Abstract: . . .  subcutaneously to avoid intravenous line infection. Oral bosentan has been shown to improve haemodynamics, exercise capacity and functional class in patients in the New York Heart Association (NYHA) Class II and III. It can be used as first-line therapy in such patients. e. This patient can be offered either a heartlung, double-lung or single-lung transplant depending on donor availability and on centre expertise. The overall survival at 1 year Ch19.qxd 30/11/04 12:24 PM Page 288 Page 13 289 PULMONARY HYPERTENSION SELF-ASSESSMENT 19 is 60%, at 5 years 40%, and at 10 years 30%. There does not appear to be a recurrence of pulmonary hypertension in the transplant recipients. However, the main cause of mortality is obliterative bronchiolitis. f. In the familial form of PPH, the age of onset is variable and the penetrance is incomplete, such that members of families with PPH can inherit the gene as well as have progeny with PPH yet may never develop the disease. The gene for PPH has been localized to chromosome 2q31-32 and has been associated with mutations of BMPR2. It has to be explained to the patients that, although there is a screening test for BMPR2 mutations, only a proportion of those with the gene alteration actually develop the disease. Until further research is carried out, she should be advised that her  . . .
. . .  avoid intravenous line infection. Oral bosentan has been shown to improve haemodynamics, exercise capacity and functional class in patients in the New York Heart Association (NYHA) Class II and III. It can be used as first-line therapy in such patients. e. This patient can be offered either a heartlung, double-lung or single-lung transplant depending on donor availability and on centre expertise. The overall survival at 1 year Ch19.qxd 30/11/04 12:24 PM Page 288 Page 13 289 PULMONARY HYPERTENSION SELF-ASSESSMENT 19 is 60%, at 5 years 40%, and at 10 years 30%. There does not appear to be a recurrence of pulmonary hypertension in the transplant recipients. However, the main cause of mortality is obliterative bronchiolitis. f. In the familial form of PPH, the age of onset is variable and the penetrance is incomplete, such that members of families with PPH can inherit the gene as well as have progeny with PPH yet may never develop the disease. The gene for PPH has been localized to chromosome 2q31-32 and has been associated with mutations of BMPR2. It has to be explained to the patients that, although there is a screening test for BMPR2 mutations, only a proportion of those with the gene alteration actually develop the disease. Until further research is carried out, she should be advised that her children could . . .
--3000,2,750,3173,30253