Abstract Rationale The role of vasomodulator therapy (VT) for patients with portopulmonary hypertension (PoPH), in particular patients who are not transplant candidates, is not well defined. This systematic review and meta-analysis aims to study the clinical metrics and survival of non-transplant patients with PoPH who are treated with VT. Methods A systematic literature was conducted to identify patients with PoPH on VT who did not undergo a liver transplant. Impact of VT on traditional treatment response assessment metrics of pulmonary hypertension was assessed by computation of a mean difference using a random effects model. Cumulative survival was computed using a fixed effects model. We also compared survival of PoPH patients with data on pulmonary hypertension (PH) from other causes. Data are reported descriptively. Results A total of 8 studies were identified enrolling 1,359 patients; 5/8 studies derived data from national registries. Combination VT, phosphodiesterase 5 inhibitor therapy, and endothelin receptor antagonist therapy was used in two studies each. Treatment with VT led to improvement in pulmonary artery pressure, pulmonary vascular resistance, and pulmonary artery wedge pressure (Table). Interestingly, treatment with VT reduced the cardiac index slightly. The 1-, 2-, 3-, and 5-year survival for patients with PoPH on VT who did not undergo liver transplantation was 85%, 68%, 67%, and 48%. In contrast, based on data from 1 study, the 5-year survival of PoPH patients who did not undergo VT was 14%. On indirect subgroup of analysis of VT drug types and combinations, patients who were treated with combination therapy had a relatively lower survival based on data from 1 study (table), which is likely a reflection of PoPH severity. Conclusion Patients with PoPH who did not undergo transplant benefited from VT, with a 5-year survival that is comparable to patients with PH from other causes, whereas those who did not undergo VT had a 5-year survival of 14% (based off data from 1 study). Hence, even in severe PoPH cases, treatment with VT may provide an incremental gain in survival over no therapy. VT led to improvement in pulmonary artery pressure, pulmonary artery wedge pressure, and pulmonary vascular resistance, which can allow for effective assessment of disease response and treatment management. A reduction in cardiac index was noted with VT, the clinical significance of which remains unknown. These findings require further validation in a randomized trial of patients with PoPH who are ineligible for liver transplantation. This abstract is funded by: None
Hussain et al. (Fri,) studied this question.