance of the relationship between the RV and the pulmonary circulation in PAH has been gaining increased recognition.8 As a pump, the RV generates the same stroke volume as the left ventricle (LV) with onefourth the stroke work because of the lower resistance of the normal pulmonary vasculature. 9 The RV is thin walled, with the free wall measuring 2-5 mm, and contains onesixth the muscle mass of the LV. 9 It is a crescent-shaped chamber with a high capacitance and a greater ability to handle changes in preload than in afterload. When chronically exposed to increased afterload, the RV can adapt with myocardial hypertrophy, since increase in wall pressure leads to increase in ...