Echocardiographic features of primary pulmonary hypertension

Primary pulmonary hypertension (PPH) is essentially a diagnosis of exclusio n and usually is made late because of the nonspecific nature of the early s igns and symptoms. Echocardiography is a key screening test in the diagnost ic algorithm of patients with suspected PPH. The purpose of tl;is study was to define the echocardiographic Doppler features in patients with PPH at t he time of diagnosis. From 1992 to 1997, 51 patients were diagnosed with PP H at our institution. All underwent a standardized transthoracic echocardio graphic examination, including a contrast study and transthoracic echocardi ographic examination if indicated. Pulmonary artery systolic pressure was c alculated from the tricuspid regurgitation jet. The majority of patients ha d pulmonary artery systolic pressure greater than 60 mm Hg (96%) associated with systolic flattening of the interventricular septum (90%), enlarged ri ght atrium (92%) and ventricle (98%), and reduced right ventricular systoli c function (76%). There was an increase in the interventricular septal thic kness (>1.2 cm) in 21 (43%) of 49 patients, accompanied by a septal/posteri or wall ratio greater than 1.3 in 11 (22%) of 49. Although a reduction in b oth left ventricular systolic and diastolic volumes was noted, global left ventricular systolic function was preserved in all patients. Mitral E/A rat io was less than 0.7 in 7 (22%) patients studied. Color Doppler revealed mo derate to severe tricuspid regurgitation and pulmonic insufficiency in 41 ( 80%) of 51 and 16 (31%) of 51 of cases, respectively. Pericardial effusion (7 small and 1 moderate) and patent foramen ovale (n = 12) were also freque ntly detected. At the time of initial diagnosis, PPH is associated with sec ondary cardiac abnormalities in the majority of patients.