Pulmonary hypertension, family and environment

The focus of interest on pulmonary hypertension (PH) has come through recent therapeutic advances which prolong and improve quality of life. From this interest, recent observations form family studies indicate that a gene located on chromosome 2 is associated with PH. Environmental factors, the use of an anorectic agent, dexfenfluramine, has been associated with a risk of developing primary PH. A link with hypoxic induced PH has emerged in that a strain of rat, the fawn-hooded rat (FHR) which has a susceptibility to developing PH with mild hypoxia has an inherited storage defect to serotonin. Dexfenfluramine, by inhibiting the serotonin transporter, produces a similar pattern of disturbance of biogenic amine metabolism. This observation provides some insight into potential mechanisms for this complex disorder.