Hemodynamic features and impaired arterial oxygenation in patients with portopulmonary hypertension.

Abstract Portopulmonary hypertension (P-PHT) is sporadically found in cirrhosis patients who have portal hypertension. We retrospectively investigated the clinical features of six patients with P-PHT and compared their hemodynamics and arterial oxygenation with data from 60 cirrhosis patients without pulmonary hypertension (non-PHT cirrhosis) admitted to our department. The mean pulmonary artery pressure and pulmonary vascular resistance index of P-PHT patients ranged from 25 to 57 mmHg and from 399 to 1405 dynes s cm −5  m −2 , respectively, and their arterial oxygenation was impaired. The systemic vascular resistance and cardiac index of P-PHT patients were similar level to those of patients with non-PHT cirrhosis. We found 10 patients with non-PHT cirrhosis in whom pulmonary vascular resistance exceeded the critical level for pre-capillary pulmonary hypertension (120 dynes s cm −5 ). These patients showed a distinctive hemodynamic profile, including a decrease of cardiac output due to contraction of the plasma volume and resultant elevation of systemic vascular resistance. However, the decrease of cardiac output contributed little to the elevation of pulmonary vascular resistance. Our findings suggested that certain factor(s) were acting to raise pulmonary vascular tone in these patients, which might cause chronic damage to the pulmonary vascular bed, leading to the onset of pulmonary hypertension.