Portopulmonary hypertension natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Natural History
Complications
Prognosis
Following diagnosis, mean survival of patients with PPH is 15 months.[1] The survival of those with cirrhosis is sharply curtailed by PPH but can be significantly extended by both medical therapy and liver transplantation, provided the patient remains eligible.
Eligibility for transplantation is generally related to mean pulmonary artery pressures (PAP). Given the fear that those PPH patients with high PAP will suffer right heart failure following the stress of post-transplant reperfusion or in the immediate perioperative period, patients are typically risk-stratified based on mean PAP. Indeed, the operation-related mortality rate is greater than 50% when pre-operative mean PAP values lie between 35 and 50 mm Hg; if mean PAP exceeds 40-45, transplantation is associated with a perioperative mortality of 70-80% (in those cases without preoperative medical therapy).[2][3] Patients, then, are considered to have a high risk of perioperative death once their mean PAP exceeds 35 mm_Hg.[4] The focus on mean PAP values as a chief prognostic index has achieved a consensus according to a recent multicenter study: 45% of patients with PPH were denied OLT candidacy based on the degree of their pulmonary hypertension, while no patient with mPAP < 35 mm_Hg was denied (Between those accepted and those denied, there was no significant difference in cardiac output or right atrial pressure).[5]
Survival is best inferred from published institutional experiences. At one institution, without treatment, 1-year survival was 46% and 5-year survival was 14%. With medical therapy, 1-year survival was 88% and 5-year survival was 55%. Survival at 5 years with medical therapy followed by liver transplantation was 67%.[6] At another institution, of the 67 patients with PPH from 1652 total cirrhotics evaluated for transplant, half (34) were placed on the waiting list. Of these, 16 (48%) were transplanted at a time when 25% of all patients who underwent full evaluation received new livers, meaning the diagnosis of PPH made a patient twice as likely to be transplanted, once on the waiting list. Of those listed for transplant with PPH, 11 (33%) were eventually removed because of PPH, and 5 (15%) died on the waitlist. Of the 16 transplanted patients with PPH, 11 (69%) survived for more than a year after transplant, at a time when overall one-year survival in that center was 86.4%. The three year post-transplant survival for patients with PPH was 62.5% when it was 81.02% overall at this institution.[7]
References
- ↑ Ramsay et al. Severed PHTN in liver Transplant candidates. Liver Transplant Surg 1997 3:494
- ↑ Csete M. Intraoperative management of liver transplant patients with pulmonary hypertension. Liver Transplant Surg 1997:3:454-55
- ↑ Kim et al. Accuracy of Doppler Echos in the assessment of PTHN in liver transplant candidates. Liver Transplant. 6:453, 2000
- ↑ Krowka et al. Pulm Hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver Transplant. Liver Transpl 2000;6:443-450
- ↑ Krowka et al. Hepatopulmonary syndrome and portopulmonary hypertension: A report of the multicenter Liver transplant database. Liver Transplant. 2004;10:174-182
- ↑ Swanson KL et al. Survival in Portopulmonary Hypertension: Mayo Clinic Experience Categorized by Treatment Subgroups. Am J Transpl 2008; 8: 2445–2453
- ↑ Tapper EB, Knowles D, Heffron T, Lawrence EC, Csete M. Portopulmonary hypertension: imatinib as a novel treatment and the Emory experience with this condition. Transplant Proc. 2009 Jun;41(5):1969-71.