Primary pulmonary hypertension: clinical, echocardiographic and haemodynamic features in 14 patients

Clinical, echocardiographic and haemodynamic features in 14 patients (8 male and 6 females) with primary pulmonary hypertension, diagnosed by strict clinical and haemodynamic criteria are described. Age of the patients at diagnosis ranged from 11 years to 40 years with a mean of 23 years. The mean interval from onset of symptoms to diagnosis was 2.6 years. Common symptoms included dyspnoea on exertion (86%), fatigue (78%) and palpitation (78%). Raynaud's phenomenon was not encountered but one patient had signs and symptoms of left recurrent laryngeal nerve palsy. Pulmonary function studies showed mild restrictive ventilatory impairment (mean forced vital capacity 80% of predicted) with hypoxaemia and hypocapnea. The M-mode tracing of the pulmonary valve showed flat or negative E-F slope, a small or absent 'a' wave, and midsystolic notching in all the patients. Two-dimensional echocardiographic images showed a thickened right ventricular wall in 12(86%) patients; a normal to small left ventricular end-diastolic internal dimension in all the patients and right ventricular and right atrial enlargement in 78% of patients. Significant tricuspid regurgitation and pulmonary regurgitation was documented by Doppler interogation in 87% and 62% of patients examined respectively. Haemodynamic findings consisted of a marked rise of pulmonary artery pressure and pulmonary vascular resistance, low cardiac index and normal pulmonary artery wedge pressure. At cardiac catheterisation the mean right atrial pressure (mean +/- SD) was 9.8 +/- 4.8 mmHg; mean pulmonary artery pressure, 63.2 +/- 14.3 mmHg; cardiac index, 2.2 +/- 0.9 L/min. m2; and pulmonary vascular resistance index, 22.3 +/- 10.6 mmHg/L/min. m2; in these patients. No, death nor any sustained morbid events occurred during the diagnostic evaluation of the patients.