Echocardiographic features of primary pulmonary hypertension

Purpose: The aim of this study was to describe the Transthoracic Two-dimensional Echo (TTE) features of primary pulmonary hypertension (PPH) at the time of diagnosis. Methods: From 1992 to 1997, 51 pts (42±12 yrs, range 17 to 68, 6 men vs. 45 women) were diagnosed with PPH at our institution. All underwent a standardized TTE examination, including a contrast study. Pulmonary artery systolic pressure (PASP) was calculated from the tricuspid regurgitation (TR) jet. Results: Elevated PASP was present in all pts (95±24, 65-166mmHg). TTE demonstrated enlargement of right atrium (RA) (n=47; 92%) and ventricle (RV) (n=50; 98%), RV hypertrophy (n=24; 47%), S: moderately reduced RV systolic function (n=39; 76%), > moderate TR (n=41; 80%), interventricular septal thickness > 1.3 (1.7±0.3; range 1.4 to 2.3) (n=7; 14%) and increased septal/posterior wall ratio [1.2±0.3, 0.7 to 2.7cm (mean±SD, range)] and peak left ventricular outflow tract gradient of 16mmHg (n=1). Systolic flattening of the septum occurred in 46 pts (90%); ≥moderate pulmonary insufficiency was noted in 16 pts. (31%). In 14 of 51 pts (27%), a contrast study showed interatrial right to left shunt consistent with a patent foramen ovale. The mitral E/A ratio was abnormal (<0.7) in 7 (22%) cases (age 47±12yrs, range 28 to 62yrs). Pericardial effusion [n=8: 7(small) + 1(moderate)] was also noted. The global left ventricular systolic function was normal in all patients. Conclusions: At the time of initial diagnosis, PPH is associated with secondary cardiac abnormalities in the majority of patients. Clinical Implications: TTE is a key screening test in the diagnostic algorithm of patients with suspected PPH.