A best evidence topic was constructed according to a structured protocol. The following question was addressed: of the following two procedures, heart–lung transplantation or bilateral-lung transplantation (BLTx), which offers the best outcome for patients with pulmonary hypertension (PH) listed for thoracic transplantation? Of the 77 papers found using a report search for PH and thoracic transplantation, 9 represented the best evidence to answer this clinical question. Overall, 1189 (67%) lung transplantations and 578 (33%) heart–lung transplantations have been reported worldwide for idiopathic PH. For patients with Eisenmenger's syndrome, HLTx represents up to 70% of the transplantation procedures they undergo. On the whole, neither procedure demonstrated an overall survival benefit, when compared with the other. However, PH patients represent a heterogeneous population according to (i) the primary mechanism of PH and (ii) the consequences of PH on right or/and left heart function. With regard to the latter consideration, the current evidence shows that HLTx offers excellent functional and survival outcomes for patients with congenital heart disease and Eisenmenger's syndrome, severe right or/and left heart dysfunction, and who are chronically inotropic dependent. As far as heart dysfunction is concerned, the published evidence approximated cut-off values at 10–25% for the right ventricle ejection fraction (RVEF) and at 32–55% for the left ventricle ejection fraction (LVEF). In the case of lower values for RVEF and LVEF, HLTx should be performed. In all other patients with PH, the evidence demonstrated that BLTx offers a comparable outcome with the advantage of better organ sharing for other recipients. In order to reduce the waiting time on transplantation lists, cardiac repair and BLTx can be offered in experienced centres to patients with simple cardiac anomalies such as atrial septal defect, patent ductus arteriosus or perimembranous ventricular septal defect.