UNEXPLAINED PULMONARY-HYPERTENSION IN CHILDREN IN THE HIGHLANDS OF PAPUA-NEW-GUINEA

We report the clinical features, electrocardiographic and chest X-ray findings, and Doppler-measured pulmonary artery pressures in 22 childr en admitted to Goroka Base Hospital with primary pulmonary hypertensio n and compare them with findings in 10 controls, Symptoms frequently r eported by patients were recurrent cough (95%) and shortness of breath (77%), 82% of the patients had increased intensity of the pulmonary c omponent of the second heart sound, 45% of the patients had clinical e vidence of right heart failure, Chest X-rays were readable in 14 patie nts: the mean cardio-thoracic ratio was 65% and in the controls 58%; t he mean diameter of the descending branch of the right pulmonary arter y was 9 mm and in the controls 6.4 mm, 13 patients and 8 controls had electrocardiograms: 11 patients had right axis deviation, 2 a normal a xis, and all showed evidence of right ventricular hypertrophy; 7 contr ols had a normal axis, 1 had right axis deviation, and 3 controls had right ventricular hypertrophy, Pulmonary artery pressures in the patie nts ranged from 41 to 137 mmHg with a mean of 79 mmHg, The control gro up had a mean pulmonary artery pressure of 28 mmHg, but 5 of the 10 co ntrols had pulmonary artery pressures greater than the accepted norm ( peak systolic pressure less than 30 mmHg), Altitude and chest infectio n may be playing a role in the pathogenesis of pulmonary hypertension in patients in the highlands but further studies need to be done to de fine the causes and the pathological changes in the pulmonary vasculat ure, as well as to determine local norms and the natural history of pu lmonary hypertension in highland children.