[Pulmonary arterial hypertension of unknown etiology. Study of the cardiopulmonary function]

Thirty two cases of pulmonary arterial hypertension of unknown etiology (PAH-UE) were studied. The diagnosis was established by exclusion after the results of hemodinamics studies. Ninety percent of cases had severe PAH (100 mmHg). The end diastolic right ventricular pressure was elevated in 46% of cases. The total pulmonary vascular resistance was increased in the order of the 1700 dinas. seg. cm-5. The cardiac index was 3.3 1/m2 with a right ventricular work index of 3.96 K gm min. The total lung capacity (TLC) was normal, with a vital capacity (VC) of 78% of the predicted value. The residual volume was increased. The mean value of the maximal midexpiratory flow rate (MMFR) was 78%. The A-a gradient of O2 was 30 mmHg, with a venous mixture expressed as a percentage ratio of the cardiac output (Qva/Qt) of 26%. The mean paO2 mmHg and the anatomical pulmonary artery to vein shunt (Qs/Qt) was 9%, with a DLco normal. The clinical hemodinamic correlation was in good agreement. Our results suggested that PAH-UE "per se" has effect in pulmonary function changing slight lung mechanics, and in a moderate degree lung gas exchange. The hipoxemia results meanly from V/Q imbalance.